University of Southern California Dissertations and Theses

A clinical investigation of hearing deficiencies and some possible etiological factors in a group of cerebral palsied individuals

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A clinical investigation of hearing deficiencies and some possible etiological factors in a group of cerebral palsied individuals

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Content A CLINICAL INVESTIGATION OF HEARING DEFICIENCIES A N D SOME POSSIBLE ETIOLOGICAL FACTORS IN A GROUP OF CEREBRAL PALSIED INDIVIDUALS A D i s s e r t a t i o n P re s e n te d to th e F a c u lty o f th e D epartm ent o f S peech The U n iv e r s ity o f S o u th e rn C a lif o r n i a I n P a r t i a l F u lf illm e n t o f th e R eq u irem en ts f o r th e D egree D o c to r o f P h ilo so p h y by F ra n k M aurice Lassman J u ly 1950 UMI Number: DP31983 All rights reserved INFORMATION TO ALL USERS The quality of this reproduction is dependent upon the quality of the copy submitted. In the unlikely event that the author did not send a complete manuscript and there are missing pages, these will be noted. Also, if material had to be removed, a note will indicate the deletion. Oissiitafcn %bli&kng UMI DP31983 Published by ProQuest LLC (2014). Copyright in the Dissertation held by the Author. Microform Edition © ProQuest LLC. All rights reserved. This work is protected against unauthorized copying under Title 17, United States Code ProQuest LLC. 789 East Eisenhower Parkway P.O. Box 1346 Ann Arbor, Ml 48106- 1346 pK.Û. This dissertation, written by F R M S .M U m E .J U 3 3Jî!lM.............................................. under the guidance of Faculty Committee on Studies, and approved by all its members, has been presented to and accepted by the Council on Graduate Study and Research, in partial ful fillment of requirements for the degree of D O C T O R OF P H I L O S O P H Y Date JX A j^iA -..... Committee on Studies CkSkman ill ACKNO W LED G EM ENT The a u th o r w ish e s to e x p re s s h i s a p p re c i a t i o n to D r, Lee Edward T ra v is t o r h i s v a lu a b le g u id a n c e and g e n u in e encouragem ent th ro u g h o u t th e p ro g re s s o f th e in v e s t i g a t i o n and d u rin g th e p e r io d o f th e a u t h o r ’ s g ra d u a te s tu d y w ith him* The a u th o r a ls o w ish e s to th a n k D r, R o b ert H a rrin g to n , Dr* Jo se p h J o h n s to n , D r, F lo y d Ruch, and D r, M edford W esley who gave o f t h e i r v a lu a b le tim e and i n t e r e s t d u rin g th e c o u rs e o f th e s tu d y . S p e c ia l g r a t i t u d e i s due th e s t a f f o f The C h ild re n ’ s H o s p ita l, Los A n g e le s, and p a r t i c u l a r l y to Dr* M arg aret J o n e s , D ir e c t o r , and M rs, E liz a b e th O lson, M édical S o c ia l W orker, o f th e C e re b ra l P a ls y T re atm en t C e n te r, to M iss Rose W ile , O u tp a tie n t D epartm ent D ir e c t o r , and to D r, V ic to r G o o d h ill and D r, W arren L in e o f th e D epartm ent o f O to la ry n g o lo g y and th e E a r, N ose, and T h ro a t C l i n i c , Iv TABLE OP CONTENTS CHAPTER PAGE I . THE PROBLEM .............................................. . . 1 I I . REVIEW OP THE LITERATURE . 6 G en e ra l in f o rm a tio n c o n c e rn in g c e r e b r a l p a ls y . • ................................ 6 In c id e n c e 6 C auses . . . . . . . . . . . . . . V Types 11 S p a s t i c i t y ♦ • • • . . . # • » • • • • 13 A th e to s is • • • • • * • • « • • • « • 15 R ig id ity • . • . , . $ . . . . . . . 17 Trem or . . . . . . . . . . . . . . . . 18 A ta x ia • • . • # • • 19 Combined o r m ixed ty p e s • • • • • • • 20 N eu ro p ath o lo g y 21 S p a s t i c i t y • •* • •• • •• , «* • • 22 A th e to s is « • • • • • • • • • • « • • 25 R i g id ity 28 Trem or 30 A ta x ia 31 T reatm en t ....................... 33 P h y s io th e ra p y . . . . . . . . . 34 V CHAPTER PAGE O c c u p a tio n a l th e ra p y 57 B ra c in g 58 S u rg e ry ........................ 59 D rugs • « • • * • « • • . . . • . • • 42 S p eech th e ra p y . . . . . . . . 44 ^ L i t e r a t u r e on th e h e a rin g o f c e r e b r a l p a l s i e d c h ild r e n 49 I I I . DESIGN AND PROCEDURE . . . . . . . . . . . . 55 S e le c ti o n o f s u b je c ts . . . . . . . , . « 54 C e re b ra l p a ls y e x a m in a tio n . . . . . . . 56 O to r h in o la r y n g o lo g ic a l e x a m in a tio n . . . 56 A u d io m etric t e s t room and t e s t equipm ent . 57 T e s t room . . . . . . . . . . . . . 57 E quipm ent . . . . . . . . . . . 58 C a lib r a t io n * • . . . • • • * . , • • • 58 S p e c ia l c h a i r . . . . . . . . . . . . . 59 T e s t b a t t e r y and t e s t p ro c e d u re . . . . . 60 A ir c o n d u c tio n th r e s h o ld t e s t . . . . . 60 Bone c o n d u c tio n th r e s h o ld t e s t • « • • . 62 S peech r e c e p tio n th r e s h o ld t e s t « • • • 64 A u x ilia r y p ro c e d u re s i n a u d io m e tric t e s t i n g . ..................................... 66 Summary ....................... 69 Vi CHAPTER PAGE IV . RESULTS . . . . . . . . . . . . 70 D e s c r ip tio n o f th e s u b je c t sam ple .... 71 D ia g n o s tic s u b -ty p e c a te g o r ie s . . . « 72 Age . . . . . . . . . . . 74 Sex . . ............................ 75 E x te n t 75 S e v e r ity . . . . . . 81 E tio lo g y . . . . . . . . . . . 84 D rugs . . . . . . . . . . . . 90 A u d io m etrio t e s t f in d in g s « •« • •. . , 94 P ure to n e a i r c o n d u c tio n m easu res • . # 97 Means . . . . . . . . . . . . . . . 97 V a r i a b i l i t y . . . . . . . . . . . . . 100 M edians . • . . • . • • • • • • . . . 101 S ig n if ic a n c e o f th e means * . • . . . 101 C om parison w ith c o n tr o l group • . • • 104 P olygons .......... 108 A verage o f th e sp e ech f r e q u e n c ie s « « • 115 Spondee word t e s t m easu res . . . . . . 117 R ig h t e a r - l e f t e a r c o r r e l a t i o n s . . . 121 T e n sio n v s . n o n - te n s io n a t h e to i d s . . . 123 P u re to n e bone c o n d u c tio n m easu res . • 124 U n i l a t e r a l and b i l a t e r a l m a rg in a l l o s s e s . . . . . . . . . ........................ 126 v l i CHAPTER PAGE S p o o ifio BO t e s t r e s u l t s • 128 Summary * « • • • • • • • • • « • • • • 154 T e s t - r e t e s t r e l i a b i l i t y 157 O to r h in o la r y n g o lo g ic a l e x a m in a tio n f in d in g s . . . . . . . . . . . . . . 144 The ear . . . . . . . . . . . . . . 147 The n o se . . ♦ . . . . . . • . . ♦ . . . 149 The m outh . . . . . . . . . .. . . 154 P hary n x . • . . . . . . # . . . . . . . 154 N asopharynx 154 D is c u s s io n . . . . . . . . . . . . . 158 I n t e r r e l a t i o n s h i p s o f a u d ito r y t e s t , m e d ic a l, and c a s e h i s t o r y d a ta . • • • . 160 Type o f l o s s 160 Judgm ent c r i t e r i a and p ro c e d u re . . . . 161 Judgm ent r e s u l t s ............................................ 162 E tio lo g y and h e a rin g im p airm en t • . • • • 166 Background f a c t o r s .. .. .. . 166 C o r r e la tio n s . . . .. . .. . . 170 F u r th e r r e l a t i o n s h i p s . . . . . . 175 D rug d o sag e and h e a r in g im p airm en t . . . . 177 V. GENERAL DISCUSSION . . . . . . . . . . . . . 182 E r y t h r o b la s to s is . . . . . . . . . . . . 185 P e r tin e n t la b o r a to r y e v id e n c e . . . . . . 190 v l i i CHAPTER PAGE V I. SHMMARÏ . . . . . . . . . . . ........................ . 19Ü BIBLIOGRAPHY . ....................................................... 205 APPENDIX ..................................................................... 254 ix vLIST OF TABLES TABLE PAGE I . In c id e n c e o f V a rio u s Types o f C e re b ra l P a ls y I I . Mean Age, S ta n d a rd D e v ia tio n , and T o ta l Range f o r Each C e re b ra l P a ls y Type and f o r th e T o ta l Sam ple . . . . . . . . . 76 I I I . C om parison o f Sex D i s t r i b u t i o n o f P re s e n t Sam ple w ith P re v io u s S tu d ie s . . . . . 77 IV . Sex D i s t r i b u t i o n o f th e P re s e n t Sam ple A cco rd in g to Type o f C e re b ra l P a ls y . . 78 V# E x te n t o f M otor In v o lv em en t . . . . • . . 79 V I. C om parison w ith P re v io u s S tu d ie s i n R egard to th e E x te n t o f M otor In v o lv em en t . • 82 V I I . D i s t r i b u t i o n o f S e v e r ity Among Sub-T ypes and f o r th e T o ta l Sample . . . . . . . 85 V I I I . R e la tiv e In c id e n c e o f V ario u s E tio lo g ie s f o r Each P a ls y Type and f o r th e T o ta l Sam ple . . . . . . . . . . . . 86 IX . P e rc e n ta g e s o f P re s e n t Sam ple i n V ario u s E t i o l o g i c a l C a te g o rie s Compared to O th er C l i n i c a l S tu d ie s . . . . . . . . 89 X# In c id e n c e o f Drug D osage f o r Each P a ls y Type and f o r th e T o ta l Sam ple . . . . . 91 X TABLE PAGE XI* A ir C o n d u ctio n T h re sh o ld A c u ity M easures, i n D e c ib e ls , o f C e n tr a l Tendency and D is p e rs io n f o r Each C e re b ra l P a ls y Type W ith T R a tio s o f S ig n if ic a n c e o f th e D e v ia tio n from th e T o ta l P o p u la tio n * « 98 X II. T R a tio s o f th e D if f e r e n c e Betw een Each Group Mean and th e P o p u la tio n Norm, and T h e ir S ig n if ic a n c e . . . . . . . . 105 X I I I . C om parison o f A ir C o n d u ctio n T h re sh o ld M easures f o r Each C e re b ra l P a ls y Type W ith T h re sh o ld M easures o f C o n tro l Group ............................................... 105 XIV. T R a tio s o f D if fe re n c e Betw een Each Group Mean and th e C o n tro l Group Mean, and T h e ir S ig n if ic a n c e . . . . . . . . . . 107 XV. A verage o f S p eech F re q u e n c ie s (5 1 2 , 1024, and 2 0 4 8 ), P u re Tone A ir C o n d u ctio n M easures f o r Each C e re b ra l P a ls y Type » 116 XVI. M easures o f C e n tr a l T endency and V a ria b i l i t y , i n D e c ib e ls , o f S p eech R e c e p tio n T h re sh o ld s f o r Spondee Word T e s t . . . 119 X V II. C om parison o f R ig h t E ar and L e ft E ar f o r A ir C o n d u ctio n and P u re Tone D a ta . ♦ . 122 xi TABLE .page XVIII* B i s e r i a l C o e f f ic ie n ts o f C o r r e la tio n Betw een T e n sio n and H e a rin g A c u ity , W ith th e Mean V alues f o r E ach F req u en cy and f o r th e S pondees o f th e T e n sio n and Non- T e n sio n Sub-G roups 126 XIX* I n c id e n c e o f U n i l a t e r a l and B i l a t e r a l M a rg in a l L o sses . # . • . . . . * * 1^7 XX. Bone and A ir C o n d u ctio n V alues o f M a rg in al L oss Groups • . . . . . . . . . 150 XXI# M easures o f C e n tr a l T endency, V a r i a b i l i t y , and S ig n if ic a n c e f o r M easures o f D if f e re n c e Betw een F i r s t T e s t and Second T e s t D a ta , F o u rte e n S u b je c ts . . . . . . 140 XXII * F in d in g s o f M edical E x am in atio n o f th e E ar 148 X X III. N asa l E x am in atio n F in d in g s . . . . . . . 150 XXIV. O ra l E x am in atio n F in d in g s . . . . . . 155 XXV. Judgm ent o f E ar P a th o lo g y Type . . . . . . 165 XXVI. P o i n t - B i s e r i a l C o e f f ic ie n ts o f C o r r e la tio n Betw een C e re b ra l P a ls y E tio lo g y and H e a rin g L oss . . . . . . . . . . . . 172 XXVII# Judgm ent o f E a r P a th o lo g y T ype, E r y t h r o b la s to s i s S u b je c ts E x clu d ed . . . 174 xX% TABLE PAGE X X V III. Mean and S ta n d a rd D e v ia tio n V alu es f o r E r y th r o b la s to s i s A th e to id s and A th e to id s o f O th er E t i o l o g i c a l C l a s s i f i c a t i o n s W ith T R a tio M easures o f S ig n if ic a n c e o f th e D e v ia tio n s fro m th e Norm , . . • 176 XXIX. A verage o f S p eech F re q u e n c ie s (5 1 2 , 1024, and 2 0 4 8 ), P u re Tone A ir C o n d u ctio n M easu res, f o r Each C e re b ra l P a ls y Type W ith S u b je c ts o f E r y t h r o b la s to s i s E tio lo g y E x clu d ed . . . . . . . . . 178 XXX. P o i n t - B i s e r i a l C o e f f ic ie n ts o f C o r r e la tio n B etw een A d m in is te re d Drugs and H e a rin g A c u ity . . . . . . . . . . 180 XXXI. A n a ly s is o f S u b je c ts G iven "M ixed" R a tin g o f S e v e r ity . . . . . . . . 242 XXXII. Age, E x te n t o f In v o lv e m e n t, and S e v e r ity o f th e H andicap i n th e T e s t- R e te s t R e l i a b i l i t y Group . . . . . . . . . 245 X X XIII. S p e c i f ic Judgm ents o f P a th o lo g y Type by Two Ju d g es 244 xiii LIST OF FIGURES FIGURE PAGE 1 . The P r e o e n tr a l M otor C o rte x o f M a n ................... 25 2* Mean A verage A ir C o n d u ctio n T h re sh o ld A udiogram s, R ig h t E ar and L e f t E a r, f o r Types o f C e re b ra l P a ls y • • 99 5# F req u en cy P olygons o f A ir C o n d u ctio n T h re sh o ld s f o r Each F re q u e n cy , R ig h t E ar and L e f t E a r, S p a s ti c S u b je c ts (N»58) w ith Mean A udiogram s . . . . . . . . 109 4* F re q u en cy P olygons o f A ir C o n d u ctio n T h re sh o ld s f o r Each F re q u e n c y , R ig h t E ar and L e ft E a r, A th e to id S u b je c ts (N -4 1 ), w ith Mean A udiogram s . . . . . . . . . I l l 5* F re q u en cy P olygons o f A ir C o n d u ctio n T h re sh o ld s f o r Each F re q u e n c y , R ig h t E ar and L e ft E a r, R ig id ity S u b je c ts (N®9), w ith Mean A udiogram s ... 112 6 . F re q u en c y P olygons o f A ir C o n d u ctio n T h re sh o ld s f o r Each F re q u e n c y , R ig h t E ar and L e ft E a r, Combined S u b je c ts (N’s i l ) , w ith Mean A udiogram s . . . . . . . . . . 115 7 . S uperim posed F req u en cy P olygons o f A ir C o n d u ctio n T h re sh o ld s f o r S p a s t i c , A th e to id , R i g i d i t y , and Combined C a te g o rie s • • , , 114 xiv FIGURE page 8 . S c a t t e r D iagram o f T h re s h o ld V alu es o f F i r s t T e s t Compared to Second T e s t T h re s h o ld s ............................. 145 9 . Form L e t t e r to P a re n ts I n v i t i n g R e se a rc h P a r t i c i p a t i o n ........................................... 255 1 0 . Form f o r R eco rd in g E a r, Nose and T h ro a t E x am in atio n D ata .......... 256 1 1 . Mean Auddogram f o r F o rty Norm al C h ild re n Used as C a lib r a t io n Group . . . . . . . 259 12# A udiogram Form Used I n R e se a rc h . . . . . 240 1 5 , L i s t s o f Spondee Words (R ecorded) U sed i n S p eech R e c e p tio n T h re sh o ld T e s t • • • • 241 CHAPTER I THE PROBLEM The s tu d y o f th e fu n d a m e n ta l n a tu r e o f th e p roblem o f c e r e b r a l p a l s y , o f th e u n d e rly in g c a u se s and p a th o g e n e s i s o f th e c o n d itio n , i s b a s ic t o th e s t o r e o f tr e a tm e n t te c h n iq u e s we w ish to am ass. The developm ent o f th e r a p e u tic p ro c e d u re s f o r c e r e b r a l p a ls y p a t i e n t s h a s b een c o m p lic a te d 'a n d d e la y e d by an e r r o r o f o v e r - g e n e r a l iz a tio n . The r e c o g n itio n o f s e p a r a te g ro u p s , c l i n i c a l l y d i f f e r e n t i a t e d , w ith in th e l a r g e r body o f p a t i e n t s , h a s b ee n o n ly a r e l a t i v e l y r e c e n t ad v an ce. F iv e g ro u p s o r ty p e s a r e now re c o g n iz e d by m ost w o rk ers^ a c t i v e l y engaged i n th e f i e l d : s p a s t i c i t y , a t h e t o s i s , r i g i d i t y , tre m o r, and a t a x i a . E ach v a r i e s s i g n i f i c a n t l y from th e o th e r s in d e s c r i p t i o n and d y n am ics.^ ^W inthrop M. P h e lp s , "The C e re b ra l P a l s i e s , " M ltc h e ll- H elso n T extbook o f P e d i a t r i c s * Waldo E. N elso n , e d i t o r ( P h i1a d e lp h ia : W. B. S au n d ers Company, 1 9 4 5 ), p p . 1111-1116. ^Meyer A. P e r l s t e i n , The P roblem o f C e re b ra l P a ls y Today (New Y ork: A s s o c ia tio n f o r th e A id o f C rip p le d C h ild re n , Ï 9 4 7 ) , p . 7 . ^ E a rl R. C a rls o n , "T reatm en t - I n f a n t i l e C e re b ra l P a ls y ," N orthw est M e d ic in e . 4 1 :1 6 0 , May, 1942. "^ ra n k B odolph F o rd , D is e a s e s o f th e N ervous System i n I n f a n c y . C h ild h o o d , and A&oïesco n ce ( S p r in g f ie I d : & iar1es G. Thomas Company, Ï 9 3 7 ) , 953 p p . R eal p r o g re s s h a s been made s in c e 1861 ^ e n D r. L i t t l e ^ v i v i d l y d e s c r ib e d and a lm o st u n f o r tu n a te ly c a te g o r iz e d " fe e b le -m in d e d ," " d r o o lin g ," " to e -w a lk in g ," " c r o s s - le g g e d " c h ild r e n w ith " s p a s t i c p a r a l y s i s . " But a c l e a r u n d e rs ta n d in g o f th e d i f f e r e n t i a l p a th o lo g ie s and p a th o g e n e se s o f th e d iv e r s e s u b -ty p e s i s f a r from accom plishm ent B e fo re o u r r e h a b i l i t a t i o n program s can f u n c tio n w ith m axi mum e f f e c t i v e n e s s , much r e s e a r c h i s need ed to p ro v id e in f o r m a tio n , d i f f e r e n t i a t e d b y c l i n i c a l s u b -ty p e . T h u s, te c h n iq u e s o f tre a tm e n t can be r e f i n e d i n l i n e w ith c o m p a ra tiv e d if f e r e n c e s from p a t i e n t to p a t i e n t . & The need f o r th e i n v e s t i g a t i o n w hich i s th e s u b je c t o f t h i s d i s s e r t a t i o n was made c l e a r w ith in th e p r o f e s s io n a l en v iro n m en t o f a u n i v e r s i t y sp e e c h and h e a rin g c l i n i c . R e p e a te d demands w ere made on th e i n v e s t i g a t o r by h o s p i t a l c l i n i c s , h o s p i t a l s c h o o ls , o th e r c l i n i c i a n s and co n c ern ed p a r e n ts to a s s e s s th e h e a rin g a c u i t y o f c e r e b r a l p a l s i e d c h i l d r e n . I n many i n s t a n c e s , th e program o f r e h a b i l i t a t i o n J . L i t t l e , "On th e I n f lu e n c e o f Abnorm al P a r t u r i t i o n , D i f f i c u l t L a b o rs, P re m atu re B i r t h , A sphyxia N eonatorum , on th e M ental and P h y s ic a l C o n d itio n o f th e C h ild , E s p e c ia lly i n R e la tio n t o D e f o r m itie s ." T ra n s a c tio n s o f th e O b s t e t r i c a l S o c ie ty . 3 :2 9 3 -3 4 4 , 1862. . ^W inthrop M. P h e lp s , "R ecen t S i g n if ic a n t T ren d s i n th e C are o f th e C e re b ra l P a ls y ," S o u th e rn M ed ical J o u r n a l . 3 6 :1 3 2 -1 3 8 , F e b ru a ry , 1 9 46. f o r th e c h i l d was b e in g d e la y e d o r h a n d ic a p p e d due to in d e c is i o n re g a rd in g th e h e a r in g c a p a c ity . The d i f f e r e n t i a l d ia g n o s is o f te n in v o lv e d a c h o ic e b etw een h e a rin g l o s s , m e n ta l r e t a r d a t i o n , a p h a s ia , and e m o tio n a l w ith d ra w a l. Any one o f th e s e o r any co m b in atio n o f them c o u ld a c co u n t f o r d e f e c tiv e sp eech d ev e lo p m e n t, p o o r re s p o n s e t o p h y s io t h e r a p i s t s and o c c u p a tio n a l t h e r a p i s t s , d e la y e d p ro g re s s I n s p e c ia l s c h o o lin g , and f a i l u r e i n th e v a r i e t y o f a d j u s t - m en t8 demanded o f t h e c h i l d . The c o n c e rn h e re was w ith a h a n d ic a p su p e rim p o sed , a s i t w e re , upon a h a n d ic a p . The a s s i s t a n c e o f th e a u d io lo g is t i n t h i s m a tte r was r e q u ir e d n o t o n ly by sp eech t h e r a p i s t s i n g u id in g t h e i r p e c u l i a r w ork, b u t by o th e r u n i t s i n th e r e h a b i l i t a t i o n team . The p h y s i o t h e r a p i s t , th e o c c u p a tio n a l t h e r a p i s t , th e o rth o p a e d ic s p e c i a l i s t , th e p e d i a t r i c i a n , th e n u r s e , th e t e a c h e r , th e n e u r o l o g i s t , th e p a r e n t , — ea ch w orking to w a rd a common g o a l— depend upon an a c c u ra te ; a p p r a i s a l o f h e a rin g e f f i c i e n c y f o r a b a s ic o r i e n t a t i o n i n t h e i r a p p ro a c h . They m ust b e c o n fid e n t o f com m unication w ith th e p a t i e n t o r m o d ify t h e i r c l i n i c a l ap p ro a c h e s a c c o r d in g ly . To ill u m in a te t h i s a s p e c t o f th e c l i n i c a l p roblem was th e g e n e ra l p u rp o se o f t h i s i n v e s t i g a t i o n , t h a t i s , to p ro v id e in fo rm a tio n r e g a r d in g th e c o n d itio n o f th e h e a rin g p ro c e s s i n t h i s c l i n i c a l p o p u la tio n . 4 T h is s tu d y i s d e s ig n e d (1 ) to d e s c r ib e th e n a tu r e o f h e a rin g e f f i c i e n c y i n r e p r e s e n t a t i v e g ro u p s o f d i f f e r e n t i a l l y d ia g n o se d c e r e b r a l p a l s i e d c h i l d r e n , d e te rm in in g g ro u p te n d e n c ie s and in te r - g r o u p d if f e r e n c e s ; and (2 ) t o illu m i n a t e th e c h a r a c te r o f o to lo g ic p a th o lo g y u n d e rly in g h e a r in g d e f ic ie n c y when i t o c c u rs i n t h i s p o p u la tio n . The f o llo w in g c l i n i c a l h y p o th e s e s s t a t e d i n th e form of q u e s tio n s r e q u i r in g r e s o l u t i o n , fa s h io n e d e x p e rim e n ta l d e s ig n : (a ) I s h e a rin g p a th o lo g y i n th e c e r e b r a l p a ls y group s i g n i f i c a n t l y d i f f e r e n t i n c h a r a c te r th a n p a th o lo g ic o t i c c o n d itio n s fo u n d i n o th e rw is e n o rm al p e o p le ? (b ) Can th e fo c u s o f h e a rin g p a th o lo g y be lo c a te d by d i r e c t o r i n f e r e n t i a l d a t a , i n th e e x t e r n a l e a r , tym panic c a v i t y , o r i n th e in n e r e a r - s e n s o r y n e rv e system ? (c ) I s th e in c id e n c e o f h e a rin g d e f ic ie n c y and c e r e b r a l p a ls y o n ly c o i n c id e n ta l ly fo u n d , o r does some r e l a t i o n s h i p l e s s f o r t u i t o u s e x i s t b etw een them? (d ) How d o es th e s t a t u s o f h e a rin g a c u ity r e l a t e to th e v a r io u s e t i o l o g i e s c a u s a tiv e o f th e p a ls y ? I s t h e r e any s y s te m a tic r e l a t i o n s h i p ? (e ) What c o r r e l a t i o n e x i s t s betw een h e a r in g a c u ity and c e r t a i n d ru g s p r e s c r ib e d i n tr e a tm e n t p ro c e d u re s ? W 1 ( f ) I s any r e l a t i o n s h i p a p p a re n t betw een p o s tn a t a l f e b r i l e d is e a s e s and h e a r in g d e f i c i e n c i e s ? CHAPTER X I REVIEW OP THE LITERATURE I . GENERAL INFORMATION CONCERNING CEREBRAL PALSY The r e l a t i v e l y n eb u lo u s c h a r a c te r o f " c e r e b r a l p a ls y " i s a p p r e c ia te d . I n o r d e r to c l e a r l y d e f in e th e n a tu r e o f th e p o p u la tio n s tu d i e d , a d is c u s s io n o f th e in c id e n c e , c a u s e s , ty p e s , n e u ro p a th o lo g y , and tr e a tm e n t o f c e r e b r a l p a ls y i s o f f e r e d . C e re b ra l p a l s y i t s e l f i s n o t a d is e a s e e n t i t y . I t i s r a t h e r a r e s u l t , - - a n e u ro m u sc u la r d i s a b i l i t y cau sed by in j u r y to c e r t a i n b r a in c e n t e r s . The d i s a b i l i t y may b e s e v e re o r v e ry m ild ; many m u sc les o r g ro u p s o f m u sc le s may be a f f e c t e d , o r o n ly a few o r a s in g l e g ro u p . The la c k o f c o n tr o l may b e in th e arm s, l e g s , to n g u e , sp eech m echanism , e y e s , o r i t may a f f e c t th e h e a r in g . The e x te n t o f th e d i s a b i l i t y v a r ie s w id e ly and may a f f e c t th e e n t i r e ra n g e o f norm al m u sc u lar a c tiv ity .* ^ In c id e n c e . P h e lp s h a s e s tim a te d t h a t th e r e a r e sev en new c a s e s o f c e r e b r a l p a ls y p e r one h u n d red th o u sa n d b o rn e v e ry y e a r in t h i s c o u n try . One o f th e sev en w i l l d ie ^ C h ild re n W ith C e re b ra l P a ls y —A R e p o rt on th e Problem in C a lif o r n i a and a SuRRested P rogram f o r T h e ir C a re . T Sacram ento: S ta te D epartm ent o f P u b lic H e a lth and E d u c a tio n , D ecem ber, 1 9 4 4 ), p p . 3 - 4 . w ith in a few y e a r s a f t e r b i r t h . The o th e r s i x have a re a s o n a b le chance t o re a c h a d u lth o o d . W ith a d e q u a te c a re th e m a jo r ity l i v e t o a t l e a s t m id d le age and many w e ll p a s t i t le a d in g t o a la r g e a c c u m u la tio n o f c a s e s . P h elp s and T u rn e r^ a r e co n v in ced t h a t th e num bers o f c h ild r e n w ith c e r e b r a l p a ls y a r e b e in g u n d e re s tim a te d due to v a ry in g d e f i n i t i o n s o f a " c r ip p le d c h i l d ," c o n fu s io n w ith fe e b le -m in d e d n e s s , fa m ily p r i d e , m is u n d e rs ta n d in g , and i n a c c e s s i b i l i t y o f r u r a l c a s e s . T w o -th ird s o f th e p o p u la tio n have b een ju d g ed t o be d e f i n i t e l y e d u o a b le , w ith i n t e l l i g e n c e ra n g in g from r e t a r d ed to superior. One-third have b een c o n s id e re d by th e same i n v e s t i g a t o r to b e h o p e le s s ly u n e d u c a b le . T ra in in g and r e h a b i l i t a t i o n a re b e in g fo c u s e d p r im a r il y on th e f i r s t g ro u p . C a u se s. P e r l s t e i n l o c a t e s c e r e b r a l p a ls y among th e t h r e e m a jo r ty p e s o f n e u ro g e n ic p a l s y . T h e th r e e a r e : ^ C h ild re n W ith C e re b ra l P a ls y (C h ic ag o : I l l i n o i s Com m ission f o r H andicapped C h ild re n , 1 9 4 3 ), p . 9 . ^W inthrop M. P h e lp s and T. A rth u r T u rn e r, The F a r t h e s t C orner (C h ic ag o : N a tio n a l S o c ie ty f o r C rip p le d C h ild re n and A d u lts , 1 9 4 5 ), p p . 6 -7 . Thomas M c ln tir e , " C e re b ra l P a ls y T re a tm en t E x p e rim e n t," The C rip p le d C h ild . 2 0 :9 4 -9 6 , 1 1 1 , D ecem ber, 1942. ll|^ e y e r A. P e r l s t e i n , The Problem o f C e re b ra l P a ls y Today (New Y ork: A s s o c ia tio n f o r th e A id o f C rip p le d C h ild re n . 1 9 4 7 ), p . 4 . 8 th e c e r e b r a l , th e s p i n a l , and th e p e r i p h e r a l n e rv e . The " c e r e b r a l" ty p e o f p a ls y d e s ig n a te s any " p a r a l y s i s , weak n e s s , in c o o r d in a tio n o r f u n c tio n a l a b e r r a t i o n o f th e m otor system r e s u l t i n g from a b r a in p a t h o l o g y . S p i n a l p a ls y i s a ls o d is tin g u is h e d by m otor in v o lv em en t b u t i s th e c o n se quence o f s p in a l c o rd d e g e n e ra tio n o r i n j u r y due to tra u m a s , d ev elo p m en tal d e f e c t s , o r i n f e c t i o n s . S p in a b i f i d a and p o lio m y e li tis a r e th e m ost common ex aajp les o f d ev elo p m en t a l d e f e c ts and i n f e c t i o n s , r e s p e c t i v e l y . I n j u r y t o th e m o to r n e rv e such a s a c c id e n ta l l e s i o n s o r le a d ip p is o n in g , w il l r e s u l t i n th e p e r ip h e r a l n e rv e p a l s y t ^ e . But in t h i s g ro u p , as in . th e s p in a l p a l s i e s , th e b r a i n rem a in s u n in ju r e d . B i r t h , p r e - n a t a l , o r p o s t - n a t a l c o n d itio n s may be r e s p o n s i b le f o r c e r e b r a l p a l s y . P e r l s t e i n e s tim a te s t h a t m ore th a n n in e ty p e r c e n t a r e due to p r e - n a t a l o r n a t a l f a c t o r s . A sphyxia o r a n o x ia , and c e r e b r a l hem orrhage a c c o u n t f o r m ost c e r e b r a l damage from th e tim e th e m o th er goes i n t o l a b o r u n t i l th e c h i ld i s c o m p le te ly d e l iv e r e d . l ^ P e r l s t e i n , lo o . c i t . ^^L oc. c i t . The b r a in i s v e ry s e n s i t i v e t o low oxygen in ta k e and i s e a s i l y damaged by la c k o f t h i s n e c e s s a ry e le m e n t. Thus a c h i ld who f o r any r e a s o n does n o t b r e a th e a f t e r b i r t h i s l i k e l y to s u f f e r b r a i n damage t h r o u ^ a n o x ia . Among th e c a u s e s o f such d e la y e d b r e a th in g m ig h t be d e l iv e r y of th e b re e c h b e f o r e th e h e a d , b lo c k a g e o f th e r e s p i r a t o r y p a s s a g e s by m ucus, c o lla p s e o f th e lu n g s ( a t e l e c t a s i s ) , r e l i e v i n g d ru g s such a s m orphine w hich may i n h i b i t r e s p i r a t i o n i n th e c h i l d .^ S ch reib e r^ ® p r e s e n te d c l i n i c a l d a ta to d e m o n stra te th e im p o rta n c e o f a n o x ia f o r c e r e b r a l p a l s y . P h elp s d e s c r ib e s th e la r g e p e rc e n ta g e o f c e r e b r a l hem orrhage i n j u r i e s in c id e n t to b i r t h ; T h is i s t r u e i n p re m a tu re i n f a n t s who a r e b o rn p r e c i p i t o u s l y as w e ll a s i n l a r g e f u l l te rm i n f a n t s who a r e d e liv e r e d w ith d i f f i c u l t y . The b lo o d v e s s e ls o f th e b r a i n i n th e p re m a tu re in f a n t a r e more r e a d i l y b ro k en th a n th e y a r e i n f u l l te rm i n f a n t s and t h e i r r u p tu r e may r e s u l t from to o r a p id dro p from an e x tre m e ly h ig h i n t r a u t e r i n e p r e s s u r e to a tm o sp h e ric p r e s s u r e d u rin g a p r e c i p i t a t e d e l iv e r y a s w e ll as from a c t u a l t r a u m a . . . . . . M ech an ical I n ju r y may be due to i n c o r r e c t l y a p p lie d f o r c e p s , r e s u l t i n g in f r a c t u r e o f th e s k u l l and hem orrhage on th e s u rfa c e o f th e b r a i n , and i t may a l s o b e due t o th e f a i l u r e to u se f o r c e p s i n p r o t r a c t e d and d i f f i c u l t la b o r s . E x c e s s iv e t r a c t i o n on th e n eck may a l s o be r e s p o n s ib le f o r i n t r a c r a n i a l hem orrhage due to r u p tu r e o f th e v e in o f G a le n . l ^ I b id . . p . 5 . ^ ^ P re d e ric k S c h r e ib e r , " C e re b ra l A noxia and Anes t h e s i a , " J o u r n a l o f th e M ichipian S ta te M ed ical S o c ie ty . 3 8 :1 0 S Ô rloS è, D ecem ber, 1 9^9. ^^W inthrop M. P h e lp s , "The C e re b ra l P a l s i e s , " M itc h e l l - N elson T ex tb o o k o f P e d i a t r i c s . F o u rth e d i t i o n , Waldo E. N elso n , e d i t o r ( P h ila d e lp h ia : W . B. S au n d ers Company, 1 9 4 5 ), p . 112. 10 The p e r io d o f f o e t a l d evelopm ent from c o n c e p tio n t o d e l i v e r y i s f r a u ^ t w ith o p p o r tu n ity f o r b r a in damage* F a i l u r e f o r p o r tio n s o f th e c e n t r a l n e rv o u s sy stem t o dev e lo p may le a d to c e r e b r a l p a l s y * I n f e c t i o n s o r d is e a s e s i n th e m o th e r, f o r ex am p le, and e s p e c i a l l y th e to x e m ia s o f 18 p re g n a n c y , c a n e f f e c t c e r e b r a l damage* A f te r b i r t h , th e c a u s e s o f c e r e b r a l p a l s y seem t o o p e r a te l e s s f r e q u e n t ly . A cute i n f e c t i o n s su ch as m e n in g iti s , whooping cough, s c a r l e t f e v e r , and e n c e p h a l i t i s may g iv e r i s e to c e r e b r a l i n j u r y . T r a u m a t i c o c c u rre n c e s su ch a s s k u l l f r a c t u r e s and w ar i n j u r i e s may be c a u s a t iv e . Any form of s u f f o c a tio n can h av e th e a n o x ic r e s u l t s p r e v io u s ly d e s c r ib e d . I n a d u l t s , b r a i n tu m o rs and v a s c u la r a c c id e n ts due t o c e r e b r a l h em o rrh ag e o r th ro m b o sis may r e s u l t i n p a r a l y s i s . The f a m i l i a r " s tr o k e " f a l l s i n t o t h i s ca te g o ry * Whooping cough and c o n v u ls io n s a r e s e c o n d a r ily d a n g e ro u s by s h e e r v io le n c e o f th e d is tu rb a n c e * ^ ^ p p . 1X 11-1112. C la re n c e H. Heyman, " C e r e b r a l P a ls y , A D is c u s s io n on th e E tio lo g y and S u r g ic a l T re a tm e n t," P h y s io th e ra p y R eview . 1 9 :1 8 3 -1 8 7 , J u ly -A u g u s t, 1939* . 19 H aro ld K. F a b e r, " C e re b ra l Damage i n I n f a n ts and i n C h ild re n : Some O b se rv a tio n s on I t s C auses and P o s s i b i l i t i e s o f I t s P r e v e n tio n ." A m erican J o u rn a l o f D is e a s e s o f C h ild r e n . 7 4 :1 - 9 , J u ly , 1947. ^^L oc. c i t . 11 The p a th o g e n e s is o f r h b lo o d f a c t o r in c o m p a t i b i l it y rtin s t h r o n g p r e - n a t a l , n a t a l , n e o - n a t a l, and p o s t - n a t a l p e r io d s . The s e v e re Ja u n d ic e o f e r y t h r o b l a s t o s i s f o e t a l i s w hich may damage th e b a s a l g a n g lia , th e k e r n ic te r u s o f th e l i t e r a t u r e , ^ ^ i s o b s e rv a b le a t b i r t h o r a few days a f t e r , and f o r a s much a s s i x to e ig h t weeks l a t e r . But th e p a th o lo g y i s p e c u l i a r l y dynam ic and p r o g r e s s iv e a l l th ro u g h th e p re g n a n c y . B i r th hem orrhage h a s b e e n lin k e d t o th e r h f a c t o r and to in a d e q u a te v ita m in T y p es. T h ere a r e no two c e r e b r a l p a l s i e d in d iv id u a ls # 1 0 a r e a l i k e i n c h a r a c te r o r d e g re e o f m o to r in v o lv e m e n t. But th e t o t a l c e r e b r a l p a ls y p o p u la tio n h a s b een d i f f e r e n t i a t e d i n t o f i v e c l i n i c a l c a t e g o r i e s : s p a s t i c i t y , a t h e t o s i s , r i g i d i t y , a t a x i a , and tre m o r. ^ ^ Ja c k M. D o c to r, ”H e r n ic te r u s : N e u ro lo g ic a l S e q u elae o f E r y t h r o b l a s t o s i s F e t a l i s , ” J o u r n a l o f P e d i a t r i c s , 2 7 :3 2 8 -3 2 9 , O c to b e r, 1945. ^^M artha F . D eonard, ”H em olytic D is e a s e o f th e Newborn: C l i n i c a l A n a ly s is o f F i f t y - F i v e G ases, W ith S p e c ia l R e fe re n c e to P a th o g e n e s is , P ro g n o s is , and T h erap y , ” J o u m a l o f P e d i a t r i c s . 2 7 :2 6 2 , S ep tem b er, 1945. ^ ^ P h e lp s , 0£. Git. . p. 1113. 12 E ach v a r i e s s i g n i f i c a n t l y from th e o th e r s in c l i n i c a l d y n am ics. Jo n e s h a s w r i t t e n : The f i r s t s te p i n th e c a re o f th e c e r e b r a l p a l s i e d p a t i e n t , a s w ith any p a t i e n t , i s as a c c u r a te a d ia g n o s is a s p o s s i b l e . T h is m eans more th a n c l a s s i f y i n g him as c e r e b r a l p a ls y b e c a u se th e m ethods o f tr e a tm e n t f o r th e s p a s t i c , th e a t h e t o i d , and th e a t a x i c (etc.) p a t i e n t a r e fu n d a m e n ta lly d i f f e r e n t . P h e lp s h a s r e p e a te d l y s t a t e d t h a t a c c u r a te e a r l y d ia g n o s is and ty p e c l a s s i f i c a t i o n a r e p r e lim in a r y and b a s ic to th e d e te r m in a tio n o f r e c o n s t r u c ti v e p ro c e d u re s m ost e f f e c t i v e i n th e d i f f e r e n t ty p e s# ^ ^ * The e a r l y symptoms a r e r e l a t i v e l y u n d i f f e r e n t i a t e d . The C h ild may a p p e a r s lu g g is h i n th e f i r s t few weeks o r seem e s p e c i a lly i r r i t a b l e . . D i f f i c u l t y i n f e e d in g , con v u ls io n s o r tw itc h in g 3 may be o b se rv e d , and r e c i p r o c a l k ic k in g may be a b s e n t. L a te r th e c h i l d may be u n a b le to h o ld h i s h e a d e r e c t o r s i t a lo n e . As he m a tu re s i n th e e a r l y m o n th s, th e symptoms become more d i f f e r e n t i a t e d and r e c o g n iz a b le . ^"^M argaret H. J o n e s , ”The C e re b ra l p a ls y C h i ld ,” A m erican J o u rn a l o f N u rs in g ..4 6 :4 6 5 , J u ly , 1946. ^^W inthrop M. P h e lp s , ”R ecen t S ig n i f ic a n t T ren d s i n th e C are o f C e re b ra l P a l s y ,” S o u th ern M ed ical J o u r n a l, o p . c i t . , p . 135. ”C e re b ra l B ir th I n j u r i e s : T h e ir O rtho p a e d ic C l a s s i f i c a t i o n and S u b seq u en t T re a tm e n t,” The J o u r n a l o f Bone and J o i n t S u rg e ry . 1 4 :7 7 3 , O c to b e r, i 932• 13 S p a s t i c i t y . G rin k e r d e s c r ib e s th e symptoms o f c e r e b r a l s p a s t i c d i p l e g i a : e x a g g e ra te d deep ten d o n r e f l e x e s , B a b in s k i, and o th e r p a t h o lo g ic a l r e f l e x e s ; p o s s i b ly a n k le and p e t e l l a r c lo n u s , and d e c re a s e d o r a b s e n t s u p e r f i c i a l h y p e r i r r i t a b l e to a l l s t i m u l i , r e a c tin g w ith m axim al con t r a c t i o n , and t h a t a s p a s t i c m u scle i n v a r ia b ly r e a c t s to s t r e t c h i n g o f i t s e l f o r i t s a n ta g o n is t w ith th e s t r e t c h r e f l e x . The t r u l y s p a s t i c m u scle i s n o t r i g i d b u t i s sim p ly h y p e r i r r i t a b l e and h y p e r c o n t r a c t i l e when s t i m u l a t e d . . . . . . I t i s an a b n o rm a lity o f re s p o n s e and n o t a c o n s ta n t c o n d itio n . The m ost common s tim u lu s a m u scle r e c e i v e s , a s id e from v o lu n ta r y s tim u la tio n by i t s n e r v e , i s th e s t r e t c h i t u n d e rg o e s . when i t s a n ta g o n is t i s v o l u n t a r i l y c o n tr a c te d and m o tio n i s p ro d u c e d i n th e j o i n t in th e o p p o s ite d i r e c t i o n . # ie n t h i s o c c u rs in th e norm al p e rs o n , th e m u scle r e l a x e s , a c c o rd in g to th e in n e r v a tio n th e o ry o f S h e r rin g to n , o r a t l e a s t i t rem a in s i d l e . The s p a s t i c m u sc le , how ever, n e i t h e r r e la x e s n o r re m a in s i d l e b u t c o n t r a c ts a n d . . , . . . b l o c k s th e a tte m p te d m o tio n . T h at t h i s i s a r e f l e x i s o b v io u s, s in c e th e same phenomenon o c c u rs as a r e s u l t o f p a s s iv e a s w e ll as o f a c t i v e m o tio n .^8 L id d e ll and S h e rrin g to n ,w e re th e f i r s t t o d e s c r ib e th e s t r e t c h - r e f l e x . ^ ^ But i t i s o f te n f o r g o tt e n by s tu d e n ts ^*^Roy R. G rin k e r. N eu ro lo g y ( t h i r d e d i t i o n ; S p r i n g f i e l d : C h a rle s C. Thomas Company, 1 9 4 3 ), p p . 876-77 ^^W inthrop M. P h e lp s , "Management o f th e C e re b ra l P a l s i e s , ” J o u rn a l o f th e A m erican M edical A s s o c ia tio n . 1 1 7 :1 6 2 2 , November 8 , 19411 -------------- G. T. L id d e ll and € . S. S h e r rin g to n , " R e fle x e s i n R esponse to S t r e t c h (M y o ta tic R e f le x e s ) , " P ro c e e d in g s o f th e R oyal S o c ie ty o f London. B 96:2 1 2 -2 4 2 ,,1 9 0 4 . 14 i n t h i s w ork t h a t S h e rrin g to n and h is a s s o c i a t e s w ere d is c u s s in g norm al a s w e ll a s a t y p i c a l f u n c tio n . S tr e tc h in g r e f l e x l y e l i c i t s m u sc u la r c o n t r a c tio n and t h i s a c c o u n ts f o r th e s u b s tra tu m o f m u sc u la r te n s io n W elch u n d e r lie s norm al p o s tu r e . I n th e s p a s t i c c h i l d , t h i s s p in a l s t r e t c h - r e f l e x i s e x a g g e ra te d , th e c h i ld h y p e r i r r i t a b l e o r p o s s e s s in g low th r e s h o ld s o f s e n s i t i v i t y t o s t i m u l i . S tu d y in g th e a c tio n p o t e n t i a l s o f s p a s t i c m u s c le s , H o e ffe r and Putnam o b se rv e d t h a t v o lu n ta r y movement i n a s p a s t i c m u scle was slo w , weak and s t i f f , w hereas p r o p r io c e p tiv e r e f l e x e s te n d e d t o be s tro n g and v ig o ro u s . Ho in n e r v a tio n was o b se rv e d i n th e r e s t i n g s p a s t i c m u sc le , t h a t i s , th e n o n -w o rk in g s p a s t i c m uscle i s a r e la x e d m u scle, They c o n c lu d e t h a t th e s t i f f n e s s o r s p a s t i c t o n i c i t y e x i s t s o n ly as a r e f l e x in n e rv a tio n .® ^ I t i s n o t u n u su a l t o f in d w eak, f l a c c i d m u sc les i n th e s p a s t i c c a s e s . H. W. Magoun and R uth R h in e s . S p a s t i c i t y ; The S tr e tc h - R e f le x and E x tra p y ra m id a l System s T S p r ln g f ie ld ; ( â ia rle s Ù. Thomas Ccanp an y , Ï 9 47 ) , p . 11* ® ^Paul F . A. H o e ffe r and T ra cy J . Putnam , " A c tio n o f P o t e n t i a l s o f M uscles i n S p a s tic C o n d itio n s ," .A rc h iv e s o f N eu ro lo g y and P s y c h ia tr y . 4 3 ;2 2 , J a n u a ry , 1940. ^^Jo h n P . P u lto n , A T extbook o f P h y sio lo g y ( P h il a d e lp h ia ; W. B. S au n d ers Company, 19491", p . 268. 15 A th e t o s is . The a t h e t o i d is c h a r a c t e r i z e d hy in v o l u n ta r y p u r p o s e le s s m ovem ents. The u n c o n tr o lle d m o tio n may be su p erim p o sed upon v o lu n ta ry m otor p e rfo rm a n c e b u t th e fu n d a m e n tal a b i l i t y f o r v o lu n ta r y m o tio n i s n o t d i s t u r b e d . The in c o o r d in a tio n r e s u l t i n g i s s e c o n d a ry . P h e lp s h a s d e s c r ib e d : . . . . . .a s e r i e s o f in v o lu n ta r y m o tio n s w hich a f f e c t one m u sc le a f t e r a n o th e r t h r o u ^ o u t th e e x t r e m iti e s w ith o u t ; any p a r t i c u l a r o rd e r o f c o n t r a c t i o n s . . . . . .m ay be r a p id o r slow and a r e e n t i r e l y beyond th e c o n t r o l o f th e i n d i v i d u a l . . . . . . I n s le e p th e s e m o tio n s d is a p p e a r e n t i r e l y an d when th e p a t i e n t i s e x tre m e ly s le e p y # th e y - a r e o f v e ry sm all a m p litu d e . They a l s o d e c re a s e i n fre q u e n c y w ith re la x a tio n .® ® Note th e in v e r s e r e l a t i o n s h i p b etw een a t h e t o i d move ment and r e l a x a t i o n . The a t h e t o i d w i l l o f t e n , h o w ev er, a tte m p t to c o n t r o l th e in v o lu n ta r y m ovem ents by v o lu n ta r y te n s io n in s t e a d o f r e l a x a t i o n . So h a b i t u a l d o es t h i s become t h a t th e gro u p h a s b ee n s u b d iv id e d i n t o th o s e p a t i e n t s who d e m o n stra te te n s io n c o n tr o l and th o s e who do n o t. They have b e e n c a lle d " te n s io n a t h e to i d s " and n o n - te n s io n a t h e t o i d s ," r e s p e c t i v e l y . The s ig n i f i c a n c e o f t h i s f o r th e d ia g n o s tic ia n h a s b ee n s t a t e d by P h e lp s ; A n o th er m arked c h a r a c t e r i s t i c o f th e a t h e t o i d i s h i s te n d e n c y t o overcom e a t h e t o i d m o tio n by v o lu n ta r y ^^W inthrop M. P h e lp s , " D is e a s e s o f th e Locom otor S y s t e m , P ra ctitio n er ^ s L ib r a r y o f M edicine and S u rg e ry . V o l. 1 2 , (New Y ork: D. A p p leto n Company, 1936T, p . 8 1 8 . 16 te n s io n o f th e e x tr e m ity a s a w h o le. T h is p ro d u c e s a r i g i d i t y in th e lim b w hich s tr o n g ly s u g g e s ts s p a s t i c i t y . I t i s f o r t h i s re a s o n t h a t i t i s o f te n d i f f i c u l t to d i s t i n g u i s h betw een th e a t h e to i d and th e t r u e " s p a s t i c . " On th e o th e r h a n d , i m i t a t i o n o f a movement i n th e f in g e r , o f th e s p a s t i c arm may s e t up a k in d o f " c h a in r e a c tio n " s e r i e s o f s t r e t c h - r e f l e x e s re s e m b lin g th e in v o l u n ta r y m o tio n o f th e a t h e t o i d . T h i s d ia g n o s tic p ro b lem i s u s u a lly r e s o lv e d by th e a b se n c e o f th e s t r e t c h - r e f l e x i n th e a th e to id .® ^ kn i n t e r e s t i n g th e o r y r e g a r d in g a t h e t o s i s seem s t o have i t s s o u rc e i n P h e lp s . He s u g g e s ts t h a t th e sq u irm in g o f th e e x tre m ity i s an in v o lu n ta r y a tte m p t t o m a in ta in a change i n p o s i t i o n . I f one g ro u p o f m u sc les f a i l s to accom p l i s h t h i s , a n o th e r w i l l a tte m p t i t . I f t h i s i s p re v e n te d by m e a n s-o f b r a c in g o r o th e r o b s t r u c t iv e a p p a r a tu s , o th e r m u sc les w i l l come i n t o p la y to b r in g ab o u t th e p a r t i c u l a r d i s t o r t i o n o f th e body c h a r a c t e r i s t i c o f t h a t in d iv id u a l^ s a t h e to s i s .® ” ^ ^ i n t h r o p M. P h e lp s , " B ir t h I n j u r i e s , " The P h y sio th e ra p y R eview . 1 7 :5 , J a n u a ry -F e b ru a ry , 1 9 3 7 .. ______ , "The T re atm en t o f th e C e re b ra l P a l s i e s , " C l i n i c s , ë : 9 8 1 -8 2 , D ecem ber, 1943. "The T re a tm e n t o f C e re b ra l P a l s y ," M ed ical R e c o rd . 1 5 2 :2 1 8 , S eptem ber 1 8 , 1940. ®*^Loc. c i t . 17 I n summary th e n , a t h e t o s i s i s n o t s t r i c t l y a p a r a l y s i s b u t a c o n s ta n t in v o lu n ta r y m o tio n o f th e m u s c le s . The c h i l d i s u n a b le to s to p th e s e unw anted m o tio n s i n h i s e x t r e m i t i e s , h i s f a c i a l e x p r e s s io n , l i p s , to n g u e , o r tr u n k . Burton® ^ h â s w e ll s t a t e d t h a t th e " a th e to i d i s l i t e r a l l y a p r is o n e r w ith in a fram ew ork o f c o n s ta n t unw anted m o tio n ." R i g i d i t y . R i g id i t y i s a m o to r d i f f i c u l t y due to lo s s o f e l a s t i c i t y w ith " le a d - p ip e " s t i f f n e s s a s th e lim b i s moved.®^ The le a d - p ip e c h a r a c te r i s th e m ost o b v io u s symptom. W ech sler^ ^ d e s c r ib e s a slo w i m i t a t i o n and c o n tin u a t i o n of v o lu n ta r y movement due t o sim u lta n e o u s in v o lv e ment o f a g o n is t and a n t a g o n i s t . He s t a t e s t h a t r i g i d i t y i s m a rk ed ly re d u c e d in s le e p . P h elp s* d e s c r i p t i o n in c lu d e s a c o n s ta n t h y p e r to n ic c o n d itio n o f th e m u scle u n a f f e c te d by e i t h e r m o ti cm o r r e s t . S t r e t c h - r e f l e x i s n o t p r e s e n t. ®®Mary L. B u rto n , "G e n e ra l I m p lic a tio n s o f C e re b ra l P a ls y ," J o u r n a l o f S o c ia l C asew ork. 2 8 :9 1 , M arch, 1947. ^^W inthrop M. P h e lp s . "R ecent S i g i i f i c a n t T ren d s i n th e Care o f C e r e b r a l P a ls y ," S o u th e rn M ed ical J o u r n a l. op» Git. . p . 1 3 6 . ^ ^ I s r a e l B. W e c h sle r, A T extbook o f C l i n i c a l N euro lo g y ( f i f t h e d i t i o n , r e v i s e d ; P h ila d e lp h ia : W . B. S au n d ers Company, 1 9 4 4 ), p . 592. 41 W inthrop M. P h e lp s , " S p a s tic P a r a l y s i s , " The C y c lo p a e d ia o f M e d icin e , S u rg e ry and S p e c i a l t i e s . X I. 1945, p . 209. --------------- 18 G rIn k e r * 3^^ N eu ro lo g y c i t e s an in c r e a s e i n m uscle to n e , u s u a lly norm al deep r e f l e x e s som etim es in c r e a s e d , u s u a lly ab d o m in al r e f l e x e s te n d in g to f a t i g u e r a p i d l y , c lo n u s o r B a b in sk i som etim es p r e s e n t, p o s s i b le cog -w h eel s ig n o r i n t e r m i t t e n t r e l a x a t i o n and c o n t r a c tio n d u rin g p a s s iv e m o tio n , g r e a t e r to n e i n f le x o r s th a n t e n s o r s , Putnam and Hoeffer*^® l i k e n r i g i d i t y to a c o n s ta n t tre m o r. The a m p litu d e o f tre m o r i s so f in e t h a t a c o n d itio n o f c o n s ta n t s t i f f n e s s o f th e j o i n t r e s u l t s . T h is i s due to a c o n s ta n t c o n t r a c tio n o f a n ta g o n is ts w hich m a in ta in s con s t a n t p a r t i a l f l e x i o n in th e lim b s . T rem or. A n o th er ty p e o f in v o lu n ta r y m o tio n s im ila r to a t h e t o s i s b u t im p o r ta n tly d i f f e r e n t c o n s t i t u t e th e tre m o r g ro u p . Trem or i s m a n ife s te d b y in v o lu n ta r y , r e c i p r o c a l , and r e g u l a r ly rh y th m ic c o n tr a c tio n s o f a n t a g o n is t i c m u scle g ro u p s The tre m o rs may b e o f la r g e o r sm a ll a m p litu d e and o f v a ry in g sp eed s b u t d i f f e r from th e a t h e to i d s i n t h e i r r e g u l a r i t y . The d i f f e r e n t sp eed s from j o i n t to j o i n t make th e d i f f e r e n t i a l d ia g n o s is m ore d i f f i c u l t s in c e th e re se m b la n c e to th e %loy R. G rin k e r. N eu ro lo g y ( t h i r d e d i t i o n ; S p rin g f i e l d : C h a rle s C. Thomas Company, 1 9 4 3 ), p p . 3 9 9 , 4 1 0 -1 1 . 43 T ra c y J . Putnam and P a u l A. P . H o e f fe r, "A c tio n P o t e n t i a l s o f M uscles i n R i g i d i t y and T re m o r," A rc h iv e s o f N eu ro lo g y and P s y c h ia tr y . 4 3 :7 0 4 -2 5 , A p r i l , 1940. 19 a t h e t o s i s i s s tro n g i n some c a s e s . T rem ors a r e o f two ty p e s : (a ) i n t e n t i o n tre m o r w hich o n ly a p p e a rs w ith v o lu n ta r y m o tio n and i n t e r f e r e s w ith a tte m p te d movement; (h ) n o n - in te n tio n tre m o r in w hich th e c o n t r a c tio n s a r e p r e s e n t a t a l l tim e s . N e i l s o n d e s c r i b e s f a m i l i a l tre m o rs , s e n i l e tr e m o r s , tre m o rs o f c h ro n ic a lc o h o lis m , tre m o r o f m u lt ip le s c l e r o s i s , tre m o r o f h y s t e r i a , o f c o c a in e i n t o x i c a t i o n , o f e n c e p h a l i t i s , o f g e n e r a l p a r e s i s , and many o t h e r s . P r a c t i c a l l y a l l tre m o rs c e a se d u rin g s le e p and a r e in c r e a s e d on e x c ite m e n t. A ta x ia . The in c o o r d in a tio n o f th e a fo re m e n tio n e d c e r e b r a l p a l s i e s i s a se c o n d a ry c o n d itio n r e s u l t i n g from s p e c i f i c m o to r d is t u r b a n c e s . The in c o o r d in a tio n o f a t a x i a i s , h o w ev er, p rim a ry . The p a t i e n t i s u n a b le t o " s t e e r th e j o i n t i n th e d i r e c t i o n i n w hich he w ish e s from th e v e ry s t a r t o f th e m o tio n . ^ ‘ % in th r o p M. P h e lp s , " B ir th I n j u r i e s , " The P h y sio th e ra p y R eview . 2 :6 , J a n u a ry -F e b ru a ry , 1 937. _ , " S p a s tic P a r a l y s i s , " The C y c lo p a e d ia o f M e d ic in e . S u rg e ry , and S p e c i a l t i e s , op* c i t . . p . 21 0 . 46 J . M. N e ils o n , A T extb o o k o f C l i n i c a l N eurology (New Y ork: P a u l B. H oeber, I n c . , 19411, p p . Ï 9 8 -9 . 47 I b i d . . p . 199. '^^W inthrop M. P h e lp s , " B ir th I n j u r i e s , " op. c l t .. p. 5. ■ 20 He la c k s p o s t u r a l s e n s e . H i s s e n s e o f h i s own body p o s i t i o n in sp a ce i s l o s t . D iz z in e s s and n a u se a a re n o t uncommon. O cu la r f i x a t i o n on c lo s e o b j e c t s may a ls o be u n c o m fo rta b le o r n a u s e a tin g . B alan c e o f th e tr u n k and b a la n c e b e tw e e n g r a v i t y and m u scle pow er a r e o f te n d e f e c t iv e.® ^ S p a s t i c i t y and te n s io n a r e a b s e n t and when th e 51 p a t i e n t i s a t r e s t th e r e i s no I n v o lu n ta r y m o tio n . T h is p rim a ry in c o o r d in a tio n i s h a r d ly a p a r a l y s i s i n th e u s u a l se n se b u t seem s to f i t in to th e c e r e b r a l p a ls y group b e c a u se th e l e s i o n i s s u p r a - c o r d a i. Combined o r Mixed T y p es. T here i s some d is a g r e e ment re g a rd in g th e fre q u e n c y o f c o m b in a tio n s o f th e f i v e m a jo r ty p e s . P h e lp s w ro te in 1936 t h a t m ixed form s w ere more common th a n th e p u re c o n d itio n . Z en tay r e p o r te d i n 1937 t h a t s p a s t i c s w ith a t h e t o s i s w ere o b se rv e d a t th e Y ale S ch o o l o f P h y s io lo g y and th a t A rn o ld L . G e s e ll and C a th e rin e S. A m atruda, D ev elo p m en tal D ia g n o s is ( New Y ork: P a u l B. H o eb er, I n c .,' 1 9 4 1 ), p . 1 98. ® ^W inthrop M. P h e lp s , "The C e re b ra l P a l s i e s , ” M itc h e ll-N e ls o n T ex tb o o k o f P e d i a t r i c s , op. c i t . . p . , 1112. 51 . " B ir th I n j u r i e s , " lo o , c i t . 52 _________ > " B ir th I n j u r i e s , " P ra c t i t i o n e r * s L ib r a r y o f M edicine and S u rg e ry . V o l. 9 (New Y ork: D. A p p leto n Company, 1 9 3 6 ), p p . 5 3 5 -5 7 . 21 C < j r m otor d e f e c ts n ee d n o t b e c l e a r - c u t . By 1938 P h e lp s h ad r e v is e d h i s o p in io n t o c o n s id e r them v e ry r a r e when c a r e f u l d ia g n o s is was conducted.® *^ He h a s r e t a i n e d t h i s p o s i t i o n i n r e g a r d t o t h i s b a s i c q u e s tio n a s r e v e a le d by p u b lic a t io n s i n 1940®® and a g a in i n 1946.®® I t w ould seem to t h i s i n v e s t i g a t o r t h a t l e s i o n s o f th e b r a i n a r e n o t n e c e s s a r i l y l i m i t e d to s in g l e a r e a s . But an a r t i f i c i a l s e l e c t i v i t y m ig h t be e s t a b l i s h e d i f th e more e x te n s iv e l e s i o n s w ere r e s u l t i n g i n d e a th f o r th o s e p a t i e n t s . N e u ro p a th o lo g y . VIhen th e s e p a t i e n t s a r e b ro u g h t t o a u to p s y , s p e c i f i c le s io n s o f th e c e n t r a l n e rv o u s sy stem a r e o f te n fo u n d . W ith i t s p e c u l i a r m otor p a t t e r n , e a c h p a ls y , ty p e m ig h t b e e x p e c te d t o h a v e c h a r a c t e r i s t i c , c o n s is te n t f o c i o f b r a in dam age. But th e l i t e r a t u r e shows much d i s ag reem en t c o n c e rn in g th e l o c a t i o n o f th e l e s i o n . P a u l J . Z e n ta y , "M otor D is tu rb a n c e s o f th e C e n tr a l N ervous System an d T h e ir S ig n if ic a n c e f o r S p e e c h ,” L ary n g o s cope . 4 2 :4 2 4 , Ju n e , 1 9 37. . ® ^W inthrop M. P h e lp s , "The Gare and T re a tm e n t o f C e re b ra l P a l s i e s , ” The J o u r n a l o f th e A m erican M ed ical A s s o c ia ti o n . 1 1 1 :2 , ^ u ly , 1938. ® ® . "T reatm en t o f C e re b ra l P a l s y , ” M ed ical R e co rd . 1 5 2 : 2 l é - é o ,. S eptem ber 1 8 , 1940. . " P re s e n t M ethods Aimed a t T o ta l C o n tro l o f th e C e re b ra l P a ls y P ro g ra m ,” P ro c e e d in g s o f th e C o n feren ce on th e C e re b ra l P a l s ie d C h ild (Los A n g e le s: C a l i f o r n i a S o c ie ty f o r C rip p le d C h ild re n , 1 9 4 6 ), p . 3 1 . 22 S p a s t i c i t y . P h e lp s a t t r i b u t e s s p a s t i c i t y to l e s i o n s o f th e c e r e b r a l c o r te x . I n 1945, w ith H u tc h in so n and L a n e to t, he w ro te t h a t s p a s t i c i t y was " c a u s e d by an anom aly, i n j u r y , o r d is e a s e o f th e m otor a r e a o f th e c o r te x o f th e b r a i n . I n th e T extb o o k o f P e d i a t r i c s , a p p e a rin g th e same y e a r , Phelps®® s t a t e d t h a t s p a s t i c i t y r e s u l t e d fro m damage t o th e p re -m o to r a r e a . A rea s i x , w ith damage t o th e m o to r a r e a p r o p e r . A rea f o u r , r e s u l t i n g i n f l a c c i d m u sc u la r c o n d itio n . S in c e th e damage i s o f te n m ore g e n e r a l th a n s p e c i f i c , i t i s n o t ■unusual t o f i n d b o th m u scle ty p e s e x i s t e n t i n th e same p a t i e n t . F ig u re 1 i s in c lu d e d h e r e f o r g ra p h ic r e f e r e n c e . I t i s a c y t o a r c h l t e c t u r a l c h a r t o f th e p r e - c e n t r a l m otor c o r te x . Tower®^ h a s d e m o n s tra te d t h a t i n t e r r u p t i o n o f th e p y ra m id a l p r o j e c t i o n o f c o r t i c a l A rea f o u r , th e m o to r a r e a . 57 E s th e r H u tc h in so n , E liz a b e th H. L a n c to t, w ith W inthrop M. P h e lp s , Handbook on P h y s ic a l T h erap y f o r C e re b ra l P a ls y (C olum bus: Ohio S o c ie ty f o r C rip p le d C h ild re n , 1 9 4 7 ), p . 7 . ®®Winthrop M. P h e lp s , "The C e re b ra l P a l s i e s , " M itc h e l1 - N elso n T ex tb o o k o f P e d i a t r i c s . Waldo E . N e lso n , e d i t o r ( P h i l a d e i p h i a : W7 B. S au n d ers Company, 1 9 4 5 ), p . 1112. 59 S a ra h S. Tow er, "P y ra m id a l L e sio n i n th e M onkey," B r a in . 6 3 :3 6 -9 0 , 1940. x j* XO WH Ë â | | g > m EH 24 d o es n o t r e s u l t i n s p a s t i c i t y * P u lto n and h i s a s s o c i a t e s '^ o b ta in e d th e same r e s u l t s when th e y e x t i r p a t e d p o r tio n s o f A rea f o u r i t s e l f . The argum ent t h a t th e m echanism o f s p a s t i c i t y i s e x tra p y ra m id a l i s t h u s , b y e x c lu s io n , s u p p o rte d . When P ulto n ^^» ® ^ e x te n d e d th e a b l a t i o n o f A rea f o u r fo rw a rd t o in c lu d e A rea s i x , s p a s t i c i t y was added t o th e p a r a l y s i s syndrom e. When H in es^^ th e n c a r e f u l l y d e lim ite d a sm a ll s t r i p o f c o r te x , b etw een A rea f o u r and A rea s i x , f o r e x t i r p a t i o n , symptoms o f s p a s t i c i t y a lo n e r e s u l t e d . T h is sm a ll a r e a i s n o te d a s 4s i n F ig u re 1 . Magoun and R hines® ^ b u i l d an e x p e rim e n ta l and s tro n g t h e o r e t i c a l c a se f o r some s p a s t i c i t y r e s u l t i n g from damage t o th e b u lb a r r e c t i c u l a r f o rm a tio n . They d e m o n stra te d i n t h i s l a t t e r s tr u c tu re -c o m p le x a p o w e rfu l i n h i b i t o r y m echanism . ^^Jo h n P . P u lto n , and M a rg a re t A. K en n ard , ^A S tudy o f F la c c id and. S p a s tic P a r a ly s e s P ro d u ced b y L e sio n s o f th e C e re b ra l C o rte x i n P r i m a t e s , Proceedings o f th e A s s o c ia tio n f o r R e se a rc h i n N ervous and M ental D is e a s e s . 1 3 :1 5 8 -2 1 0 , 1934. 61 John P . F u lto n , ” S p a s t i c i t y and th e F r o n ta l L o b es. A Review,** New E n g lan d J o u rn a l o f M e d ic in e . 2 1 7 :1 0 1 7 -1 0 2 4 , D ecem ber 2 3 , 1Ô37. ®^John P . F u lto n and M arg aret A. K en n ard , l o c . c i t . ^^M arion H in e s , **The * M otor ^ C o r te x ,” B u l l e t i n o f th e Johns H opkins H o s p i t a l. 6 0 :3 1 3 -3 3 6 , May, 1 9 37. H. W. Magoun and R uth R h in e s , ”An I n h i b i t o r y M echanism i n th e B u lb a r R e c ti c u la r Form ation.** J o u r n a l o f N e u ro p h y slo lo g y . 9 :1 6 5 -1 7 1 , May, 1946. 25 h as g a th e r e d e v id e n c e to in c lu d e th e c a n d a te n u c le u s and putam en, j o i n t l y th e s t r ia tu m , in th e sy stem o f i n h i b i t o r y m echanism s* S t r i a t a l a b l a t i o n s u b s e q u en t to c o r t i c a l l e s i o n s p ro d u c in g s p a s t i c i t y s tr o n g ly augm ents th e i n t e n s i t y o f t h i s s p a s t i c i t y * The a n t e r i o r lo b e o f th e c e re b e llu m was c o n s id e re d c a p a b le o f i n h i b i t i n g s p in a l s t r e t c h - r e f l e x e s a s e a r l y as 1922.®'^'®® I n g e n e r a l, th e s e i n v e s t i g a t i o n s seem to h av e i n d i c a te d t h a t s p a s t i c i t y w i l l a p p e a r when c e n t r a l i n h i b i t o r y i n f lu e n c e s w hich n o rm a lly re d u c e s p i n a l s t r e t c h - r e f l e x e s a r e im p aired * A th e to s is * L e sio n s o f th e b a s a l g a n g lia and e x tra - p y ra m id a l t r a c t s have b een c o n s id e r e d b y P h e lp s and P . A* M e t t l e r , ‘‘E x te n s iv e U n i l a t e r a l C e re b ra l Rem ovals i n th e P r i m a t e : . P h y s io lo g ic E f f e c t s and R e s u lta n t D e g e n e r a tio n ,” J o u r n a l of C om parative N eu ro lo g y . 7 9 :1 8 5 -2 4 5 . O c to b e r, 1943. _________ , “P h y s io lo g ic E f f e c ts o f B i l a t e r a l and S im u lta n eo u s F r o n ta l L e sio n s in th e P r im a te ,” J o u r n a l o f C o m p arativ e N eu ro lo g y . 8 1 :1 0 5 -1 3 4 , O c to b e r, 1944. ^'^P. B rem er, “ C o n trib u tio n a L*Etude de l a P h y s io lo g ie du C e r v e le t. La F u n c tio n I n h i b i t r i c e du P a le o - G e re b e llu m ,“ A rc h iv e s I n t e r n a t i o n a l l e s de P h y s io lo g ie . 1 9 :1 8 9 - 2 2 6 ,.A p r i l, 19227 68 D. D enny-Brow n, J . C. E c c le s , and E. G. T. L i d d e l l , “ O b se rv a tio n s on E l e c t r i c a l S tim u la tio n o f th e C e r e b e lla r C o r te x .” P ro c e e d in g s o f th e R oyal S o c ie ty o f London. B104: 5 1 8 -5 3 6 , M aÿ; 1§29. ---------------- — ------------- 26 t o b e th e b a s i s f o r a t h e t o i d m otor b eh av io r* A l e x a n d e r h a s d e m o n stra te d th e c o n g e n ita l ab n o rm al i t y o r a b se n c e o f th e b a s a l g a n g lia i n some o f h i s p a t i e n t s . The a t h e t o i d movem ents a r e n o t d i r e c t l y r e s u l t a n t from th e l e s i o n b u t a r e r a t h e r m o to r re s p o n s e s t o c o r t i c a l im p u lse s t h a t h av e b een r e l e a s e d from g a n g lio n ic r e s t r a i n t . The t h e o r e t i c a l dynam ics o f f e r e d h e r e t o e x p la in a t h e t o s i s i s re m a rk a b ly s im ila r , t o th e i n t e r p r e t a t i o n s o f P u lto n , M e ttle r , Magoun, and o th e r s o f t h e i r e x p e rim e n ta l d a ta on s p a s t i c i t y d e s c r ib e d i n th e p re v io u s s e c tio n * S tu d y o f th e r e s e a r c h t h a t u t i l i z e s th e te c h n iq u e s o f e x p e rim e n ta l l e s i o n a r e a g a in fo u n d f r u i t f u l * I n g e n e r a l , “ i s o l a t e d le s i o n s o f an y one o f th e b a s a l g a n g lia in th e ^ ^ E s th e r H u tc h in so n , E liz a b e th H. L a n c to t, w ith W in th ro p M * P h e lp s * o p . c i t . . p . 8* ’ ^ % lin th ro p M. P h e lp s , “The C e re b ra l P a l s i e s , ” p p , c i t . , pp. 1 1 1 1 -1 1 1 2 . , . 71 B e rn e ic e R. R u th e r fo r d , G ive Them A Chance t o T a lk (M in n e a p o lis ; B u rg ess P u b lis h in g ôoïàpiany, 1 9 4 8 ), p . 1 . ^^Leo A le x a n d e r, “The F u n d am en tal Types o f ”H is to p a th o lo g ic Changes E n c o u n te re d i n C ases o f A th e to s is and P a r a ly s i s A g ita n s , “ P ro c e e d in g s o f th e A s s o c ia tio n f o r R e se a rc h i n N ervous and M ental D is e a s e s . 2 1 :3 3 4 -4 9 3 . D ecem ber, 1940. *^^Phelps, lo c * c l t . 27 a b se n c e o f th e i n j u r y t o th e c e r e b r a l c o r te x , g iv e l i t t l e i n th e way o f p o s i t i v e s y m p t o m a t o l o g y * R e s t r i c t i n g th e le s i o n t o th e c a u d a te n u c le u s i n two ch im p a n z ee s, Kennard*^^ o b se rv e d a m arked a t h e t o s i s s i m i l a r t o th e c l i n i c a l p i c t u r e i n hum ans. When she e f f e c t e d l a r g e r l e s i o n s i n any one o f th e b a s a l g a n g lia of h e r la b o r a to r y a n im a ls , e s p e c i a l l y when th e l e s i o n s w ere b i l a t e r a l , a sm a ll r e s t i n g tre m o r was o b se rv e d b u t l i t t l e t h a t c o u ld b e i d e n t i f i e d a s a t h e t o s i s . When l e s i o n s o f A rea s i x w ere fo u n d c o e x is ti n g w ith le s i o n s o f th e b a s a l g a n g l ia , a d e f i n i t e a t h e t o s i s accom p a n i e d .’ ^® Bucy*^*^ h a s r e c e n t l y p o in te d o u t t h a t a t h e t o s i s may b e e n c o u n te re d c l i n i c a l l y w ith l e s i o n s in e i t h e r th e s tr ia tu m (c a u d a te n u c le u s and p u t amen) , th e g lo b u s p a l l i d u s , o r th e th a la m u s . He c o n te n d s t h a t th e th a la m u s , s tr ia tu m and g lo b u s p a l l i d u s a r e p o r tio n s o f th e s u p p re s s o r c i r c u i t 74 John P . P u lto n , “C erebrum and B a s a l G a n g lia ,” XIV, H o w e ll*3 T ex tb o o k o f . P h y s io lo g y . e d i te d b y John P . P u lto n ( P h i l a d e lp h i a ; W . B. S a u n d e rs, Company, 1 9 4 7 ), p . 291. 75 M arg a ret A* K en n ard , “E x p e rim e n ta l A n a ly s is o f th e F u n c tio n s o f th e B a sa l G a n g lia i n Monkeys and Chimpan z e e s , ” J o u m a l o f N e u ro p h y sio lo g y . 7 :1 3 8 , M arch, 1944. p p . 1 4 8 -5 . 77 P a u l C. B ucy, “R e la tio n to Abnorm al I n v o lu n ta r y M ovem ents,” XV, The P r e - C e n tr a l M otor C o r te x , e d i t e d by P a u l C. Bucy (U rb an a: U n iv e r s ity o f I l l i n o i s P r e s s , 1 9 4 4 ), p p . 3 9 5 -4 0 8 . 28 fro m A rea four* I n t e r r u p t i o n o f t h i s c i r c u i t a t any p o in t i n i t s c o u rs e r e l e a s e s th e m otor a r e a from norm al i n h i b i t o r y c o n t r o l . He even a d v o c a te s rem o v al o f th e p re m o to r a r e a i n c e r t a i n s e v e re a t h e t o i d c a s e s . R ig id ity * R i g i d i t y i s o f te n d e s c r ib e d a s t h e m o to r d is o r d e r f o llo w in g m u lt ip le p e t e c h i a l h em o rrh ag es w ith d i f f u s e in v o lv em en t o f th e b r a i n * E n c e p h a l i t i s i s a l s o c o n s id e re d by some th e u n d e rly in g c a u se f o r t h i s syndrome.®® P ulton® ^ s t a t e s t h a t th e r i g i d i t y o f c e r e b r a l hem o rrh a g e i s due p r im a r il y t o th e i n t e r r u p t i o n of e x t r a - p y ra m id a l p r o j e c ti o n s from th e c e r e b r a l c o r te x . The r i g i d i t y i s m ark ed ly s i m i l a r to t h a t o f d e c e r e b r a te d l a b o r a t o r y an im als* The r i g i d i t y seems to be an a d d i t i v e phenom enon, i n t e n s i f y i n g a s th e num ber o f i n t e r r u p t e d op d e s c e n d in g p ath w ay s i s I n c r e a s e d . V io la E . C a rd w e ll, “The C e re b ra l P a ls i e d C h ild and H is C are i n th e Home,” (New Y ork: A s s o c ia tio n f o r th e A id o f C rip p le d C h i l d r e n ,,1 9 4 7 ), p . 1 7 . *^®Phelps, 0£ . c i t . . p. 1113. 80 E s th e r H u tc h in so n , E liz a b e t h H. L a n c to t, w ith P h e lp s , o p . c i t . . p . 9 . ®^John P . F u lto n , “D e c e re b ra te and D e c o r tic a te R i g i d i t y : The P o s tu r a l R e f l e x e s ,” V I I I , H ow ell*s T ex tb o o k o f P h y slo lo R V . op. c l t . , p. 188* . p . 19 1 . 29 K e n n a r d * i m p o r t a n t i n v e s t i g a t i o n s h av e r e v e a le d a m arked r i g i d i t y fo llo w in g a com bined l e s i o n o f th e s tr ia tu m and th e p re -m o to r c o rte x * The r i g i d i t y i s s i m i l a r t o t h a t o f c l i n i c a l c a s e s o f b a s a l g a n g lia in v o lv e m e n t, and more e a s i l y d i s c e r n i b l e and more e n d u rin g th a n th e r i g i d i t y f o llo w in g l e s i o n s o f A rea s i x a lo n e . P a r k in s o n ! s D is e a s e i s o f te n c h a r a c te r i z e d by r i g i d i t y . D is c u s s in g P a rk in s o n is m , N e ilson®^ c o n s id e r s th e e s s e n t i a l p a th o lo g ic anatom y to b e “a d e g e n e r a tio n o f th e p r o j e c t i o n f i b e r sy stem o f th e g lo b iu s p a l l i d u s w ith some c h a n g e s, b u t o f a m ild e r d e g r e e , i n th e s u b s t a n t i a m ig r a .” W echsler,® ® and B e s t and Taylor®® a r e i n e s s e n t i a l a g r e e m ent w ith N e ils o n . Putnam and Hoeffer®*^ se e b a s ic to n ic a c t i v i t y in r i g i d i t y m u sc le s a t r e s t r e f l e c t e d i n t h e i r m u scle p o te n t i a l i n v e s t i g a t i o n . They b e l ie v e t h i s a c t i v i t y t o b e r e f l e x 0% M a rg a re t A. K en n ard , l o c . c i t . ® % e ils o n , op. c i t . . p . 1 83. ® ® Israel S. W e c h sle r, A T ex tb o o k o f C l i n i c a l N eur o lo g y ( f o u r t h e d i t i o n , r e v i s e d : "phi1a d e lp h ia : W . V. S au n d ers Company, 1 9 3 9 ), p . 608. 86 C h a rle s H. B e st and Norman B. T a y lo r , The P h y s io l o g i c a l B a s is o f M ed ical P r a c tic e ( B a ltim o re : W illia m s and W ilk in s Company, 1 9 4 5 } , p. 87 8 . 87 T ra cy J . Putnam and P a u l P . A. H o e f fe r, “A c tio n P o t e n t i a l s o f M uscles i n R i g id i t y and T re m o r,” A rc h iv e s o f N eu ro lo g y a ^ P s y c h ia tr y . 4 3 :7 1 9 , A p r i l , 1940. -------- ------------ 30 o r i g i n a t e d a s w e ll a s c e n t r a l . I n m ost o f th e l i t e r a t u r e , t h e r e a r e s ta te m e n ts d e p lo rin g th e p a u c ity o f in fo rm a tio n r e g a r d in g th e p a th o lo g y o f r i g i d i t y * Walshe®® rem in d s u s t h a t n o t m ore th a n a g e n e r a tio n a g o , P ark in so n * s r i g i d i t y was c la s s e d w ith “f u n c t i o n a l ” d is e a s e s on t h i s a c c o u n t. P a r k in s o n ’ s D is e a s e i s a c h ro n ic d e g e n e r a tiv e p r o c e s s r a r e l y o c c u rrin g b e f o r e th e ag e o f f o r t y . The e x te n t to w hich one can g e n e r a liz e from P a rk in s o n to th e r i g i d i t y i n c h ild r e n i s open t o q u e s tio n . Much c l i n i c a l i n v e s t i g a t i o n i s s t i l l n eed ed to i l l u m i n a t e t h i s a r e a . T rem o rs. T rem ors a r e b e lie v e d to b e due t o i n t e r r u p tio n o f s u p p re s s o r c i r c u i t s , s im ila r t o t h a t d e s c r ib e d f o r a t h e t o s i s ab o v e. The p r e c i s e s t r u c t u r e s im p lic a te d h ave n o t y e t been a g re e d u p o n , b u t g lo b u s p a l l i d u s and su b - ■ A Q s t a n t i a n ig r a a r e m ost commonly c i t e d . N ote th e agreem en t w ith th e fo ci d e s c r ib e d f o r r i g i d i t y i n th e p r e v io u s s e c t i o n . Neilson®® f in d s c o u rs e tre m o rs in d is e a s e s o f th e ®®P. M. R. W alshe, D is e a s e s o f th e N ervous System ( f o u r t h e d i t i o n ; B a ltim o re : W illia m s and W ilk in s Company, 1 9 4 5 ), p . 190. ®®Bucy, p p . c i t . . p . 405. ® ® N eilson, o p . c i t . , p . 199. 31 p u t amen or g lo b u s p a l l i d u s , th e r e d n u c le u s o r b rac h iu m c o n ju n c t ivum . The r e l a t i o n s h i p o f tre m o r to r i g i d i t y i n c l i n i c a l syndrom es i s e a s i l y o b s e rv a b le i n P a r k in s o n ’ s D is e a s e o r P a r a ly s i s A g ita n s . N eilson® ^ n o te s t h a t th e g lo b u s p a l l i d u s s e ^ s more in v o lv e d vshen th e tre m o r i s m ore a p p a re n t; s u b s t a n t i a n i g r a seems m ore a f f e c t e d when t h e r i g i d i t y p re d o m in a te s . S tu d y in g a c t i o n p o t e n t i a l d a t a , Hoeffer®® n o te s th e g r e a t s i m i l a r i t y b etw een tre m o r an d r i g i d i t y so t h a t th e y a r e som etim es i n d i s t i n g u i s h a b l e . Of i n t e r e s t i s H u g h lin g s J a c k s o n ’ s d e s c r i p t io n of r i g i d i t y as “tre m o r r u n to g e t h e r ” and tre m o r a s “r i g i d i t y s p re a d t h i n . ”®^ A ta x ia . I t i s g e n e r a lly a g re e d t h a t th e g r o s s d i s c o n t i n u i t i e s o f movement c a l l e d “A ta x ia ” a r e th e f u n c tio n o f la r g e le s io n s o f th e c e re b e llu m .® ^ G esell® ® ® h b l d . , p . 1 8 3 , ® ^Pa«l F , A. H o e f fe r, " P h y s io lo g y o f M otor I n n e r - v a tio n i n th e D y s k in e s ia s ,” P ro c e e d in g s o f th e A s s o c ia tio n f o r R e se a rc h i n N ervous and M en tal 5 i se as e s f B a l t ^ o r e : W illia m s and W ilk in s don^pany, 1 9 4 2 ), p . 524. ®^James T a y lo r , e d i t o r . S e le c te d W ritin g s o f J . H u g h lin g s Ja ck so n (L ondon: H odder and S to u g h to n , 1 9 § 1 ), p . 221, ®^John P . F u lto n , “C erebrum and C e re b e llu m ,” H o w ell’ 3 T ex tb o o k o f P h y a lo lo R y . o p . c i t . . p . 299. Q C A rn o ld L. G e s e ll an d C a th e rin e S. A m atruda, D ev e lo p m en tal D ia g n o s is (Hew Y ork: P a u l B. H o eb er. I n c . . Ï 9 4 Ï ) , p . Ï 9 ë , --------- 32 n o te s t h a t i n some c e r e b e l l a r l e s i o n s , he f i n d s c e r e b e l l a r tr e m o r s , h y p e r to n ia , and nystagm us# E x p e rim e n ta l l e s i o n s o f f e r r e l a t e d d a ta # C om plete rem o v al o f th e c e re b e llu m i n la b o r a t o r y a n im a ls i s fa llo w e d b y th e e x p e c te d a t a x i c symptom com plex, w ith p e r io d ic moments o f r i g i d e x te n s io n o f th e lim bs# As tim e g o es o n , th e an im al g a in s some e q u ilib r iu m and v o l i t i o n a l movements b u t o f a h i ^ l y a t a x i c sort.® ® D ow ® *^ h a s shown t h a t s p e c i f i c a b l a ti o n o f th e f lo c c u lo n o d u la r lo b e c a u s e s e q u ilib r iu m d is tu r b a n c e s w ith o u t p o s t u r a l ch an g es o r d i f f i c u l t i e s i n v o l i t i o n a l movement* E x t i r p a t io n s l i m i t e d to th e p o s t e r i o r lo b e r e s u l t s i n th e s o - c a l l e d “ te r m in a l tre m o r” w hich becom es e x a g g e ra te d a s th e o b je c ti v e o f a p a r t i c u l a r move m ent i s reached#® ® A b la tio n o f th e e n t i r e a n t e r i o r lo b e i n a n im a ls o th e rw is e norm al g iv e s r i s e t o m arked e x te n s o r hypertonia*® ® I f th e a n t e r i o r lo b e i s a b la te d i n a d e c e r e b r a te d a n im a l. ® ^Pulton^ l o c # c i t . ®'^R# S. Dow, “E f f e c t s o f L e sio n s i n th e V e s tib u la r P a r t o f th e C e re b ellu m i n P r im a te s ,” A rc h iv e s o f N eu ro lo g y and P s y c h i a t r y . 4 0 :5 0 0 -5 2 0 , S ep te m b er, Ü956 * ® ® Fulton, “ Cerebrum and C e re b e llu m ,” o p . c i t . , p . 3 0 0 . O O O. J . Connor and W . J . Germ an, “F u n c tio n a l L o c a l i z a t i o n W ith in th e A n te r io r C e r e b e ll a r L o b e ,” T r a n s a c tio n s o f th e A m erican N e u ro lo g ic a l A s s o c ia tio n , 6 7 :1 8 1 -1 8 6 . 1^4X7“ ■ ---------------------------- ------------------------------ 33 th e r i g i d i t y i n c r e a s e s m a rk e d ly i n a l l f o u r e x tr e m itie s * The in c lu s io n o f th e c e r e b e l l a r l e s i o n i n th e c o n s id e r a tio n o f p o s s i b l e p a th o lo g y u n d e r ly in g s p a s t i c i t y h a s b e e n c i t e d e a r l i e r i n t h i s chapter*^® ® T reatm en t* The g o a l o f th e tr e a tm e n t p ro g ram i s to a t t a i n maximum r e h a b i l i t a t i o n f o r a s s i m i l a t i o n o f an in d e p e n d e n t i n d i v i d u a l i n t o s o c ie ty * S p e c if ic f u n c tio n a l o b je c t i v e s u s u a l l y in c lu d e t r a i n i n g f o r lo c o m o tio n , s e l f - h e l p , arm f u n c tio n , sp e e c h an d g e n e r a l a p p e a ra n c e * T re a tm e n t f o r an y one o f th e su b -g ro u p s o f c e r e b r a l p a ls y i s im p o r ta n tly d i f f e r e n t from t h a t u se d f o r any o th e r ty p e * P r o g n o s is , t h e r e f o r e , le a n s h e a v ily upon p ro p e r i n i t i a l d i f f e r e n t i a l d i a g n o s i s . M ajor tr e a tm e n t te c h n iq u e s in c lu d e : p h y s i c a l th e r a p y , o c c u p a tio n a l th e r a p y , sp eech th e r a p y , b ra c e th e r a p y , d ru g s , and su rg e ry * P s y c h o lo g ic a l c o u n s e lin g and g u id a n c e f o r p a r e n ts and p s y c h o th e ra p y f o r th e p a t i e n t a r e r a p i d l y g a in in g r e s p e c t a s a fm d a m e n ta l p a r t of th e s u c c e s s f u l ^® ®Reference on p . ^W inthrop M. P h e lp s , “The Management o f th e C e re b ra l P a l s i e s , ” J o u rn a l o f th e A m erican M ed ical A s s o c ia ti o n . 1 1 7 :1 6 2 2 , Novemb e r ,"Tl 941 . ^ ° % a r g a p e t H. J o n e s , "The C e re b ra l P a ls y C h ild ," A m erican J o u rn a l o f N u rs in g . 4 6 :4 , J u ly , 1946* program * 34 103 P h y sio th e ra p y * The s p e c i f i c o b je c ti v e s o f p h y s io th e ra p y a r e th e a c q u i s i t i o n by t h e p a t i e n t o f fu n d a m e n ta l m o tio n s , n o rm al b a la n c e b etw een a n t a g o n i s t s , a c c u ra c y o f m o tio n , d i f f e r e n t i a l r e l a x a t i o n , b a la n c e and c o o rd in a tio n * ^ ® ^ T re atm e n t f o r th e a t h e t o i d aim s t o f r e e n o rm al v o lu n ta r y m o tio n from th e in v o lu n ta r y m o tio n su p e rim p o se d . S in c e a t h e t o i d movement seem s t o d e c re a s e w ith r e l a x a t i o n , th e a t h e t o i d i s ta u g h t c o n s c io u s v o lu n ta r y r e l a x a t i o n , p r e f e r a b ly o f a norm al m u scle f i r s t . R e la x a tio n o f s p e c i f i c a t h e t o i d m u scle g ro u p s th a n fo llo w s and m o tio n from th e r e la x e d c o n d itio n i s learn ed .^® ® R e la x a tio n seems t o h e lp some s p a s t i c a b u t i s o n ly o f se c o n d a ry im p o rta n c e . The p rim a ry t r a i n i n g fo c u s i n s p a s t i c i t y i s m o tio n . The p h y s i o t h e r a p i s t f l e x e s and e x te n d s th e a f f e c t e d j o i n t i n a s m a ll r a d iu s o f m o tio n w hich w i l l n o t e l i c i t th e s t r e t c h - r e f l e x . G ra d u a lly th e ra n g e i s in c r e a s e d w ith c o n s ta n t r e p e t i t i o n w ith o u t th e s t r e t c h - 103 Mary L o u ise B u rto n , “G e n e ra l I m p lic a tio n s o f C e re b ra l P a ls y ," J o u r n a l o f S o c ia l C asew ork. 2 8 :9 4 . M arch. 1 9 47. ' ^®” % luth H ansen, "M uscle R e e d u c a tio n i n C e re b ra l P a ls y W ith T ra in in g o f P a r e n ts i n Home A d m in is tra tio n o f T re a tm e n t," (u n p u b lis h e d M aster * s T h e s i s . The U n iv e r s ity o f S o u th e rn C a l i f o r n i a , Los A n g e le s , 1 9 3 7 ), p . 45* ^ B u rto n , l o c . c i t . 35 r e f l e x , and th e c h i l d i s ta u g h t p a s s iv e l y how t o move “ th ro u g h ” th e s tre tc h -re fle x * ^ ® ® M otion i s slow a t th e s t a r t and th e n th e r a t e in c r e a s e d . When f l a c c i d m u sc les can b e i d e n t i f i e d m assage seems to in c r e a s e to n e and s t r e n g t h . B ut m assage o f s p a s t i c m u sc le s i s c o n tr a in d ic a t e d s in c e m u scle s t r e n g t h r a t h e r th a n m u scle w eakness i s th e rule.^® *^ A lth o u g h warm b a th s h av e b een u se d t o r e l a x th e a th e to id s ,!® ® h y d ro th e ra p y i s d is c o u ra g e d by P h e lp s and h i s s t u d e n t s . H is t h e o r e t i c a l argum ent i s t h a t m o tio n m ust be le a r n e d i n a “g r a v i t a t i o n a l f i e l d . “^®® B ut BrockwayÜ® r e p o r t s on th e u s e o f h y d ro th e ra p y a t th e Los A n g eles O rth o p a e d ic H o s p ita l. V a c h a ü ! r e p o r t s on swimming u se d a t !® ® Jones, l o c . c i t . 107pjj@ lpg, « C o r r e la tio n o f P h y s io th e ra p y and Occup a t i o n a l T h erap y i n . t h e T re atm e n t o f C e re b ra l P a l s y , ” o p . c i t ., p . 152. ^®®Veronica O’B r ie n , “T re atm en t o f C h ild re n W ith C e re b ra l P a l s y . ” New Y ork S ta te J o u r n a l o f M e d ic in e . 4 5 :1 5 4 9 , J u ly 1 5 , IM s Z ^°® P lielp s, l o c . c l t . ^ ^ ^ A lv ia Brockw ay, "The Problem o f th e S p a s tic C h i l d ,” J o u r n a l o f th e A m erican M ed ical A s s o c ia tio n , 106: 1 6 3 7 , M a y " 9 T " l 9 S 6 % ------------------------------------------------ ^ ^ ^ V ic to ria B. V acha, “Swimming a t S p a u ld in g ,” The P h y s io th e ra p y R eview . 1 8 :1 2 5 -3 0 , M ay-June, 1 9 3 8 . 36 th e S p atild in g S ch o o l a s a r e l a x a t l v e , and B aeth k e^ ^ ^ d e s c r ib e s g ro u p swiinming s e s s io n s w ith c e r e b r a l p a l s i e d c h ild re n # I n r i g i d i t y , r e l a x a t i o n and m o tio n seem t o b e o f e q u a l i m p o r t a n c e # B u t P h e lp s d o es n o t th in k t h a t p h y s io th e ra p y i s e f f e c t i v e w ith r î g i d i t i e s # ^ ^ ^ R e la x a tio n i s b a s ic i n some sy stem s o f tr e a tm e n t f o r tre m o r b u t a g a in P h e lp s b e l i e v e s i t in e ffe c tiv e # ^ ^ ® T ra in in g im p a ire d b a la n c e and c o o rd in a te d m ovem ents i s th e m a jo r em phasis w ith th e a t a x i c p a t i e n t . R e la x a tio n an d m o tio n a r e o n ly se co nda ry #** Th e d i f f i c u l t i e s a r e i n la c k o f q u ic k a d ju s tm e n t to an o f f - b a la n c e p o s i t i o n and la c k o f a p p r e c ia t io n o f th e h e lp o r h in d ra n c e o f g r a v i t y i n th e u s e o f an arm o r a l e g i n a g iv e n p o s i t ion# P h e lp s i n d i c a t e s t h a t tr e a tm e n t sh o u ld a s s i s t v o lu n ta r y m o to r c e n te r s t o ta k e o v e r th e f u n c tio n o f th e ce re b e llu m # ^ ^ % o ro th y E* B a e th k e , G roup T re atm en t f o r C e r e b r a l P a ls y C a s e s ,” The S a rg e n t Q u a r te r ly . 2 6 :1 1 , D ecem ber, 1940# ^^® Hanson, op# c i t . . p# 86# ^ ^ ^ P h e lp s , ”The C e re b ra l P a l s i e s , ” o p # c i t #. p# 1115# ^^^Loc# c i t # ^^®Hanson, lo c . c i t # 117 P h e lp s , ” C o r r e la tio n o f P h y s io th e ra p y and Occup a t i o n a l T h e ra p y i n th e T re a tm e n t o f C e re b ra l P a ls y ." op# P i t . , p . 1 6 5 . . \/lfinthrop M. P h e lp s , "The T re a tm e n t o f C e re b ra l P a ls y ," M ed ical R e c o rd . 1 5 2 :2 1 6 -2 2 0 , S eptem ber 1 8 , 1 9 4 0 . 37 C ard w ell o u t l i n e s a t y p i c a l program f o r th e s e v e re a t a x ie s I n s e v e re c a s e s t h i s te a c h in g w ould s t a r t w ith h e a d b a la n c e c o r r e c t io n i n th e s u p p o rte d s i t t i n g p o s i t i o n , go on t o s i t t i n g tr im k b a la n c e , fo llo w e d by knee s ta n d in g and f i n a l l y s ta n d in g b a la n c e # The ey es sh o u ld b e a llo w e d to a s s i s t a t f i r s t and th e n th e p a t i e n t i s b l in d f o ld e d and l e a r n s t o b a la n c e w ith o u t th e a id o f th e ey es i f p o s s ib le * ^ ^ ^ O c c u p a tio n a l T herapy# O c c u p a tio n a l th e r a p y i s an e x te n s io n o f p h y s io th e r a p y . When th e fu n d a m e n ta l m o tio n s can be p erfo rm e d w ith some a c c u ra c y , o c c u p a tio n a l th e ra p y i s in tr o d u c e d t o te a c h th e p a t i e n t v a r io u s s k i l l s , e s p e c i a l l y th o s e o f d a i l y liv in g # Much o f th e w ork i s c o n c e rn ed w ith th e s k i l l s t h a t make a n i n d i v i d u a l s e l f - s u f f i c i e n t # The m o tiv a tio n v a lu e of o c c u p a tio n a l th e ra p y f o r th e c e r e b r a l p a l s y c h i ld makes f o r b e t t e r a c c e p ta n c e o f p h y s io th e r a p e u tic m e a s u re s . The a c t i v i t i e s o f o c c u p a tio n a l th e r a p y may b e concern- ed w ith su c h b a s ic a c t s a s f e e d in g s e l f w ith k n i f e , f o r k and sp o o n , b u tto n in g and u n b u tto n in g c l o t h e s , and ty in g k n o ts . When th e c h i l d i s re a d y f o r m ore com plex s k i l l s , w r i t i n g , ty p in g , and w e a v in g , e t c . , a r e t a u g h t . The p a r e n ts 119 ''V io la E# O a rd w e ll, The C e re b ra l P a l s i e d C h ild and H is C are i n th e Home (Hew Y ork; A s s o c ia tio n f o r th e A id o f C rip p le d *3hiH r e n , Ï9 4 7 ) , p , 7 8 , 120h ^ E a b a t, ” S tu d ie s o f H eu ro m u scu lar D y s fu n c tio n , ” P u b lic H e a lth R e p o r ts . 5 9 :1 6 3 5 -1 6 5 0 , D ecem ber, 1 944, 38 a r e ta u g h t t o c o n s tr u c t fe e d in g ' t a b l e s , s p e c ia l c h a i r s , , t o y s , b u tto n in g b o a r d s , etc», t o f a c i l i t a t e t r a i n i n g a t home. W heeler and h e r a s s o c ia te s ^ ^ ^ d e s c r ib e g ro u p p la y , p u p p e t show s, and c r e a t i v e d ra m a tic s in c lu d e d i n th e occup a t i o n a l th e r a p y program f o r th e e m o tio n a l an d s o c i a l dev elo p m en t o f th e c h i l d . B ra c in g . The p u rp o s e o f b r a c e s i s to c o n t r o l m o tio n , < L _ c o r r e c t o r f o r e s t a l l d e f o r m iti e s , and t o o f f e r g e n e ra l s t a b i l i t y and s u p p o r t. B ra c in g may be u s e d w ith a t h e t o i d s f o r c o n t r o l o f in v o lu n ta r y m otion# P h e lp s w r i t e s : When th e b r a c e s a r e f i r s t a p p lie d , i t i s c h a r a c t e r i s t i c o f th e p a t i e n t t o f i g h t them i n v o l u n t a r i l y , b u t g ra d u a l l y u n d e r f u r t h e r t r a i n i n g i n v a r io u s form s o f r e l a x a t i o n , th e b r a c e s becom e t o l e r a t e d , and th e p a t i e n t i s w i l l i n g to a llo w th e m u sc le j o i n t c o m b in a tio n s t o c a r r y th fo u g h m o tio n s in th e m ore e x a c t and c o r r e c t p o s i t i o n f o r w a lk in g o r arm u s e .iH 4 A th e to id s a r e o b se rv e d w h ile te m p o r a r ily b ra c e d t o h e lp d e c id e w h eth e r o r th e p a e d ic s u r g e r y i s i n d i c a t e d . I f th e b r a c i n g , w hich s im u la te s th e e n d - r e s u l t o f s u r g e r y . ^ ^ ^ O a rd w e ll, o p . c i t . . p . 8 2 . ^ ^ ^ D o rls W h e ele r, e t a l . , ” O c c u p a tio n a l T h erap y and P h y s ic a l T h erapy f o r th e C e re b ra l P a ls i e d C h ild ." P u b lic W e lfa re i n I n d ia n a . 5 5 :1 0 -1 1 , 2 2 , Hovem ber, 1945* 123 B u rto n , o p . c i t . . p . 9 3 . 124vifinthrop M. P h e lp s , "The T re a tm en t o f th e C e re b ra l P a l s i e s , " C l i n i c s . 2 :9 8 4 , D ecem ber, 1943. 39 In d u c e s p o o r body p o s tu r e o r c o n t o r t i o n s , s u rg e ry i s con t r a i n d i c a t e d .^ ^ ^ W ith s p a S t i c 3 , b r a c e s a r e u s e d t o overcom e o r p r e v e n t arm and le g c o n t r a c tu r e s d e v e lo p e d b y th e c o n s ta n t s tim u la tio n o f th e s t r e t c h - r e f l e x . N ig h t b r a c in g i s o f te n c o n tin u e d o r u n d e rta k e n w ith out r e s t r i c t i n g th e c h i l d i n th e b r a c e d u rin g h i s d a y l ig h t h o u rs " t o m a in ta in c o r r e c t i o n and p r e v e n t f u r t h e r s h o r te n in g o f th e m u s c le s ."1^*^ S u rg e ry . T re a tm e n t te n d e n c ie s h a v e moved away from r a t h e r th a n to w ard s u rg e ry e x c e p t a s an a c c e s s o r y t o p h y s io th e ra p y . 1 2 8 ,1 2 9 ,1 3 0 whien s u r g e r y i s em p lo y ed , p r e lim in a r y d ia g n o s tic o b s e r v a tio n s m ust b e done w ith ex trem e c a re and 1 2 5 ^ c i t . J o n e s , l o c . c i t . 127 H ansen, o p . c i t . . p . 9 0 . ^^®John L . R u th e r f o r d , " S p a s tic P a r a l y s i s , " The P h y s io th e ra p y R eview . 1 9 ;3 2 9 , N overnber-D ecem ber, 1939. 129 W in th ro p M. P h e lp s , "The Management o f th e C e re b ra l P a l s i e s , " The J o u r n a l o f th e A m erican M ed ical A s s o c ia ti o n . 1 1 7 :162?,"” November 8 , Ï9 4 1 . 130 C la re n c e H. Heyman, " S u r g ic a l T re a tm e n t o f S p a s tic P a r a l y s i s . " S u rg e ry . G ynecology, and O b s t e t r i c s . 6 8 :7 9 2 , A p r i l , 1 9 39. ----- --------- ------------------------- 40 13T s p e c i f i c m u scle d e s c r i p t i o n m ust b e a c c u r a t e S u r g e r y seem s t o be more s u c c e s s f u l w ith s p a s t i c s and i s e s p e c i a l l y c o n c e rn e d w ith c o n t r o l o f c o n t r a c t u r e s . P h e lp sl® ^ l i s t s s u r g i c a l p ro c e d u re s f o r c e r e b r a l p a l s y t o in c lu d e te n d o n le n g th e n in g , te n d o n t r a n s p l a n t a t i o n (te n o to m y ), n e u re c to m y , and a r t h r o d e s i s . Where s p a s t i c i t y o f th e le g s i s ex trem e and se em in g ly beyond th e r a p y , s e c tio n in g o f th e s p in a l co rd p o s t e r i o r r o o t s i s som etim es d o n e. The S t o e f f e l o p e r a tio n i s u se d w ith c o n t r a c tu r e s and c o n s i s t s o f r e s e c t i o n o f a p o r ti o n o f th e m o to r n e rv e s u p p ly to an i s o l a t e d m u s c le .1®® The P o e r s te r o p e r a t io n , c u t t i n g some s e n s o ry r o o t s , aim s to rem ove e x c e s s a f f e r e n t s e n s o ry im p u lse s w h ich k eep up th e hyp e r t o n i c i t y . 1®^ S u rg e ry f o r th e a t h e t o i d i s an in f r e q u e n t p r e s c r i p t i o n . The in v o lu n ta r y m o tio n o f c e r t a i n m u sc le s w hich h a v e now b e e n re n d e re d I n a c t i v e b y s u rg e ry i s ta k e n up b y o th e r T . G reen an d L. J . M cD erm ott, " O p e ra tiv e T re atm en t o f C e re b ra l P a ls y o f S p a s tic T y p e ," The J o u r n a l o f th e A m erican M ed ical A s s o c ia ti o n . 1 1 8 ;4 3 4 ^ 4 4 0 , F e b ru a ry 7 , 1942. ^ ^ ^ i n t h r o p M. P h e lp s , M itc h e l 1 -N e lso n T ex tb o o k o f P e d i a t r i c s . e d i te d by Waldo E . N elso n ( P h i la d e l p h i a ; W. B. S au n d ers Company, 1 9 4 5 ), p . 1115. 133 R o b e rt V. P u n s te n and C a rm e lita C alderw ood, O rth o p a e d ic N u rsin g (St. L o u is : 0 . V. Mosby Company, 1 9 4 3 ), p . 4 6 9 . ^ ^ ^ I s r a e l S. W e c h s le r, A Texkbook o f C l i n i c a l N eur o lo g y ( f o u r th e d i t i o n , r e v i s e d ; P h i l a d e lp h i a ; W. B. S au n d ers Company, 1 9 3 9 ), p . 57 1 . 41 m u s c u la tu re re m a in in g a v a ila b le .^ ® ® A b la tio n o f th e m o to r c o r te x h a s b een done t o d e c re a s e th e im p u lse s g o in g to th e b a s a l g an g lia.^® ® But t h i s p ro c e d u re o f t e n r e s u l t s i n a f l a c c i d p a r a l y s i s o r a f l a c c i d i t y t h a t r e v e r t s t o a t h e t o s i s a f t e r a few y e a rs * time.^®*^ Putnam h a s a ls o s e c tio n e d th e l a t e r a l colum ns o f th e c e r v i c a l c o rd t o d im in is h in v o lu n ta r y m otion.^® ® But Jones^® 2 r e p o r t s t h a t le s s and l e s s s u r g e r y i s b e in g p r e s c r ib e d a s o a s e s a r e se en e a r l i e r and c o n t r a c tu r e s a r e p re v e n te d i n tim e . ^®®Winthrop M. P h e lp s , "T re a tm e n t o f C e re b ra l P a l s y , ” C l i n i c s . 2 :9 8 1 -9 9 1 , D ecem ber, 1 943. 136 P a u l C. B ucy, " C o r t i c a l E x t i r p a t i o n i n T re a tm e n t o f I n v o lu n ta r y M ovem ents," P ro c e e d in g s o f t h e A s s o c ia tio n f o r R e s e a rc h i n N ervous and M en tal D is e a s e s (B a ltim o re ; W illia m s and W ilk in s Company^ 1942 ) , XXI, p p . 5 5 1 -5 9 5 . 1 3 7 p h e ip s , l o c . c i t . 138^^ j . Putnam , " R e s u lts o f T re atm en t o f A th e to s is by S e c tio n o f th e E x tra -P y ra m id a l T r a c ts i n th e S p in a l C o rd ." A rc h iv e s o f N eu ro lo g y an d P s y c h ia tr y . 3 9 :2 6 8 -2 7 5 . February; 'ïg sô . — ----------- ^ 139 M a rg a re t H. J o n e s , "The C e re b ra l P a ls y C h ild ," A m erican J o u r n a l o f N u rs in g . 4 6 :4 . J u l y , 1 9 46. 42 A b la tio n o f A rea f o u r h a s b een recom m ended f o r r e l i e f o f P a rk in s o n t r e m o r a n d Putnam ^^^ does a p y ra m id a l t r a c t s e c tio n a t th e s p in a l c o rd f o r s i m il a r p u rp o s e . T h ere a r e no s u r g i c a l p ro c e d u re s c u r r e n t l y em ployed f o r tr e a tm e n t o f c e r e b e l l a r l e s i o n s ( A ta x ia ) . D ru g s. The u s e o f d ru g s i n t h e tr e a tm e n t p ro g ram i s u s u a lly f o r e s t a b l i s h i n g a c o n d itio n i n lahich o th e r a s p e c ts o f tr e a tm e n t , su c h a s p h y s io th e ra p y and sp e e c h th e r a p y , c a n be b e t t e r c o n d u c te d . P ro s tig m in e , w hich r e c e iv e d much e a r l y s e n s a ti o n a l p u b l i c i t y , h as b een shown to be m ost e f f e c t i v e w ith f i n e c o o r d in a te d movement r a t h e r th a n w ith la r g e m u sc le g ro u p s , and e f f e c t i v e w ith r i g i d i t y and some s p a s t l c s b u t n o t w ith th e o th e r ty p e s . S p eech i s r e p o r te d to be w e ll f a c i l i t a t e d a s i n th e u se o f c o r r e c t i v e b ra c e s f o r c o n tr a c tu r e s .^ ^ ® » 3.44 ^ ^ ^ Jo h n P . F u lto n , "C erebrum and B a s a l G a n g lia ," XXV, H o w e ll* a T ex tb o o k o f P h y s io lo g y , e d i te d b y John P . P u lto n ( P h i l a d e l p h i a ; W. B. S au n d ers Company, 1 9 4 7 ), p . 2 9 2 . J . Putnam , - "T re a tm e n t o f U n i l a t e r a l P a r a l y s i s A g ita n s by S e c tio n o f th e L a te r a l P y ra m id a l T r a c t ," A rc h iv e s o f N eu ro lo g y an d P s y c h i a t r y . 4 4 :9 5 0 -9 7 6 , Novem ber, 1940. 142 Mary L. B u rto n , " G e n e ra l I m p lic a tio n s o f C e re b ra l P a ls y ," J o u r n a l o f S o o ia l_C asew ork. 2 8 :9 2 , M arch, 1947. 143p^ N. J e p s o n , "The Use o f P ro s tig m in e i n th e T re a tm e n t of I n f a n t i l e C e re b ra l P a r a l y s i s , " J o u r n a l o f P e d i a t r i c s . 2 8 :6 5 -6 8 , J a n u a ry , 1946. ^^^H. J . S c h a u b e l, " P ro s tig m in e a s A d ju n c t i n th e T re a tm e n t o f C e re b ra l P a ls y ," P h y s io th e ra p y R eview . 2 4 :2 3 6 - 2 3 7 , November-D ecem ber, 1944. 43 D i l a n t i n , o f th e h y d a n to in g ro u p , l a u s e d t o c o n t r o l th e e p i l e p t i c s e iz u r e s o f some o f th e p a t i e n t s . As a r e l a x a n t , i t r e p la c e d p h é n o b a r b i ta l, t o w h ich o b je c tio n s h a d b e e n r a i s e d b e c a u s e o f t h e s e d a tiv e effe c t. T e n sio n a t h e t o i d s seem t o h av e b e e n r e la x e d w ith t h i s d ru g b u t i t d o es l i t t l e fo r s p a s t i c a o r r i g i d i t i e s . 3-^® The u s e o f m y an esin ( t o l s e r o l ) w ith a t h e t o i d s h a s b een r e p o r te d r e c e n t l y , w i t h some f a v o r a b le r e s u l t s , b u t i t i s to o e a r l y t o s t a t e c o n c lu s iv e ly i t s e ffe c ts . The u se o f c u r a r e i n a t h e t o i d s a s a r e l a x a n t h a s b ee n t r i e d w ith in c o n c lu s iv e r e s u l t s to d a t e . 3-'^'^ H yoscene i s u s e d o c c a s io n a lly w ith tre m o rs a s a r e l a x a n t • 3-48 G lu tam ic a c id h a s b e e n g iv e n much r e c e n t a t t e n t i o n a s a p o s s ib le s tim u la n t f o r th e " s lu g g is h c h i ld " and ch an g es h av e b een r e p o r te d i n i n t e l l i g e n c e s c o re s a f t e r i t s u s e . But m ost w o rk e rs f e e l t h a t th e e x p e rim e n ts w ere r a t h e r « T ^ e W in th ro p M. P h e lp s , "R ece n t S i g n i f i c a n t T re n d s i n th e C are o f C e re b ra l P a ls y ," S o u th e rn M ed ical J o u r n a l . 3 9 :1 3 8 , F e b ru a ry , 1 946. 3-^^Eric D e n h o ff, Raymond H olden and C a ro l S i l v e r , " E v a lu a tio n o f T o ls e r o l i n t h e T re atm en t o f C h ild re n w ith C e re b ra l P a ls y ," New E ng lan d J o u r n a l o f M e d ic in e . 2 4 1 :6 9 5 - 6 9 8 , N ovaaber 3 , Ï S -1 9 . ^^^A . H a to f f , " C u ra re ," M e d ica l R e co rd . 1 6 5 :2 2 7 -2 2 9 . A p r i l , 1 9 4 3 . . 3-^®Phelps, l o c . c i t . 44 u n c o n tr o lle d and t h a t c o n c lu sio n s a t t h i s tim e would be 1 4.Q p re m a tu re . Speech Therapy# The m otor d is c o o r d in a tio n o f c e re b ra l- p a ls y may b e found i n any of th e m uscle system s o f th e body# I t i s n o t u n u su a l to f i n d p o r tio n s o f th e speech mechanism u n a b le to p erfo rm speech f u n c tio n s w ith in th e fram ework o f s p a s t i c m u sc le s , in v o lu n ta r y m u sc le s , r i g i d m u sc le s , m uscles i n tre m o r, o r in a t a x i c c o n d itio n # The to n g u e , th e m u scles o f th e jaw , th e l i p s , th e t h r o a t , th e p a l a t e , th e diaphragm and o th e r p a r t s o f th e b r e a th in g mechanism may a l l be in v o lv e d . Methods i n speech th e ra p y have been v a r ie d , and, as Wepman^-^O h as w r i t t e n , many o f th e p ro c e d u re s have b een p r a c t i s e d on a t r i a l - a h d - e r r o r b a s is # A r a t i o n a l e f o r speech th e ra p y i n t h i s c l i n i c a l group h a s n o t b een e s t a b l i s h e d i n th e l i t e r a t u r e • R e la x a tio n seems to be a b a s ic p r e lim in a r y p ro c e d u re 1 4 9 p e rso n a l com m unication w ith D r. M argaret Jo n e s. 150 Jo sep h M. Wepman, "A Speech C o r r e c tio n T e c h n iq u e ,” The C rip p le d C h ild . 2 1 :7 5 , 8 3 -8 4 , O cto b e r, 1943. 45 f o r a l l ty p e s of c a s e s . 3 - ® 3 . , 152,153 B re a th in g problem s a re o f te n fo u n d ; some a r e u n a b le to s u s t a i n a d e q u a te d u r a tio n of e x h a la tio n f o r speech an d , i n s p a s t i c s e s p e c i a l l y , re v e r s e d b r e a th in g f o r speech i s se e n . B lu m en th al, Carl son, 3-®® Milward,^-®® Rutherford, 3-®*^ Perlstein and Shere,3-®2 Edm und Ja c o b se n , Proiairessive R e la x a tio n (C hicago: U n iv e r s ity of C hicago P re ssé ÎL929 ) , 493 pp . 3*®^May W atrous N ile s , "Use o f G eneral Sem antic T echniques i n P h y s io th e ra p y ." P a p e rs from th e Second ^ e r i c a n Congress on G en eral Sem antic s . e d i t ed l)y M. Kendig {C hicago: I n s t i t u t e of G en eral S em an tics, 1 9 4 1 ), p . 185. ^®®paula P. E g e l, Technique o f T reatm ent f o r th e C e re b ra l P a ls y C h ild ( S t. L o u is: C. V. Mosby Company, 1 948), p p . 132-155. 3"®^dna B lu m en th al, "Speech C o rre c tio n f o r C e re b ra l P a ls y ," The P h y s io th e ra p y R eview . 2 1 :7 1 -7 2 , 1941. 3 * ® ®Earl C a rlso n , Born T hat Way (New Y ork: John Day Company, 1 9 4 1 ), p . 145. 3-®®E. j . M ilw ard, "S peech Therapy as a U n it i n th e P r o je c t f o r th e T reatm en t o f C e re b ra l P a ls y ," J o u r n a l o f O cc u p atio n a l Therapy and R e h a b i l i t a t i o n . 1 8 :7 9 -8 3 , A p r i l, 1^939 . B e rn e ic e R u th e r fo rd , Give Them a Chance t o T alk (M in n e a p o lis: B urgess P u b lis h in g Company, 1 9 4 8 ), p p . 4 9 -5 6 . ^^®Meyer A. P e r l s t e i n , and M arie S h e re , "Speech T herapy f o r C h ild re n W ith C e re b ra l P a ls y ," A m erican Journ& o f D is e a s e s o f C h ild r e n . 7 2 :3 8 9 -3 9 8 , O cto b e r, 1946. 46 Zentay,3.59 and Pittinger^-® ® a l l o f f e r d e v is e s f o r a t t a i n i n g c o n t r o ll e d b r e a th in g f o r sp e ech . Proeschels^-®^. a rg u es f o r s im u la te d and a c t u a l chewing to e x p l o it th e r e l a t i o n s h i p betw een speech and d i g e s t i v e m echanism . Wepman^*®^ o f f e r s an i n t e r e s t i n g p ro c e d u re which a tte m p ts to d i r e c t m u scu lar o v erflo w away from th e speech mechanism and tow ard d i s t a l p a r t s . Xn h i s c l i n i c a l p r a c t i c e , t h i s i n v e s t i g a t o r c o n s tr u c ts h i s speech th e ra p y program on two b a s ic fo u n d a tio n p o i n t s : (1) Speech i s an " o v e r la id " f u n c tio n , superim posed on a m u sc u la tu re o r i g i n a l l y in te n d e d f o r o th e r p u rp o s e s , l i f e p u rp o s e s . I t u t i l i z e s i n t h i s s e n s e , th e r e s p i r a t o r y system and a p o r tio n o f th e d i g e s t i v e sy stem . (2) Speech i s an e x p r e s s io n o f th e t o t a l o u tg o in g p e r s o n a l i t y . I t i s a f i f t h e x tr e m ity , a s i t w ere, re a c h in g o u t i n t o th e environm ent and s e n s i t i v e l y r e a c t i n g t o i t s e x p e rie n c e s . 159 P a u l J . S e n ta y , "M otor D is o rd e rs o f th e C e n tr a l Nervous System and T h e ir S ig n if ic a n c e f o r S p eech ," The L ary n g o sco p e. 4 7 :1 4 7 -1 5 6 , March, 1937. ^ ^ ^K ath erin e P i t t i n g e r , " S p a s tic Speech D is o rd e rs and T h e ir T re a tm e n t," The P h y sio th e ra p y R eview . 2 2 :1 3 8 -1 4 2 , M ay-June, 1942. A. P r o e s c h e ls , "A C o n trib u tio n t o th e P a th o lo g y and T herapy of D y s a r th r ia Due to C e r ta in C e re b ra l L e s io n s ," Jo u rn a l o f Speech D is o r d e r s . 8 :3 0 1 -3 2 1 . D ecem bæ . 1943. . ^ ^ ^ e p m a n , op. c i t . , p . 83. 47 B u ild in g on th e l a t t e r p re m is e , t h e r e f o r e , th e c h i ld m ust f in d speech f u n c t i o n a l , m ust f in d h i s s o c ie ty co m fo rt a b le and a c c e p tin g , and m ust d e s i r e to u se th e speech v e h ic le f o r com m unication w ith h i s community. I f th e c h i ld i s c o n s i s t e n t l y r e j e c t e d , i f h i s s o c ie ty does n o t a c c e p t him , i f h is a tte m p ts t o communicate w ith h i s community a re met w ith f r u s t r a t i o n , r e j e c t i o n , and f a i l u r e , th e c h i l d may c a u tio u s l y d e lim it t h i s r e l a t i o n s h i p . A cceptance of th e f i r s t p rem ise im p lie s t h a t good speech th e ra p y w i l l depend upon good k i n e s i o l o g i c a l p r i n c i p l e s . Speech th e ra p y w ith c e r e b r a l p a l s i e d c h i ld r e n needs t o be b a s e d upon th e d i f f e r e n t i a l d ia g n o s is , c o n sc io u sn e ss o f dynamic d if f e r e n c e s i n m uscle to n e , in m uscle f u n c tio n , th e p r i n c i p l e o f an tag o n ism , i r r i t a b i l i t y and h y p e r i r r i t a - b i i i t y , r e f l e x re s p o n s e to s t r e t c h , u n c o n tr o lle d su p e r imposed a t h e to i d and tre m o r movements, f l a c c i d m u sc le s , e t c . Speech th e ra p y m ight be d e fin e d b ro a d ly a s any m o d a lity t h a t w i l l a c h ie v e norm al f u n c tio n o f sp eech m u s c u la tu re f o r speech f u n c tio n , an d , s p e c i f i c t o c e r e b r a l p a l s y , any m o d a lity t h a t w i l l l i m i t , c o n t r o l , e l im in a te , o r circum vent th e d is o r d e r in g e f f e c t o f th e p e c u l i a r p ath o p h y sio lo g y # Speech th e ra p y m ust th e n be s e n s i t i v e t o d i f f e r e n t i a l d ia g n o s is and be dependent upon c a r e f u l and a c c u r a te m uscle t e s t i n g and d e s c r i p t i o n o f th e i n t r i n s i c and e x t r i n s i c m uscle system s of sp eech p ro d u c tio n # 48 U n less speech c o r r e c t i o n î s t s can h e ta u g h t b a s ic p h y s io l o g ic a l and k i n e s i o l o g i c a l p r i n c i p l e s , premium i s i n e v i t a b l y p la c e d upon t h e r e l a t i o n s h i p betw een th e sp eech t h e r a p i s t and th e p h y s i o t h e r a p i s t . The r e l a t i o n s h i p i s s i m i l a r t o t h a t o f th e p h y s i o t h e r a p i s t and th e o c c u p a tio n a l t h e r a p i s t . Speech can b e cco isid ered a m uscle s k i l l and th e r o l e o f the p h y s i o t h e r a p i s t becomes one of a s s i s t i n g th e c h i ld to th e s t a t e o f r e a d in e s s f o r th e more advanced m uscle s k i l l s of sp eech . The speech t h e r a p i s t i s m ost con ce rn e d w ith th e s e s k i l l s . A c h i ld p ro d u ces sp eech a c c o rd in g to th e way he h e a rs i t . I f th e c h i l d f a i l s t o h e a r some sounds o r h e a rs them d i s t o r t e d , h i s own speech r e p r o d u c tio n s w i l l r e f l e c t t h i s c o n d itio n . I n t a c t h e a rin g i n th e c h i ld w ith c e r e b r a l p a ls y i s im p o rta n t n o t o n ly f o r t h i s b a s ic r e a s o n , b u t b e c a u se th e c o r r e c tin g o f th e d e f e c t i v e speech i s c o n s id e r a b ly ham pered b y ,th e c h ild * s f a i l u r e t o h e a r a c c u r a te ly th e speech t h e r a p i s t * s exam ples o r h i s own i m i t a t i o n s . Superim posed upon th e speech c o r r e c t io n problem p r e s e n te d by s p a s t i c , f l a c c i d , or a t h e t o i d c o n d itio n s in th e speech musc u l a t u r e , d e f e c tiv e h e a rin g c o m p lic a te s an a lre a d y d i f f i c u l t problem * 49 I I . LITERATURE ON THE HEARING OP CEREBRAL PALSIED CHILDREN B ecause o f th e c r ip p li n g n a tu r e o f th e p a l s i e d con d i t i o n many o f th e c h ild r e n a r e u n ab le to r e a c t t o a u d ito r y s ti m u li as q u ic k ly o r a s e f f i c i e n t l y as norm al c h i ld r e n . I t i s n o t u n u s u a l, t h e r e f o r e , f o r many w orkers t o g e n e r a liz e from t h e i r im p re s sio n s and c o n s id e r many o f t h e i r more s e v e r e ly h an d icap p e d c h ild r e n a s d e a f c h i l d r e n . On th e o th e r han d , c h i l d r e n who f a i l to comprehend speech b ec au se o f h e a rin g d e f ic ie n c y a r e o f te n c o n s id e re d m e n ta lly d e f e c tiv e or b eh av i o r p ro b i ems . 3-® ® H earin g d e f e c ts h av e been s p e c i f i c a l l y c o n s id e re d c h a r a c t e r i s t i c o f th e a t h e t o i d C a r d w e l l s t a t e s t h a t "a b o u t tw en ty p e r c e n t o f th e a t h e t o i d s have some im p a ir ment o f t h e i r h e a r i n g . "3.®5 Rutherford^®® found f o r ty - o n e E . C a rd w e ll, The C e re b ra l P a l s i e d C h ild and His Care i n th e Home (New York: A s s o c ia tio n f o r th e Aid o f C rip p le d C h ild re n , 1 9 4 7 ), p . 28. 3"® W in th r o p M. P h e lp s , " D i f f e r e n t i a l C h a r a c t e r i s t i c s of S p a s t i c i t y and A th e to s is i n R e la tio n to T h e ra p e u tic M e asu res," New York S t a t e J o u r n a l o f M e d icin e. 4 1 :1 8 2 7 -3 1 , Septem ber 15, 1941. ^®®Cardwell, l o c . c i t . 3-®®Bernelce R. R u th e rfo rd , "H earin g Loss i n C e re b ra l P a ls i e d C h ild re n ," J o u rn a l o f Speech D is o r d e r s . 1 0 :2 3 7 -2 4 0 , S eptem ber, 1945. . 50 p e r cent of a l l of h e r c a s e s to have a h e a r in g lo s s and tw en ty -tw o and f i v e - t e n t h s p e r cent o f W o l f e s u b j e c t s had a h e a rin g l o s s f o r p u re to n e s . Of Wolfe * s tw en ty -tw o and f i v e - t e n t h s p e r c e n t, o n ly about f i v e p e r c e n t had a h e a rin g l o s s of a s e v e r i t y t o r e q u i r e s p e c ia l t r a i n i n g . U n fo r tu n a te ly , W olfe p r e s e n te d d a ta on o n ly s i x a t h e t o i d s , th e b u lk o f th e m a t e r i a l b e in g d e s c r i p t i v e o f s p a s t i c s ( t h i r t y - s i x s u b j e c t s ) . Along w ith H u tch in so n and L a n c to t, Phelps^®® i s even more s p e c i f i c i n a s c r i b i n g h e a r in g l o s s to th e "neck and arm" ty p e o f a t h e t o i d . In th e l i s t o f s u b -ty p e s o f a t h e t o i d s d is t in g u is h e d by P h e lp s ,3-® ^ th e "d e a f a t h e to i d " i s c o n s id e re d numerous enough to be ac co rd ed r e c o g n i t i o n 1 67 "W illiam W olfe, "A C l i n i c a l E v a lu a tio n o f F i f t y C e re b ra l P a ls i e d I n d i v i d u a l s ," (u n p u b lish e d D o cto r * s d i s s e r t a t i o n . S ta te U n iv e r s ity o f Iow a, Iowa C i t y , 1 9 4 7 ), p • 46 . 1 A Q E s th e r H u tch in so n , E liz a b e th H. L a n c to t, w ith W inthrop M. P h e lp s , Handbook on P h y s ic a l T herapy f o r C e re b ra l P a ls y (Columbus: Ohio S o c ie ty f o r C rip p le d C h ild re n , 1 9 4 6 ), p . 15. ^^^A s r e p o r te d to t h i s r e s e a r c h e r by D r. M argaret Jones fo llo w in g h e r c o n fe re n c e w ith D r. P h elp s a t th e m e etin g o f th e Am erican Academy o f C e re b ra l P a ls y , 1948: r e p o r te d as p e r s o n a l com m unication by J u l i e t t e M. G ra tk e , H elp Them Help Them selves ( D a lla s : Texas S o c ie ty f o r ü r ï p p ïe d C hiiïdren, 1 9 4 7 ), p . 26. 51 a s a s u b -ty p e . P h e lp s 3 -V O f^jp th er s t a t e s t h a t ad e n o id s n e a r th e e u s ta o h ia n tu b e e f f e c t i n g a m iddle e a r i n f e c t i o n may be th e cau se f o r t h i s d e a f n e s s . C h ild re n who a re l a t e in w alking and s i t t i n g , spending so much o f t h e i r tim e on th e i r b a c k s, a r e s a i d by P h e lp s to have more a d e n o id a l 171 grow th th a n norm al c h i l d r e n . W ith n u rs e s t e s t i n g th e h e a rin g a c u i t y o f c h ild r e n a t th e Bov/ling S ch o o l, R u th e rfo rd d id a more s y s te m a tic s tu d y of h e a rin g i n th e p o p u la tio n . D iv id in g h e r c l i n i c a l group in to two g ro u p s , th e p y ram id a l and e x tra p y ra m id a l, R u th e rfo rd found g r e a t in c id e n c e o f h e a rin g l o s s i n th e e x tra p y ra m id a l group w ith d e f e c tiv e speech th a n in th e p y ram id a l d e f e c tiv e sp eech g ro u p . But t h e r e was g r e a t e r in c id e n c e o f h e a rin g l o s s i n th e p y ra m id a l group when th e "good"speech in d iv i d u a ls were s tu d ie d . R u th e rfo rd * s s tu d y was n o ta b le i n a tte m p tin g t o d iv id e th e c e r e b r a l p a l s y group by p a th o lo g y . But i t i s n o t c l e a r from h e r d a ta how t h e a r b i t r a r y l i n e s o f ^ ^ ^ P h e lp s, " D i f f e r e n t i a l C h a r a c t e r i s t i c s o f S p a s t i c i t y and A th e to s is i n R e la tio n t o T h e ra p e u tic M e a su re s," l o c . c i t . 171 I n q u e s tio n and answ er p e r io d fo llo w in g l e c t u r e b y Dr. W inthrop M. P h elp s a t th e M eetings o f th e S t. L ouis and M isso u ri S o c i e t i e s f o r C rip p le d C h ild re n , December 2-3, 1945. loQ , c i t . 52 c le a v a g e i n gro u p in g "p y ram id al" and " e x tra p y ra m id a l," w ere e s t a b l i s h e d . The d i f f e r e n c e s betw een th e s e g roups w ere n o t t r e a t e d f o r s t a t i s t i c a l s ig n i f ic a n c e making i n t e r p r e t a t i o n d i f f i c u l t . CHAPTER I I I DESIGN AND PROCEDURE The demands o f th e r e s e a r c h were s e v e r e . A so u rce of s u b je c ts was n o t o n ly n e c e s s a ry b u t a d i f f e r e n t i a l d ia g n o s tic e x a m in a tio n of each s u b je c t by an e x p e rt in th e c e r e b r a l p a ls y f i e l d was a minimum n eed . In o rd e r to stu d y h e a rin g a c u i t y , in s tr u m e n ta tio n f o r m easurem ent o f a u d ito r y p erfo rm an ce w ith in c o n t r o l l e d a c o u s t i c a l e n v iro n ment was r e q u ir e d . An e a r , n o s e , and t h r o a t ex a m in a tio n o f each s u b je c t b y an o to la r y n g o l o g is t to augment th e a u d io m e tric d a ta f o r d ia g n o s tic p u rp o se s was a t h i r d m ajor r e q u ir e m e n t. The r e s e a r c h re q u ire m e n ts w ere met o n ly w ith th e a c tiv e i n t e r e s t and c o o p e ra tio n o f th e C h ild r e n ’ s H o s p ita l, Los A n g e le s, C a l i f o r n i a . The C h ild re n ’ s H o s p ita l was i d e a l and i t s c o o p e ra tio n most f o r t u n a t e i n o f f e r i n g a good so u rce of c e r e b r a l p a l s i e d c h ild r e n i n a m ajor d ia g n o s tic and tr e a tm e n t c e n t e r , a so u n d -p ro o f room a d a p ta b le f o r t h i s r e s e a r c h , and a D epartm ent o f O to la ry n g o lo g y f o r d ia g n o s tic o to lo g ic e x a m in a tio n , a l l c o n ta in e d in one b u i l d in g . No o th e r agency i n t h i s m e tr o p o lita n a r e a co u ld s a t i s f y a l l o f th e r e s e a r c h demands. The m a jo r c o o p e ra tin g d e p a rtm e n ts were O to la ry n g o lo g y 54 and C e re b ra l P a ls y . The O u tp a tie n t C lin ic s t a f f im m easurably a s s i s t e d th e sc h e d u lin g and r o u tin g o f th e s u b je c ts and t h e i r p a r e n t s . The N ursing S t a f f was e v e r p r e s e n t and h e l p f u l i n making s u b je c ts and p a r e n ts co m fortable* I . SELECTION OF SUBJECTS The s u b je c ts were a random sam pling o f c e r e b r a l p a l s ie d in d iv id u a ls i n th e a c t i v e f i l e s o f th e S ta te C e re b ra l P a ls y D ia g n o s tic and T reatm en t C lin ic a t th e C h i ld r e n ’ s H o s p ita l O u tp a tie n t D epartm ent, Los A n g eles, C a l i f o r n i a . A c a rd f i l e in d e x was s e t u p , l i s t i n g a l l c e r e b r a l p a l s i e d i n d i v i d u a l s v i s i t i n g th e c l i n i c f o r e x a m in a tio n , r e - exam i n â t io n , o r tr e a t m e n t . The i n v e s t i g a t i o n had t o b e conducted w ith in th e l i m i t a t i o n s o f h o s p i t a l p o l i c y . Only th o s e c h i ld r e n c u r r e n t l y v i s i t i n g on an e s t a b l i s h e d sc h e d u le and e n r o lle d i n th e s t a t e c e r e b r a l p a l s y program c o u ld be in c lu d e d . T ra n s p o r ta t i o n had to be a v a ila b le w ith no undue h a r d s h ip i n t h i s r e g a r d . D is ta n c e s from th e h o s p i t a l were c a r e f u l l y c o n s id e r ed and t r a v e l l i n g tim e o f a p p ro x im a te ly one h o u r i n one d i r e c t i o n was a r b i t r a r i l y s e t a s a l i m i t i n g s e l e c t i o n f a c t o r . Use of a v a i l a b l e sp a ce and th e a s s i s t a n c e o f n u rs e s was r e s t r i c t e d t o T uesday, Wednesday-, and T hursday f o r each week of a fo u r-m o n th p e r io d . No s u b je c t c o u ld be seen f o r 55 ex a m in a tio n and t e s t i n g more th a n f o u r m onths a f t e r th e f i r s t t e s t i n g d a te due t o th e expense t o th e h o s p i t a l i n sp a c e , tim e , and p e r s o n n e l. The s u b je c ts w ere seen i n a l p h a b e t i c a l o r d e r . A form l e t t e r was devised^-V® and was m a ile d t o th e s u b j e c t ’ s p a r e n ts two weeks b e f o r e th e d a te of th e ap p o in tm e n t. The w ording o f th e l e t t e r had to be b r i e f and sim ple enough to be u n d e rs to o d by a l l p a r e n ts r e c e iv in g i t . A pproval o f th e l e t t e r was r e q u ir e d of th e d i r e c t o r o f th e O u tp a tie n t C lin ic s e r v i c e s , s in c e i t b o re h e r o f f i c i a l s ig n a tu r e and th e name o f th e h o s p i t a l . I I . CEREBRAL PALSY EXAMINATION Each s u b je c t was g iv e n a g e n e ra l p h y s ic a l ex a m in a tio n and n eu ro m u scu lar e v a lu a t io n . A ll e x a m in a tio n s were done by D r. M argaret Jo n e s, M edical D ir e c to r ^ o f th e S ta te C e re b ra l P a ls y D ia g n o s tic and T reatm en t C e n t e r . 3-V4 D i f f e r e n t i a l d ia g n o s is was made a t t h i s tim e . 3-V®see A ppendix, p . 235 174 D r. Jones i s a member o f th e A m erican Academy o f C e re b ra l P a ls y , a D iplom ate o f th e A m erican Board o f P e d i a t r i c s , and M edical D i r e c t o r of th e School f o r C e re b ra l P a ls y C h ild re n , A lta d e n a , C a l i f o r n i a , i n a d d itio n t o h o ld in g th e d i r e c t o r s h i p o f t h e s t a t e c l i n i c . 56 A m e d ic a l s o c i a l worker^-VS a s s i s t e d D r. Jo n es i n doing a p e d i a t r i c s tu d y o f each p a t i e n t . A p e d i a t r i c h i s t o r y was ta k e n from th e m other o f each s u b je c t# T h is in c lu d e d p r e - n a t a l d a t a , b i r t h h i s t o r y , and th e d is e a s e , a c c id e n t, and d e v e lo p m e n ta l h i s t o r y o f each s u b j e c t . W herever n e c e s s a r y , r e f e r r a l f o r o rth o p a e d ic con s u l t a t i o n r e g a r d in g b ra c e s or s u rg e r y was made. R e f e r r a l f o r p s y c h o lo g ic a l, ey e , e le c tr o e n c e p h a lo g r a p h ic , e p i l e p t i c , and o th e r e x a m in a tio n s w ere made t o th e a p p r o p r ia te c l i n i c . R ecords o f th e s e e x a m in a tio n s were k ep t i n th e p a t i e n t ’ s h o s p i t a l f i l e . I I I . OTORHINOLARYNGOLOGICAL EXAMINATION An e a r , n o se , and t h r o a t e x a m in a tio n was done by th e r e s i d e n t otolaryngologist^-V ® of th e h o s p i t a l . The p u rp o se of t h i s e x a m in a tio n was to e v a lu a te th e c o n d itio n o f h e a l t h o f th e e a r , t o h e lp l o c a t e th e fo c u s o f any e x i s t e n t p a th o lo g y , and to m e d ic a te w here r e q u ir e d . The n o s e , t h r o a t and e a rs were exam ined and th e s e d a ta were 175 M rs. E liz a b e th O lson, m e d ical s o c i a l w orker and s p e c i a l i s t in c e r e b r a l p alsy * 3-V ® a11 of th e o to r h in o la r y n g o lo g ic a l e x a m in a tio n s w ere done b y D r. W arren L in e. A rrangem ents f o r t h i s ex a m in a tio n and f o r th e u se o f th e so u n d -p ro o f room were made by Dr. V ic to r G o o h ill o f th e D epartm ent o f O to la ry n g o lo g y . 57 augm ented by an o t o l o g i c a l h i s t o r y and d is e a s e d a ta ob ta i n e d from th e p a r e n t . The exam ining d o c to r was a s s i s t e d by a r e g i s t e r e d n u r s e . A m e d ic a l s e c r e t a r y k e p t ru n n in g re c o r d s of th e e x a m in a tio n . T hese r e c o rd s were rev ie w e d and v e r i f i e d by t h e o t o l o g i s t . The form u se d f o r r e c o r d ing. th e d a ta f o r th e e a r , n o s e , and t h r o a t e x a m in a tio n 3 77 can be seen i n th e A ppendix. IV. AUDIOMETRIC TEST ROOM AND TEST EQUIPMENT T e s t Room. The a u d io ra e tric t e s t i n g was acco m p lish ed i n a so u n d -p ro o fed cham ber, 11* x 7 * , w ith a c e i l i n g e ig h t f e e t above th e f l o o r . A c o u s ti-C e lo te x t i l e covered th e s u r f a c e of c e i l i n g and w a l l s , and a rug co v ered th e f l o o r . T here was m inim al r e v e r b e r a t i o n o f sounds o r i g i n a t in g w ith in th e cham ber. But a glass-w indow ed door allo w ed some tr a n s m is s io n o f n o se from th e busy c l i n i c c o r r i d o r . To e l im in a te t h i s i n t e r f e r e n c e , a t h r e e - q u a r t e r in c h f i r t e x b o a rd was n a i l e d to th e e n t i r e i n s i d e s u rfa c e o f th e d o o r. Heavy c u r ta in s were hung j u s t i n s i d e th e door and were moved i n t o p la c e when t e s t i n g was u n d er way. T h is c o n d itio n in g o f t h e room produced e x c e l l e n t so u n d -p ro o f c o n d itio n s , s u b j e c t i v e l y ev a lu ated * I n s t r u m e n ta tio n f o r o b je c tiv e m easurem ent of th e a c o u s t i c a l 177 See A ppendix, p . 236 58 c h a r a c t e r i s t i c s of th e room was n o t a v a i l a b l e to th e i n v e s t i g a t o r . E quipm ent. The t e s t s o f a c u i t y f o r p u re to n e s were done w ith an a u d io m e te r. Type 50-E , m a n u fa c tu re d by th e 178 Audio Development Company o f M in n e a p o lis, M innesota* A s p e c ia l t u r n t a b l e f o r re c o rd e d speech r e c e p tio n t e s t s was s e t up n e x t to th e a u d io m e te r, th e p ic k -u p c i r c u i t e d th ro u g h th e a u d io m e te r a m p lif ie r and e a rp h o n e s . T h is p e r m itte d r e g u l a t i o n of th e o u tp u t by a d ju stm e n t o f th e h e a r in g l o s s c o n t r o l c a l i b r a t e d in f i v e d e c ib e l s t e p s . The equipm ent th u s p ro v id e d an in d iv i d u a l sp e ech lo s s t e s t f o r each e a r. C a l i b r a t i o n . The two a u d io m e tric c i r c u i t s , p u re tone and re c o rd e d sp e e c h , were c a l i b r a t e d a t th e o u ts e t o f th e study* T h e ir a d ju s tm e n ts w ere checked a t th e s t a r t o f each w eek’ s a c t i v i t y a g a in s t th e known h e a r in g c u rv es and speech r e c e p tio n s c o re s o f th r e e i n d i v i d u a l s . The audiogram s and sp eech s c o re s s e c u re d d u rin g the ch eck in g p ro c e s s d id n o t i n any in s ta n c e d e v ia te more th a n f i v e d e c ib e ls from th e o r i g i n a l s c o r e s . 3.78qjjç 0^ th e au d io m eters a c c e p te d by th e C ouncil o f P h y s ic a l M edicine, Am erican M edical A s s o c ia tio n . 59 D uring th e c o u rse of th e i n v e s t i g a t i o n , f o r t y norm al in d iv i d u a ls were g iv en a i r c o n d u c tio n p u re to n e t e s t s . These were c h ild r e n and young p eo p le who w ere accompanying r e l a t i v e s to o th e r c l i n i c s , had no p r e s e n t p h y s ic a l com p l a i n t s , and had no p r e s e n t or r e c e n t c o ld s . S t a t i s t i c a l tr e a tm e n t of th e combined d a ta g a in e d from t h i s group i n d i c a t e d l i t t l e o r no d e v ia tio n from th e z e ro of th e a u d io m e te r. The mean audiogram f o r t h i s c a l i b r a t i o n g ro u p , w ith s ta n d a r d d e v i a ti o n from th e mean a t each fre q u e n c y i s shown i n F ig u re 10, i n th e A ppendix. On th e b a s i s o f th e s e c a l i b r a t i o n p ro c e d u re s , i t was f e l t t h a t th e equipm ent rem ain ed i n e s s e n t i a l c a l i b r a t i o n d u rin g th e p e rio d o f s tu d y . S p e c ia l C h a ir. When th e s u b je c ts were n o n -am b u la to ry th e y w ere w heeled in to th e t e s t room and exam ined s e a te d i n t h e i r own w h e e l-c h a ir s . When t h i s was n o t p o s s i b l e , th e c h i l d was c a r r ie d i n t o th e t e s t room by th e i n v e s t i g a t o r and s e a te d i n a c h a i r e s p e c i a l l y a d a p te d f o r t h i s i n v e s t i g a t io n . The s p e c ia l c h a ir was a m o d ifie d b ro a d b a se wooden a rm c h a ir. C ushions were p la c e d a c r o s s th e back o f th e c h a i r , to a h e ig h t above th e s u b j e c t ’ s h e a d , and w ere ta c k e d i n t o 179 See A ppendix, p . 239 60 p la c e . T h is a f f o r d e d a c o m fo rta b le , s t a b l e b a s e f o r each c h i ld w ith a minimum of s t r a i n , and made r e l a x a t i o n o f th e a t h e t o i d s im p le r. The in v o lu n ta r y m o tio n o f th e a t h e t o i d was cu sh io n ed , p r o te c tin g h i s body and e s p e c i a l l y h i s h ead from p h y s ic a l i n j u r y . In a d d i t i o n , a wooden box was ad a p ted f o r u se a s a f o o t s t o o l t o o f f e r more s t a b i l i t y . Sand b ag s were p la c e d a lo n g th e th ig h s o f th e a t h e t o i d to g iv e f u r t h e r s u p p o rt. V. TEST BATTERY AND TEST PROCEDURE A s e l e c t i o n o f t e s t s t h a t co u ld a c c u r a te ly d e s c r ib e th e c h a r a c t e r i s t i c s o f h e a rin g was an i n i t i a l problem . I t was s tr o n g ly f e l t t h a t th e t e s t i n g p e r io d be b r i e f as p o s s i b le s in c e f a t i g u e would te n d t o r a i s e a c u i t y th r e s h o ld s The tim e i n t e r v a l o p e ra te d th u s as a l i m i t i n g f a c t o r . A ir C onduction T h re sh o ld T e s t . A m easure o f t h r e sh o ld a c u ity f o r a i r cond u cted p u re to n e s was c o n s id e re d e s s e n t i a l f o r com parison o f r e s u l t s w ith th e norm al p o p u la t i o n . T h is m easure was d e s ig n a te d a s T e s t AC and was p erfo rm ed w ith th e ADC P ure Tone A udiom eter p r e v io u s ly d e s c r ib e d . The th r e s h o ld f o r each s u b je c t was d e te rm in e d , f o r each e a r s e p a r a t e l y , f o r th e f o llo w in g f i v e f r e q u e n c ie s : 256, 512, 1024, 2048, and 4096 c y c le s p e r second. The 61 procéder© o f a d m in is t r a tio n o f th e s e t e s t s and m ethod o f th r e s h o ld d e te r m in a tio n h av e been d e s c r ib e d i n d e t a i l by B u n c h , F o w l e r , N e w h a r t and Heger,^® ^ and o t h e r s . In e s s e n c e , th e fre q u e n c y was in tro d u c e d a t an i n t e n s i t y le v e l lo u d enough f o r th e s u b je c t t o h e a r w ith o u t d o u b t. The i n t e n s i t y was th e n d e c re a s e d i n te n d e c ib e l s te p s u n t i l th e s u b je c t d id n o t re sp o n d to th e s tim u lu s , im p ly in g an i n t e n s i t y b ^ o # th r e s h o l d . The stim u lu s i n t e n s i t y , was th e n in c r e a s e d i n f i v e d e c ib e l s te p s and v a r ie d i n f i v e d e c ib e l s te p s u n t i l th e lo w e s t i n t e n s i t y a t w hich th e s u b je c t re sp o n d e d to th e s tim u lu s f i f t y p e r c e n t of th e tim e was d e te rm in e d . T his i n t e n s i t y was re c o rd e d a s th e s u b je c t* s th r e s h o l d of h e a rin g i n t h a t e a r f o r th e fre q u e n c y t e s t e d . The same p ro c e d u re was fo llo w e d f o r each o f th e o th e r f r e q u e n c ie s i n each e a r . - The th r e s h o ld audiogram th u s c . Bunch, C l i n i c a l A udiom etry ( S t . L o u is: C. V. Mosby Company, 1 9 4 3 ), p p . 45-46. E. P . F o w ler, " T e s ts f o r H e a rin g ," X I, N elson*s Loose L eaf M edicine o f th e E a r * e d i te d by E . P. F o w le r, J^r. , (New Y ork: N elso n , 1 9 3 9 ), p . 388. 182 Horace Newhart and S c o tt N. R e g er, " S y lla b u s o f A udiom etric P ro c e d u re s i n th e A d m in is tra tio n o f a Program f o r th e C o n se rv a tio n o f H earing o f School C h ild re n ," Sup-plement t o th e T r a n s a c tio n s of th e A m erican Academy o f O phthalm ology and O to la ry n g o lo g y (Omaha: D ouglas P r i n t i n g Company, 1 9 4 5 ), p . 8 . 62 o b ta in e d was re c o rd e d f o r each e a r on an a p p r o p r ia te a u d io gram s h e e t. S ince, f a t i g u e c o n t r o l was d e s i r a b l e , th e m easurem ents o f th r e s h o ld f o r 128, 8192, and 11,384 c y c le s p e r second were a r b i t r a r i l y e x c lu d e d . The c o r r e l a t i o n betw een m easures a t 128 c . p . s . and 256 c . p . s . i s good, so t h a t in f e r e n c e s from th e f in d in g s a t 256 c y c le s m ight be a p p lie d to th e O ctave p o in t below i t . Bone C onduction T h re sh o ld T e s t . T h re sh o ld a c u ity f o r bone co n d u cted ( o s c i l l a t o r a p p lie d to m a sto id ) p u re to n e s t i m u l i was a ls o c o n s id e re d d e s i r a b l e . The r a t i o o f a i r co n d u c tio n to bone c o n d u c tio n a c u i t y c o n t r ib u t e s to th e d ia g n o s is of ty p e o f h e a r in g l o s s , t h a t i s , c o n d u c tiv e , p e r c e p tiv e , m ixed, etc. ^ ® 188 The fo c u s of ^^^Copy o f audiogram s h e e t i s i n A ppendix, p . 240 ^® ^W illis € . B e a s le y , " C o r r e la tio n Between H earin g Loss M easurem ents by A ir C onduction on E ig h t T o n e s," J o u rn a l o f The A c o u s tic a l S o c ie ty o f A m erica. 1 2 :1 0 4 -1 1 3 , J u ly , 1940. ^®®Dean M. L i e r l e and S c o tt H. R eger, " C o r r e la tio n s Between Bone and A ir C onduction A c u ity M easurem ents Over Wide F requency Ranges i n D i f f e r e n t Types o f H earing Im p a ir m e n ts ," The L ary n g o sco p e. 5 6 :1 8 7 -2 2 4 , May, 1946. ^^^Ben H. S e n tu r ia and A. R. Thea, "Bone C onduction i n A u d io m etry ," The L ary n g o sco p e. 5 2 :6 8 5 , S eptem ber, 1942. 187 George E. Shambaugh, "The I n t e r p r e t a t i o n o f Bone C onduction and O ther T e s ts o f H e a rin g ," A nals o f O to lo g y . R h in o lo g y . and L ary n g o lo g y . 4 5 :7 7 9 -7 9 7 , Sep te m b e r, 19^67 ^®®I. H. Jones and Vern 0* Khudson, "The D ia g n o sis o f H ea rin g Im p a irm e n t," The L aryngoscope. 4 5 :2 4 -4 7 . J a n u a ry . 1935. ------------------------- 63 p a th o lo g y w ould, i n t h i s way, be i n d i r e c t l y d e lim ite d * The fo llo w in g f r e q u e n c ie s were t e s t e d : 256, 512, 1024, 2048, and 4096 c y c le s p e r seco n d . The bone c o n d u c tio n t e s t , o f r i g h t and l e f t e a r s e p a r a t e l y , was d e s ig n a te d as T e s t BO. Masking p ro c e d u re s were r o u tin e w ith a l l bone co n d u tio n m easurem ents a c c o rd in g t o recommended le v e l s of i n t e n s i t y . M a s k i n g was done i n a i r co n d u c tio n t e s t i n g when t h r e s h o l d d i f f e r e n c e betw een two e a r s exceeded tw e n ty - f i v e d e c ib e ls a t a g iv e n fre q u e n c y . S in ce i n t e r e s t in th e bone c o n d u c tio n v a lu e was d e lim ite d h e r e to i t s c o n t r ib u t io n to th e d e s c r i p t i o n of p a th o lo g y f o c u s , th r e s h o ld s f o r bone co n d u c ted s tim u li were d e r iv e d o n ly when im pairm ents o f a c u i t y by a i r conduc t i o n t e s t h ad been p r e v io u s ly o b ta in ed * M arg in al AC lo s s c r i t e r i a were a r b i t r a r i l y e s t a b l i s h e d a t f i f t e e n d e c ib e ls i n two f r e q u e n c ie s o r tw en ty d e c ib e ls f o r one fre q u e n c y . A l o s s o f t h i s m agnitude or more was c o n s id e re d n e c e s s a ry b e f o r e th e bone c o n d u c tio n t e s t was g iv e n . L osses o f s m a lle r m ag n itu d es o f te n o r f i v e d e c i b e l s , w ere c o n s id e re d w ith in " n o rm a l" l i m i t s . D i f f i c u l t i e s i n o b ta in in g v a l id bone c o n d u c tio n m easurem ents when th e e a r i s norm al o r n e a r norm al su p p o rte d t h i s c r i t e r i o n . The c o n s ta n t concern 189 B u l l e t i n No. 58 (M in n e a p o lis: Audio Developm ent Company, 1 9 4 6 ), p . 1. 64 w ith f a t i g u e te n d e d to arg u e a g a in s t m easurem ents t h a t would n o t c o n t r ib u t e to th e r e s o l u t i o n of th e m ajo r p r o blems o f t h e s tu d y . Speech R e c e p tio n T h re sh o ld T e s t . P ure to n e t e s t s a r e somewhat r e s t r i c t e d in th e e x te n t t o which th e y can d e s c r ib e th e h e a r in g h a n d ic a p f o r s p e e c h . I n r e c e n t y e a r s , " s p e e c h -ty p e " t e s t s have been d ev elo p ed w hich a tte m p t t o y i e l d th r e s h o ld d a ta f o r speech u n d e ra ta n d - ability. 1 9 2 , 1 9 5 Tiie t e s t s f a l l i n t o two g ro u p s; a) th e m o n ito re d v o ic e o f th e exam iner r e a d in g word o r se n te n c e l i s t s th ro u g h a h ig h f i d e l i t y a m p lif ie r - s p e a k e r c i r c u i t ; b) c a l i b r a t e d r e c o r d in g s o f a sp e ak er r e a d in g th e same m a te r ia l to be p la y e d th ro u g h h ig h f i d e l i t y c i r c u i t E. P . F o w ler, " I s th e T h re sh o ld Audiogram S u f f i c i e n t f o r D eterm ining H ea rin g C a p a c ity ? " j o u r n a l o f th e A c o u s tic a l S o c ie ty o f A m erica. 1 5 ;6 7 -6 0 , J u ly , 1943. ^ C h a rle s V. H udgins, e t a l . , "The Developm ent o f R ecorded A u d ito ry T e s ts f o r M easuring H earin g Loss f o r S p eech ," The L ary n g o sco p e. 5 7 ;5 7 -8 9 , J a n u a ry , 1947. 192 J . D. H a r r is , " S tu d ie s i n th e C om parative E f f ic ie n c y o f th e F re e V oice and th e Pure Tone A udiom eter f o r R o u tin e T e s tin g o f A u d ito ry A c u ity ," A rc h iv e s o f Oto1a ry n g o lo g y . 4 4 :4 5 2 -4 6 7 , O cto b er, 1945. James P. E gan, " A r t i c u l a t i o n T e s tin g M ethods, I l f OSRD R e p o rt. No. 5802. P sy c h o -A c o u stic L a b o ra to ry , H arv ard H n iv e rs i ty,"l^ovem ber, 1944. 65 The c h o ic e was d e lim ite d to th e re c o rd e d ty p e t e s t w hich seemed b e t t e r s u i t e d t o th e r e p e t i t i o n o f c o n s ta n t c o n d itio n s , c a l i b r a t e d s ta n d a r d s , and r e s e a r c h m ethods. A u d ito ry T e s t No. 14, Spondee 'Words, c o n s ta n t l e v e l , d ev e lo p ed and re c o rd e d by th e P sy c h o -A c o u stic L a b o ra to ry a t H arvard U n iv e r s ity , was f i n a l l y s e l e c t e d f o r o b ta in in g speech r e c e p tio n t h r e s h o l d s . T h e Spondee w ords, two s y l l a b l e words of eq u a l s t r e s s p e r s y l l a b l e , were re c o rd e d by th e H arvard L a b o ra to ry a t a c o n s ta n t l e v e l . The word l i s t s , i d e n t i f i e d as l i s t s 5-A and 6-B a r e shown i n th e A ppendix. A f t e r a s h o r t in i t a l c a l i b r a t i o n , th e w ords were p la y e d f o r th e s u b je c t and r e p e a te d by him as he h e a rd them t h r o u ^ th e s in g le ea rp h o n e. The words a r e re c o rd e d slo w ly enough, w ith a pause b etw een , so t h a t th e p e rso n b e in g t e s t e d h as tim e to l i s t e n and r e p e a t each word b e f o r e th e n e x t p r e s e n t a t i o n . The t e s t p ro c e d u re in v o lv e d e s t a b l i s h i n g an i n i t i a l c o m fo rta b le u n d e rs ta n d in g l e v e l . The l e v e l o f sound was 1 Q4. H udgins, e t a l . , l o c . c i t . ^®®Loo. o i t . A ppendix, p . 241 197 The c a l i b r a t i o n p ro c e d u re s fo llo w e d th o se f o r th e ADC equipm ent d e s c r ib e d in S im p lifie d Speech A udiom etry (M in n e a p o lis: A udio Development Company, 194§1, p . 3 , 66 g r a d u a lly re d u c e d a s I n d ic a te d on th e h e a rin g lo s s d i a l , u n t i l th e th r e s h o ld f o r speech was d e te rm in e d . The th r e s h o ld f o r speech was d e fin e d a s th e lo w e s t i n t e n s i t y l e v e l a t w hich f i f t y p e r c e n t o f th e spondee words c o u ld be re c o g n iz e d and r e p e a te d by th e p a t i e n t . T hree a u d i to r y t e s t s were th u s a d m in is te re d and i n th e fo llo w in g o r d e r : T e st AG Measurement o f a i r c o n d u c tio n th r e s h o l d a c u i t y f o r p u re to n e s , r i g h t and l e f t e a rs s e p a r a te ly . T e st BC Measurement o f bone c o n d u c tio n th r e s h o l d a c u ity f o r p u re to n e s , r i g h t and l e f t e a r s s e p a r a te ly . T e st SRT M easurement of sp eech r e c e p tio n th r e s h o ld a c u i t y f o r re c o rd e d spondee w ords. A u d ito ry , T e st No. 14 , r i g h t and l e f t e a r s , s e p a r a t e l y . . The m e d ic a l e a r , n o se , and t h r o a t ex a m in a tio n fo llo w ed th e t e s t b a t t e r y in each in s t a n c e . VI. AUXILIARY PROCEDURES IN AUDIOMETRIC TESTING % The ADC a u d io m e tric equipm ent employs a f l e x i b l e headband h o ld in g th e two r e c e i v e r s to th e e a r s . T his k in d o f r e c e i v e r s t a b i l i t y i s v e ry d e s i r a b l e when t e s t i n g c e r e b r a l p a l s i e d c h i l d r e n . I t does not demand t h a t th e s in g le r e c e i v e r be h e ld to th e e a r by th e s u b je c t as does a n o th e r m ajor ty p e o f eq uipm ent, w hich c o n d itio n would b e v e ry d i f f i c u l t f o r many o f t h i s c l i n i c a l p o p u la tio n . 67 S in ce th e c e p h a lic in d e x of b r a in in j u r e d c h i ld r e n v a r i e s o v er a wide ra n g e , i t became n e c e s s a ry to m odify th e h e a d s e t as needed. For m ic ro c e p h a lia te n d e n c y , i t som etim es became n e c e s s a ry f o r th e r e c e i v e r t o be removed from th e headband and to be h e ld to th e s u b j e c t ’ s e a r . A n u r s e ’ s a id e a s s ig n e d to th e r e s e a r c h i n v e s t i g a t i o n a s s i s t e d a t th e s e tim e s . W ith t h i s c l i n i c a l p o p u la tio n , com fort and r e l a x a t i o n w ere even more a t a premium. The s p e c ia l c h a ir d e v is e d tow ard t h i s aim h a s been p r e v io u s ly d e s c r ib e d . Sem antic r e l a x a t i o n p ro c e d u re s were u t i l i z e d w ith some o f th e more s e v e re a t h e t o i d s . When f a t i g u e symptoms w ere n o tic e d , th e t e s t was h a l t e d m o m en tarily and a n o th e r p r e o c c u p a tio n o f f e r ed u n t i l th e c h i l d was re a d y a g a in . In th e i n t e r e s t o f f a t i g u e c o n t r o l , th e s u b je c t was s e a te d o p p o s ite and f a c in g th e in v e s t i g a t o r a c ro s s th e au d io m eter t a b l e . The a u d io m e tr is t was th u s a b le to o b serv e more c l o s e ly th e s u b j e c t ’ s c o n d itio n th a n i f th e s u b je c t were f a c in g i n a n o th e r d i r e c t i o n . The s u b je c t was i n tu r n a b le to o b se rv e th e a u d io m e tr is t and to e x p l o i t th e s h o r t r e s t betw een each d i s c r e t e s tim u lu s . Use o f th e s ta n d a rd p u s h -b u tto n s ig n a l system to i n d i c a t e re sp o n se t o p u re to n e s t i m u l i was found in a d e q u a te b e c a u se of poor m otor c o n tr o l i n t h i s g ro u p . V erb al communi c a tio n was r e s o r t e d t o in most in s t a n c e s . C e r ta in c h ild r e n 68 w ith l i t t l e speech due to s e v e re b r e a t h in g mechanism involvem ent co u ld g a sp , "Y esI" and "Nol" r e l i a b l y . A few co u ld o n ly nod and sm ile o r shake t h e i r h e a d s . C o n firm a tio n o f th e s i g n a l s o f t h i s l a s t group was g a in e d w ith c o o p e ra tio n of th e p a r e n t , b u t se v e re c o n s e rv a tis m was e x e rc is e d i n a c c e p tin g th e s e re s p o n s e s . The t e s t i n g n a tu r e of th e s i t u a t i o n was somewhat co n c e a le d from th e v e ry young s u b j e c t s . The s ta n d a rd p ro c e d u re s f o r t e s t i n g were r e t a i n e d , b u t th e sound s tim u lu s became th e sound of " th e c r i c k e t w alk in g down th e r o a d , f a r t h e r and f a r t h e r aw ay," or of "Johnny G ra ssh o p p e r." The a n a lo g ie s o f th e a v i a t o r ’ s h e a d s e t, th e te le p h o n e , and th e r a d i o were u se d w ith some o f th e younger s u b j e c t s . L ib e r a l u se o f th e v o ic e c i r c u i t was made to h e lp e s t a b l i s h a c c e p t ance of th e t e s t . Care was ta k e n to m inim ize th e s t i m u l i t h a t m ight ap p ro ach th e th r e s h o ld o f o th e r s e n s e s . The lig h t b u l b i n th e room was k ep t a t on ly 60 w a tts to d u l l th e r e f l e c t i o n from th e iv o r y - c o lo r e d a c o u s t i - c e l o t e x w a ll s . Sudden movements o f th e r e s e a r c h e r w ere e lim in a te d a s much as p o s s i b l e . C o n tact w ith th e body o f th e c h i l d was r e s t r i c t ed to th e a u d io m e te r r e c e i v e r and f o r r e l a x a t i o n te c h n iq u e . The r e s e a r c h e r r e l i e d on h i s own e a s e and com fort in th e s i t u a t i o n , on h i s r a p p o r t w ith th e s u b j e c t s , to h e lp m ain t a i n t h e i r c o m fo rt. 69 V II. SUM M ARY In summary, th e fo llo w in g e x a m in a tio n s were a d m in is t e r e d : 1. A g e n e ra l p h y s ic a l e x a m in a tio n and n eu ro m u scu lar e v a lu a tio n d u rin g which a d i f f e r e n t i a l d ia g n o s is o f c e r e b r a l p a ls y was made. 2 . A p e d i a t r i c s tu d y in v o lv in g p r e - n a t a l d a t a , b i r t h h i s t o r y , and th e d is e a s e , a c c id e n t, and d ev e lo p m e n tal h i s t o r y of each s u b je c t. 3 . An e a r , n o s e , and t h r o a t e x a m in a tio n perform ed by a h o s p i t a l r e s i d e n t o to la r y n g o l o g is t and augm ented by o t o l o g i c a l h i s t o r y and g e n e r a l d is e a s e d a ta o b ta in e d from th e p a r e n t • 4 . A p u re to n e a i r co n d u c tio n t e s t o f th r e s h o ld a c u i t y . 5. A pure to n e bone co n d u c tio n th r e s h o l d t e s t when a m a rg in a l l o s s was o b ta in e d by a i r c o n d u c tio n p ro c e d u re . 6 . A u d ito ry T e s t No. 14, spondee words re c o rd e d by H arvard P sy ch o -A co u stic L a b o ra to ry . CHAPTER IV RESULTS An ex a m in a tio n c h a r t was c o n s tr u c te d f o r th e t a b u l a t i o n o f th e d a t a . Each c h a r t h a d th e s u b je c ts l i s t e d down th e o r d in a te and th e exam ined item s were l i s t e d a c r o s s th e a b s c is s a * A f te r d i f f e r e n t i a l d ia g n o s tic c a te g o r ie s were d e te rm in e d , th e s u b je c ts , were d iv id e d i n t o d ia g n o s tic sub- ty p e groups and th e e x a m in a tio n c h a r t s were re a d y f o r an a n a ly s is of group te n d e n c ie s . In l i k e manner, th e a u d io m e tric t e s t d a ta were c h a r te d by g ro u p s, w ith s u b je c ts l i s t e d down th e o r d in a te and fre q u e n c y i n c y c le s p e r second a c ro s s th e a b s c i s s a . The a u d ito r y t e s t d a t a , o to r h in o la r y n g o lo g ic a l exam in a t i o n f i n d i n g s , and m e d ic a l and h e a rin g h i s t o r i e s were exam ined by two o t o l o g i s t s and judgem ents r e g a rd in g th e p ro b a b le r e s t r i c t e d fo c u s o f p a th o lo g y were o f f e r e d . The d a t a w ere s u b je c te d t o s t a t i s t i c a l a n a l y s i s and th e e x p e rim e n ta l su b -g ro u p s com pared. The r e s u l t s of t h i s i n v e s t i g a t i o n a r e t o be p r e s e n t ed i n s e c tio n s . The f i r s t s e c tio n i s a d e s c r i p t i o n of th e sample s tu d ie d w ith re g a rd t o , a) d ia g n o s tic su b -ty p e c a t e g o r i e s , b) a g e , c) se x , d) e x te n t o f in v o lv e m e n t, e) s e v e r i t y , f ) e t i o l o g y of c e r e b r a l p a l s y , and g) drug d o sa g e . 71 The second s e c tio n i s an a n a ly s is o f th e a u d ito r y t e s t r e s u l t s . The t h i r d s e c t i o n i s a d is c u s s io n o f t e s t - r e t e s t r e l i a b i l i t y . The f o u r t h s e c t i o n i s a d e s c r i p t i o n o f o to r h in o la r y n g o lo g ic a l e x a m in a tio n f i n d i n g s . S e c tio n f i v e r e l a t e s th e a u d i t o r y t e s t d a t a , e a r , nose and t h r o a t e x a m in a tio n f i n d i n g s , and c a se h i s t o r y d a ta . The r e l a t i o n s h i p s betw een e t io lo g y and h e a rin g a c u ity i s t r e a t e d i n th e s i x t h se c tio n * In s e c tio n se v e n , r e l a t i o n s h i p s betw een h e a rin g a c u i t y and drug dosage a r e exam ined. I . DESCRIPTION OP THE SUBJECT SAMPLE The f in d in g s w ith r e g a r d t o th e s u b je c t p o p u la tio n s tu d ie d i s o f acme i n t e r e s t in d e m o n s tra tin g th e r e p r e s e n t a t iv e n e s s o f th e sam ple. One h u n d red and s e v e n ty -fo u r p a t i e n t s r e g i s t e r e d i n th e d ia g n o s tic and tr e a tm e n t c e n te r were i n v i t e d by form l e t t e r ( to th e p a r e n t s ) t o ta k e p a r t i n th e i n v e s t i g a t i o n . F i f t y - s i x , o r alm o st o n e - th ir d of t h i s p o t e n t i a l g ro u p , d id n o t a p p e a r, most of them w ith o u t e x p la n a tio n . A few of th e p a r e n ts i n t h i s number te le p h o n e d to t e s t i f y t h a t th e c h i l d ’ s h e a rin g was norm al and t h a t th e p a r e n t d id n o t have th e tim e to make th e t r i p . 72 One hu n d red and e ig h te e n (6 7 .8 p e r cen t o f th e i n i t i a l p o p u la tio n ) resp o n d e d by a p p e a rin g a t th e sc h e d u le d tim e , o r th e p a r e n t r e q u e s te d and r e c e iv e d a more s u i t a b l e tim e f o r a p p e a ra n c e . Only th o s e from whom w ere o b ta in e d r e l i a b l e and v a l i d t e s t s of p u re to n e h e a rin g a c u i t y f o r each e a r s e p a r a t e l y were in c lu d e d i n th e w orking r e s e a r c h sam ple. S eventeen s u b je c ts were th e r e f o r e e x c lu d ed b ecau se o f f a i l u r e t o meet th e s e re q u ire m e n ts . One h u n d red and one s u b je c ts d id m eet t h i s re q u ire m e n t and th e y c o n s t i t u t e d th e f i n a l c l i n i c a l sam pling f o r th e i n v e s t i g a t i o n . D ia g n o s tic Sub- Type C a te g o r ie s . The in c id e n c e o f th e v a rio u s d ia g n o s tic ty p e s o f c e r e b r a l p a ls y in c lu d e d in t h i s s tu d y i s shown i n T able I . The one hundred and one s u b je c ts sample c o n s is te d o f t h i r t y - e i g h t s p a s t i c s (3 7 .6 p e r c e n t ) , f o r ty - o n e a t h e to i d s (4 0 .6 p e r c e n t ) , n in e r i g i d i t i e s (8 .9 p e r c e n t ) , two tre m o rs ( 2 .0 p e r c e n t ) , and e le v e n o f com bined o r m ixed c l a s s i f i c a t i o n (1 0 .9 p e r c e n t ) . F iv e s u b je c ts of a t a x i a d ia g n o s is were se en b u t none c o u ld be t e s t e d s u c c e s s f u l l y , a c c o u n tin g f o r a com plete ab sen ce o f a t a x i c s u b -ty p e i n th e sa m p lin g . Phelps^^® e s tim a te s t h a t 40 p e r cent o f c e r e b r a l 198winthrop M. Phelps, "Treatment of the Cferebral P a l s i e s from the Functional Viewpoint," Journal of Occupational Therapy and Rehabilitation. 1 7 :6 7 -8 0 , April, .73 TABLE I INCIDENCE OP VARIOUS TYPES OF CEREBRAL PALSY Type P re s e n t Number S tudy P e r c e n t P h e lp s ’ E stim a te P e r c e n t C h i-S q u are S p a s t i c i t y 38 3 7 .6 40 A th e to s is 41 40.6 40 R i g id ity 9 8.9 Tremor 2 2.0 ao A ta x ia 0 0.0 Combined 11 10.9 T o ta l 101 100 100 .391 74 p a l s i e d in d iv i d u a ls a r e o f th e s p a s t i c ty p e , 40 p e r c e n t o f a t h e t o i d c h a r a c t e r , w ith a t a x i a , r i g i d i t y and tre m o r co m p risin g th e rem a in in g 20 p e r c e n t. I f P h e lp s ’ e s tim a te o f th e in c id e n c e o f ty p e s o f c e r e b r a l p a l s y i s a c c u a te , i t would seem t h a t th e sam pling in t h i s s tu d y c l o s e l y re se m b le s th e l a r g e r p o p u la tio n from w hich i t has b een draw n, w ith r e s p e c t to s p a s t i c s and a th e to id s * But th e f a i l u r e o f any a t a x ic to be s u c c e s s f u l l y t e s t e d e x c lu d e s them from s t u i y , and i t i s n o t c l e a r w h eth er P h e lp s ’ e x p e c te d f r e q u e n c ie s would in c lu d e as la r g e a p e rc e n ta g e of "com bined" ty p e as th e 1 0 .9 p e r c e n t o f t h i s sam ple. When th e ty p e s e x c lu d in g s p a s t i c s and a t h e t o i d s a re g rouped to g e th e r f o r p u rp o se s o f com parison w ith P h e lp s ’ p e r c e n ta g e s , a c h i- s q u a r e t e s t of "goodness o f f i t " can be done. The c h i- s q u a r e v a lu e o f .3 9 1 , d . f .» 2 , i n d i c a t e s no s i g n i f i c a n c e f o r th e sm all d iv e rg e n c e o f th e o b se rv e d f r e q u e n c ie s i n th e sample when compared w ith P h e lp s ’ h y p o th e t i c a l f r e q u e n c i e s . F o u rte e n o f th e a t h e t o i d s , more th a n a t h i r d , made c o n s is te n t u se o f te n s io n t o c o n tr o l in v o lu n ta r y movements and were s u b - c l a s s i f i e d a s te n s io n a t h e t o i d s . The rem a in in g tw e n ty -se v e n were th u s in th e n o n -te n s io n g ro u p . Age. The mean a v e ra g e age f o r th e t o t a l group 75 s tu d ie d was t e n y e a rs and one m onth. They ra n g e d from f i v e y e a r s , t e n months to n in e te e n y e a r s , and te n m onths, w ith a s ta n d a rd d e v i a ti o n o f th r e e y e a r s , one m onth. These d a t a , w ith a breakdow n by s u b - ty p e , a r e shown i n T ab le I I . The two s u b je c ts in th e tre m o r group a r e seen i n th e l i m i t s o f th e t o t a l ra n g e f o r t h a t g ro u p . Sex. More th a n 62 p e r c e n t of t h e sam ple were m a le s, and alm o st 38 p e r c e n t of th e fem ale se x . I n th e p re v io u s c l i n i c a l s t u d i e s , B r o c k w a y , a n d Lipscomb and K rusen^^^ found s i m i l a r p r o p o r tio n s i n th e g ro u p in g s by se x , as shown in T able I I I , but th e m agnitude o f th e d if f e r e n c e i s some what g r e a t e r in th e p r e s e n t sam p lin g . T his r e l a t i o n s h i p i n th e sex d i s t r i b u t i o n i s c o n s is te n t when each su b -ty p e i s c o n s id e re d a lo n e i n T ab le IV, e x c e p t f o r r i g i d i t i e s where th e o b se rv e d r e l a t i o n s h i p . i s r e v e r s e d . E x te n t. T ab le V i s an a n a l y s i s o f th e sample w ith r e g a r d to th e e x te n t o f th e p a l s y , t h a t i s , th e involvem ent of th e e x t r e m i t i e s . The g r e a t m a j o r i t y o f a t h e to i d s w ith ^ ^ % lv ia Brockway, "The Problem o f th e S p a s tic C h ild , C l i n i c a l Summary o f 1000 G a se s," J o u r n a l o f th e A m erican M edical A s s o c ia ti o n . 106:1 6 3 6 , May 9 , 1936. ^^^P aul R* Lipscomb and F ran k H. K rusen, " C e re b ra l B i r th P a ls y With S p e c ia l R e fe re n c e to P h y s ic a l T h e ra p y ," A rc h iv es of P h y s ic a l T h erap y . 2 4 :3 4 4 , Ju n e , 1943. 76 TABLE I I M EAN AGE, STANDARD DEVIATION, AND TOTAL RANGE FOR EACH CEREBRAL PALSY TYPE AND FOR THE TOTAL SAMPLE Type Mean Age ( y ea rs-m o n th s ) S ta n d a rd D e v ia tio n T o ta l Range (y e a rs-m o n th s) (y ears-m o n th s) S p a s ti c A th e to id R i g i d i t y Tremor Combined 10-2 10-5 9 -6 16-5 10- 10 5 -2 2-9 2-4 5 -2 3-7 5-10 to 19^10 5-10 to 17-5 6-5 to 14-8 15-5 to 19-7 6-4 to 16-2 Grand T o ta l 10-1 3 -1 5-10 to 19-10 77 TABLE I I I COMPARISON OP SEX DISTRIBUTION OP PRESENT ,SAMPLE WITH PREVIOUS STUDIES S tudy Male Fem ale - N P e r c e n t N P e r c e n t P r e s e n t S tudy 63 6 2 .4 30 3 7 .6 Brockway 550 5 5 .0 450 4 5 .0 Lipscomb and K rusen 93 5 5 .6 75 4 4 .4 W olfe 26 5 2 .0 24 4 8 .0 '78 TABLE IV SEX DISTRIBUTION OP THE PRESENT SAMPLE ACCORDING TO TYPE OF CEREBRAL PALSY Type N Male P er c e n t N Fem ale P er c e n t S p a s t i c i t y 24 6 3 .2 14 3 6 .8 A th e to s is 27 65.9 14 3 4 .1 R i g id ity 3 3 3 .3 6 6 6 .7 Tremor 1 6 0 .0 1 5 0 .0 "Combined" 8 7 2 .7 3 2 7 .3 A ll Types 63 6 2 .4 38 3 7 .6 79 I g H c ô E-i § I O u 0 1 •H T î •H b O •H « w »H m O 4 3 ® 4 3 M 4 3 *H O •H 4 3 D 3 as & I S 1 0 ri O J Ci î> S (M Ci 6 0 Ci OJ 6 0 OJ cd A •H © C t J a s cS b O •H *r-| •H < d © b O b O bO • H r i P ® ® ® b O A r i r i r i © • H A A A r i U r i 0 •H cd A t j cd C U • H cd 43 0 © G d U PL 0 S A E~i C 3 ? E4 iH S r i r i OJ Ci 5Î co 60 80 a l l f o u r e x t r e m i t i e s in v o lv e d i s n o te w o rth y . The quad r i p l e g i c c o n s t i t u t e d more th a n 95 p e r cen t of th e t o t a l a t h e t o i d group* The s p a s t i c q u a d r ip le g ic s (60 p e r c e n t) a ls o outnum ber th e o th e r s i n t h i s g ro u p , w ith th e h e m ip le g ic o n ly 24 p er c e n t. T h is may be o f p a s s in g i n t e r e s t when th e f r e q u e n t r e f e r e n c e s in th e l i t e r a t u r e to " th e s p a s t i c h e m ip le g ic " a r e c o n s id e re d . I t would seem from th e s e d a ta t h a t th e l e s i o n or le s io n s u n d e r ly in g th e in v o lu n ta r y movements of a t h e t o s i s and trem or te n d to a f f e c t th e t o t a l p h y s ic a l body r a t h e r th a n i s o l a t e d p o r tio n s . A s i m i l a r te n d en cy i s e v id e n t f o r th e com bined group and f o r th e s p a s t i c s , b u t th e l a t t e r have a l a r g e r p r o p o r tio n o f " p a r t i a l " inv o lv em en t th a n any o th e r ty p e . P re v io u s i n v e s t i g a t o r s o f f e r somewhat c o n f l i c t i n g in f o rm a tio n re g a rd in g th e r e l a t i v e in c id e n c e o f th e e x te n t o f th e handicap. , 2 0 3 , 2 0 4 , 2 0 5 A com parison of th e s e ^^^Brockway, l o c . c i t . ^ ^ ^ S te e le P . S te w a rt, "The R e la tio n s h ip Between Cause and D i s t r i b u t i o n o f S p a s t i c i t y i n C h ild h o o d ," The W estern J o u r n a l of S u rg e ry . O b s t e t r i c s , and C ynecology. 4 7 :5 0 7 , S eptem ber, 1939. “ 2 0 3 '^in tu ro p M * P h e lp s , "The T reatm ent of th e C e re b ra l P a l s i e d ," J o u r n a l of Bone and J o in t S u rg e ry . 2 2:1006, O cto b e r, 1940. ^^^Lipscomb and K rusen, l o c . c i t . ^^^W illiam W olfe, "A C l i n i c a l E v a lu a tio n o f F i f t y C e re b ra l P a l s i e d I n d i v i d u a l s , " (u n p u b lis h e d D o c to r’ s d i s s e r t a t i o n , S t a t e U n iv e r s it y o f Iowa, Iowa C ity , 1 9 4 7 ), p . 17. 81 d a ta w ith t h a t o f th e p r e s e n t i n v e s t i g a t i o n i s shown in T ab le VI* The g e n e r a l c h a r a c t e r of th e d i s t r i b u t i o n o f t h i s s tu d y seems s i m i l a r t o th e r e s u l t s o f th e i n v e s t i g a t i o n s o f P h elp s^ ^ ^ and Lipscomb and K r u s e n . A p a r t from W olfe, th e e a r l i e r r e s e a r c h e r s u n f o r tu n a te l y d id n o t d i s t i n g u i s h su b -ty p e d ia g n o s is and a com parison o f th e in c id e n c e o f th e p a r a l y s i s f o r each su b -ty p e i s n o t p o s s ib le * S in ce W olfe was a b le t o r e p o r t on o n ly s ix a t h e t o i d s , a com parison w i l l n o t be o f f e r e d w ith h is s u b - ty p e s . S e v e r i t y * A d ia g n o s is o f " s e v e r ity " was made f o r each s u b je c t and an a n a l y s i s o f th e d i s t r i b u t i o n o f s e v e r i t y i s shown i n T able V II. Each s u b je c t was g ra d e d on a t h r e e - p o i n t s c a le of s e v e r i t y , e i t h e r " m ild ," " m o d e ra te ," o r " s e v e r e ." The "m ild" e v a lu a tio n , was made when th e h a n d ic a p was of a n a tu r e t h a t would n o t i n t e r f e r e w ith o r d in a r y grow th and developm ent. The " s e v e re " c l a s s i f i c a t i o n was u se d w ith th e s u b je c t who c o u ld n o t be e x p e c te d t o p r o g r e s s w ith o u t b r a c e s , e x te n s iv e p h y s io th e r a p y , and, i n g e n e r a l , a good d e a l o f h e l p . Those s u b je c ts who were r a t e d on a p o r t i o n of th e continuum betw een th e two extrem es were ^ P h e lp s , l o o , c i t . ^^’ ^Lipsoomb and K ru sen , l o c . c i t . 208w olfe, l o c . c i t . 82 > I g o S â ë - ^ > M O co > pa % M M i§ O O E H O CO (3 O H M ÎU 5 ^ g ë . M ^ g§ ^ E H g S CO W M tu A Eh I Q 4 3 © o o A tO O r-i H O ^ U ^ © A a n © <-^43 6 © 0 0 n o p co © o LfH o w Q n A ^ u •H tU W - © wl n A (d ü T O CD © A O O r 4 6 0 © Il ^ X î ^ © A 4 3 îb O c d C O ^ 0 3 © Il 4 3 C O '— l>50 C d o S S o ^ â ' ^ A 43 £ 3 © O U © A 43 © o U © A P 43 43 co rH O 4 3 I — I U'à m * — © 6 fl © s a © o © A 0 0 0 0 0 • • • • • tO 0 I> to *> to H 0 G O 1 0 to O) # • • 1 0 0 1 0 03 to 03 fH to to Ci 00 to 03 • • • ' • • rH rH rH % > £> 03 t— i to £> » L O tO C O A tO * 1 0 C O 0 3 to 0 00 0 • • • # • 03 03 C O rH C O to 03 03 0 o> 0 0 0 1 • ' . • « H 03 C O rH c- cd •iH cd ♦ f H cd •H cd cd •H bO © bû bO bo © •H rH © © © •H bO A rH 1 —i rH bO © •H A A A © i"H d 0 « iH cd 1 — 1 A t J d d A •H cd 0 © cd •H d d S M A A A G? TABLE VII DISTRIBUTION OP SEVERITY A M O N G SUB-TYPES AND FOR TOTAL SAMPLE M ild M oderate S ev ere Mixed Type N P e r c e n t N P er c e n t N P er c e n t N P er c e n t T o ta l S p a s t i c i t y 12 3 1 .6 13 3 4 .2 5 1 3 .2 8 21 .0 100 A th e to s is 11 2 6 .8 . 14 3 4 .1 13 3 1 .7 3 7 .3 100 R i g i d i t y 3 3 3 .3 4 4 4 .4 2 2 2 .2 100 TVemor 1 5 0 .0 1 5 0 .0 100 "Combined" 4 3 6 .4 2 1 8 .2 2 1 8 .2 3 2 7 .3 100 T o ta l Sample 30 2 9 .7 34 3 3 .7 21 2 0 .8 16 1 5 .8 100 84 g iv e n th e "m oderate" e v a lu a t io n . "Mixed" e v a lu a tio n s were g iv e n th o s e s u b je c ts w here d i s s i m i l a r d e g re e s o f h a n d ic a p were r e g i s t e r e d f o r d i f f e r e n t p a r t s of th e body: f o r ex am ple, an a t h e t o i d q u a d r ip le g ia w ith se v e re in v o lv em en t o f th e le g s and only m ild h a n d ic a p o f th e arm s. P h e l p s r e p o r t on 271 s u b je c ts d is c lo s e d a s i g n i f i c a n t l y l a r g e r p e r c e n ta g e o f m o d erate r a t i n g s , 4 4 .3 p e r c e n t o f h i s sam ple, th a n was found i n th e p r e s e n t g ro u p . He a l s o found 2 8 .0 p e r c e n t s e v e r e c a s e s . No "m ixed" r a t i n g s were g iv e n to h i s s u b je c ts and th e h y p o th e s is t h a t th e d if f e r e n c e s in th e p e r c e n ta g e s m ight l i e w ith in th e s ix t e e n c a s e s of m ild s e v e r i t i e s p r e s e n te d i t s e l f . The m ixed group were c a r e f u l l y exam ined and e le v e n o f them show ed "m o d e ra te-m ild " c o m b i n a t i o n s . T h i s c o u ld ac c o u n t f o r th e d is c re p a n c y in th e m o d erate c l a s s , but th e d e v i a ti o n i n th e p e r c e n ta g e of se v e re s u b je c ts i s not th u s e x p la in e d . E tio l o g y . The c a u s a tio n o f th e p a r t i c u l a r p a l s y i s o f i n t e r e s t and im p o rta n ce f o r p u rp o se s of sam pling d e s c r i p t i o n . I t a ls o s e rv e s to in tr o d u c e th e i n v e s t i g a t i o n C r i t e r i a f o r g ra d in g e n t i r e l y a r b i t r a r y , e s t a b l i s h ed b y D r. M argaret Jo n e s. ^ ^ ^ P h e lp s, l o c . c i t . A ppendix, T able X X X I^,p>.242 85 o f th e r e l a t i o n s h i p betw een c e r e b r a l p a th o lo g y and h e a rin g p a th o lo g y . T ab le V III shows th e r e l a t i v e in c id e n c e o f th e v a r io u s b a s ic e t i o l o g i e s f o r each su b -ty p e o f p a ls y and f o r th e t o t a l sample* Seven c a te g o r ie s o f e tio lo g y g ro u p in g w ere u se d : p r e - n a t a l i l l n e s s , p r e - n a t a l a c c id e n t, b i r t h i n j u r y , e r y t h r o b l a s t o s i s f e t a l i s , p o s t n a t a l i l l n e s s , and an u n d i f f e r e n t i a t e d g ro u p . W ith th e e x c e p tio n o f e r y th r o b l a s t o s i s , each group can b e f u r t h e r a n a ly z e d b u t i s b e in g c o n s id e re d a s a homogeneous group a t t h i s tim e . The "u n - j d l f f e r e n t i a t e d " c a te g o r y in c lu d e d s u b je c ts f o r whom an in a d e q u a te e t io lo g y h i s t o r y was o b ta in e d o r f o r whom two o r more c a u s a tiv e o r c o n t r ib u t o r y f a c t o r s seemed t o o p e r a te . The p e rc e n ta g e o f e t i o l o g i e s in th e n a t a l p e r io d i s th e l a r g e s t f o r th e t o t a l sample and f o r a t h e t o i d , tre m o r and combined g ro u p s. The " u n d i f f e r e n t i a t e d " group was alm o st a s l a r g e , co m p risin g 52 p e r cent of th e sa m p le. McGovern and Y annet^^^ have shown by in d u c tiv e re a s o n in g on c l i n i c a l h i s t o r i e s t h a t th e u n d i f f e r e n t i a t e d group b e a r s a v e ry la r g e p r o p o r tio n o f b i r t h trau m a e t i o l o g i e s . I f th e y a r e c o r r e c t , th e " tr u e " a n a ly s is f o r th e p r e s e n t sam ple would i n d ic a t e an even l a r g e r lo a d in g o f th e b i r t h i n j u r i e s . 212john McGovern and Herman Y a n n e t," Asymmetric S p a s tic I n f a n t i l e C e re b ra l P a ls y : A C l i n i c a l S tu d y ," Am- e r ic a n J o u r n a l o f th e D is e a s e s o f C h ild re n . 7 4 :1 2 1 . A u su st. 1947. " * ------------------------------------ ' H M > I g 8 C O p L i' ë t cb m o O C M E 4 o m B i ï I I ê i H M > 4 Q 6-t H O > 4 ' ^ co H i-^ g 3 H m E H o |â 4^ 1 a < D C Q rt* to x t« £> < D © o ■ • • * • • Q - l -P lO to H < « H 0 5 c O C Q 1 0 to C O •H * H © Tj 4 :> 04 G a ^ |3 © ;^ to 0 m 03 H H to r4 +3 o r f 4 a >3 © •H rH 0 to o> 1 — 1 0 a 0 5 # • # • © s F h 1 0 H L O b O 0 © H C î a 04 0 ^ 0 ^ C Q 03 H to -P iH Ü c d 0 3 © 03 p w 0 • • • c d © 00 03 cî a ? 4 H P iH © © iH Ph 0 M 04 % % > H 00 P © G 1 * H © 0 03 03 0 © 0 • 0 fO 0 Æ p F h 0 03 i — 1 P O T © } > » c d 04 Fi M _ M 1 2 ; 0 H H H 1 — l p ( 4 © I> 0 03 0 to to 0 • • # • • • ^ F 4 C O 03 03 0 to 1 0 P F * Fi O J tO 03 0 to to F * © H •H F 4 04 P Q H ■ JSi 03 to 03 03 I> to H to P ' ( 4 H P © c d cî 0 to 0 p © # o 5 T b F i C V Î H F ! "H © © 0 04 Fi 0 1 0i H H p ü H © 00 1 — 1 03 c d O T 0 ♦ . • • p O T t o 03 1 —1 *> o d © Fi H H Fî F3 © © H 04 Fi H 1 04 M ^ îO H 1 —1 00 © H A P 6 © H 'd w < d A 0 •H p © w { > > ♦ H 0 •H Fi F: EH P P *d 0 •H H O T © •H a c d c d v d b û © S P A P •H Fi 0 0 C O < î 0 3 Eh 0 EH 86 87 I t would seem from th e d a t a in T able V II I t h a t th e i l l n e s s e s , p r e n a t a l and p o s t n a t a l , te n d to r e s u l t i n a s p a s t i c r a t h e r th a n any o f th e o th e r ty p e s of p a l s y . The i l l n e s s e s o f th e m o th ers who w ere b e a rin g th e e v e n tu a lly c e r e b r a l p a l s i e d c h i ld r e n in c lu d e d u rem ia o r k id n e y t r o u b l e ” i n t h r e e c a s e s , one pneum onia, one " f l u ” and two u n i d e n t i f i e d d is e a s e s w hich cau sed n au se a and v o m itin g th ro u g h o u t th e g e s t a t i o n . Six out o f th e seven c h ild r e n were b o rn one to two and a h a l f m onths p re m a tu re . N fo d e f i n i t e te n d en cy o f i l l n e s s ty p e i s e v id e n t. T h ere i s b u t one re c o g n iz e d e n c e p h a l i t i s , a lth o u g h two o th e r s u b je c ts had h ig h f e v e r s o f u n d e te rm in e d c a u se . O n e -c h ild had p e r t u s s i s and a n o th e r had p e r u s s i s c o m p lic a te d w ith pneum onia. I n f a n t d i a r r h e a w ith h ig h f e v e r was r e p o r te d f o r one s u b je c t and th e l a s t of t h i s group was an a r r e s t e d h y d r o c e p h a lic . S u b je c ts w ith e r y t h r o b l a s t o s i s f e t a l i s e t i o l o g y were d i s t i n g u i s h e d i n two ways w orthy o f comment. A ll o f th e s u b je c ts of e r y t h r o b l a s t o s i s background w ere a t h e t o i d s ; no o th e r s u b -ty p e h ad any s u b je c ts w ith t h i s e t i o l o g y . 2 6 .9 p e r cent o f a l l a t h e t o i d s u b je c ts were e r y t h r o b l a s t o s i s , a l a r g e r p e rc e n ta g e th a n p re v io u s l i t e r a t u r e m ight le a d us to expect* A com parison o f th e p r e s e n t sample w ith o th e r c l i n i c a l s t u d i e s in r e g a r d to th e in c id e n c e o f th e v a r io u s 88 e t i o l o g i e s i s seen i n T a b le IX. The p e r c e n ta g e o f e r y th r o b l a s t o s i s in th e t o t a l sam ple i s v e ry s i m i l a r to th e f i n d in g of McG-overn and Y annet, b u t b o th o f th e s e i n v e s t i g a t i o n s d is c lo s e d te n tim es as many e r y t h r o b l a s t o s i s as w ere d e s ig n a te d by S te w a rt» ^ ^ ^ The d i r e c t i o n o f t h i s r e l a t i o n s h i p may w e ll depend upon th e d a te o f th e i n v e s t i g a t i o n s s in c e S te w a rt* s work was p u b lis h e d i n 1939 when a t t e n t i o n was j u s t b e g in n in g t o fo c u s upon th e ja u n d ic e neonatorum symptom* We even f i n d h i s s u b je c ts i d e n t i f i e d w ith " ja u n d ic e ” e t i o l o g y r a t h e r th a n th e l a t e r e r y t h r o b l a s t o s i s o r ”r h iso im m u n iz a tio n ” te rm in o lo g y . The l a r g e p e rc e n ta g e o f b i r t h i n j u r y e t i o l o g i e s p r e v io u s ly n o te d i s c o n s i s t e n t w ith th e f in d in g s of th e o th e r s t u d i e s . P o s t n a t a l i l l n e s s e t i o l o g i e s o ccu r w ith th e approx im a te fre q u e n c y o f S te w a r t’ s group b u t McGovern and Yannet ju d g ed a p o s t n a t a l i l l n e s s t o be c a u s a tiv e f o r tw ic e as l a r g e a p r o p o r ti o n . The com plete ab sen ce o f p r e n a t a l c a u sa t i v e f a c t o r s i n th e McGovern and Yannet s tu d y d e f i e s e x p la n a t i o n u n le s s th e r e l a t i v e l y l a r g e p e rc e n ta g e o f u n d if f e r e n t i a t e d e t i o l o g i e s c o n ta in s them . The f iv e s u b je c ts w ith c o n g e n ita l anom aly e t io lo g y i n th e p r e s e n t sam pling have n o t one c o u n te r p a r t i n th e o th e r i n v e s t i g a t i o n s . PI 3 ^•^^Stewart, op. cit.. p. 508* :\89 TABLE IX PERCENTAGES OP PRESENT SAMPLE IN VARIOUS ETIOLOGICAL CATEGORIES COMPARED TO OTHER CLINICAL STUDIES. C a teg o ry P re s e n t S tudy (N=101) p e r c e n t McGovern and Y annet(N=68) p e r c e n t S te w a rt (N=265) p e r c e n t P r e n a t a l I l l n e s s 7 .9 1 .5 P r e n a t a l A cc id e n t 1 .0 0 .4 B i r th I n j u r y 3 5 .6 17.9 6 2 .1 E r y t h r o b l a s t o s i s 1 0 .9 10.4 1 .1 . P o s t n a t a l I l l n e s s 7 .9 16.4 8 .0 P o s t n a t a l A cc id e n t 4 .9 C o n g e n ita l Anomaly 5 .0 B ra in Tumor 1 .5 M ongolian 0 .4 U n d i f f e r e n t i a t e d 3 1 .7 5 3 .7 2 1 .6 , 90 In l i k e f a s h i o n , no s u b je c ts o f p o s t n a t a l a c c id e n t b a s i s a r e found i n t h i s s tu d y o r i n th e McGovern and Yannet g ro u p , b u t S te w a rt fo u n d t h i r t e e n s u b je c ts o f t h i s ty p e . D rug 3 ♦ R e sea rc h on th e e f f e c t s o f drug dosage on human s u b j e c t s i s d i f f i c u l t w ith in a c l i n i c a l program . Drug dosage must be d i c t a t e d by need. Q u a n ti tie s w i l l be v a r ie d by n eed . I n a d d i t i o n , i t I s n o t p o s s i b le t o e x e r c is e c o n tr o l o v e r a l l o t h e r f a c e t s o f th e r e h a b i l i t a t i o n program w h ile stu d y in g th e e f f e c t s o f th e drug v a r i a b l e on th e human f u n c tio n in g m echanism . A r e p o r t of drug dosage i s j u s t i f i e d , how ever, f o r p u rp o se s o f sample d e s c r i p t i o n . I t was a l s o s tr o n g ly f e l t t h a t any c o n s is te n t r e l a t i o n s h i p betw een h e a r in g im pairm ent and s p e c i f i c drug d o sag e sh o u ld b e made known and be c a r e f u l l y c o n s id e re d w ith in th e fram ew ork o f th e d i f f i c u l t i e s i n e x p e rim e n ta l design* T ab le X shows th e r e l a t i v e in c id e n c e o f s p e c i f i c dru g s u se d in th e tr e a tm e n t program f o r each p a ls y ty p e and f o r th e t o t a l sam ple. No a t t m p t was made to c o n s id e r th e . dru g dosage q u a n t i t a t i v e l y s in c e s y s te m a tic c o n tr o l co u ld n o t b e e x e r c is e d over q u a n t i t y w ith in th e c l i n i c a l th e ra p y s i t u a t i o n . The d ru g s a d m in is te r e d in c lu d e d g lu ta m ic a c id , p r o s tig m in e , p h é n o b a r b i ta l, t r i d i o n e , m e s a n to in , c u r a r e , and th y r o id e x t r a c t . More th a n one drug was g iv e n i f th e 91 i g 02bsOQ O H ©Sb so q .pexTM p-pooXiïI, I uioq.UBS©H X^%T quB qo ua-qd IBq.Tq*iBqou0qd: -©uoTpTJOi upoquBsaM -ouoppiaji upoquBS©]^ pBqjquBqouaqdE auoppî«ij, ©upmSpqsoaa: PTOV opi£[Bq.nx-D D- o CO rH O 03 0 3 rH CD bO tO rH O r-t rH rH rH H rH fH rH rH H H 1 —1 1 — 1 rH 0 3 rH à rH rH rH CO tO 1 — 1 rH iH M î>s 4J> O T *fH •H «Tj O O T 4 ^ © •H O •H S h fl - P +3 O •H rH O T © v 4 a ' S cd oJ h bO © 0 4 3 A - P •H F, o O CO < 04 EH o EH 92 f i r s t co u ld n o t a c h ie v e th e in te d e d p u rp o se . C om binations o f d e p r e s s a n ts and a n t i - c o n v u ls a n ts a r e l i s t e d s e p a r a te ly v h i l e o th e r m ixed d o sag es a r e g ro u p ed to g e t h e r . P ro s tig m in e c a u ses c o n t r a c t i o n of th e smooth m uscle o f th e i n t e s t i n e and in c r e a s e d p e r i s t a l s i s by s ti m u la tio n o f th e p a ra s y m p a th e tic n e rv e e n d in g s of th e autonom ic 214 n erv o u s sy stem . M uscle to n e i s in c r e a s e d . P h é n o b a r b ita l i s a d e p r e s s a n t o f th e c o r te x and o f th e s u b c o r t i c a l g a n g l ia , e s p e c i a l l y i n th e r e g io n of th e h y p o th a lam u s. I t i s u se d to c o n tr o l m otor h y p e r i r r i t a b i l i t y and c o n v u ls io n s , and can be a d m in is te re d o v er lo n g p e r io d s o f tim e w ith o u t p ro d u c in g t o l e r a n c e , h a b i t u a t i o n , o r symptoms o f c u m u la tiv e p o is o n in g . T hese f e a t u r e s make i t v a lu a b le i n th e tr e a tm e n t o f e p i le p s y . T rid io n e h a s a n a lg e s ic a s w e ll as a n t i - c o n v u lsa n t p r o p e r t i e s , b û t i s not a p p r e c ia b ly d e p r e s s a n t. I t i s u se d p a r t i c u l a r l y i n p e t i t mal and psychom otor s e iz u re s .^ ^ ® » ^ ^ ’^ 0*14 L o u is Goodman and A lf r e d G ilm an, The P harm acolo- g i c a l B a sis of T h e ra p e u tic s (New Y ork; The M acM illan Companv. l t i r T , ‘T p r ^ 3 ^ - M 8 T ' ^^^H arold N. W rig h t, and M ild re d M ontag. A T extbook o f Pharm acology and T h e ra p e u tic s ( f o u r t h e d i t i o n ; P h i l a d e l p h ia ; W. B. S aunders Conipany, 1 9 4 9 ), p p . 156-160. ^ ^ % a r r y S. M u stard , and Samuel L iv in g s to n , " T rid io n e T herapy i n E p ile p s y ," J o u r n a l o f P e d i a t r i c s . 3 5 :5 4 0 -5 4 5 , November, 1949. J . N ixon, and John L. Emery, "T oxic E f f e c t s of T r i d i o n e , ” L a n c e t. 2 5 6 ;5 9 -6 2 , Ja n u a ry 8 , 1949. 93 M esantoin i s an a n t i - c o n v u lsa n t w ith l i t t l e de p r e s s a n t a c t i o n . I t i s b e lie v e d r a t h e r t o x i c and h as n o t come in to common use.^^® F u r th e r e x p e rim e n ta tio n i s b e in g t r i e d w ith t h i s d ru g . The a c t i o n o f c u r a r e i s s o l e l y to im prove m u scu lar r e l a x a t i o n . The s i t e o f th e a c tio n seems a t th e p o in t o f 219 tr a n s m is s io n of th e im p u lse from th e n e rv e to th e m u sc le. G lutam ic a c id h as been u t i l i z e d where in c r e a s e in 220 p h y s ic a l and m e n tal a l e r t n e s s i s d e s ir e d . I t i s common l y a d m in is te re d in g r a d u a lly in c r e a s in g d o sag es w orking tow ard to le r a n c e l i m i t s . T hyroid e x t r a c t s a c t p r im a r ily on th e m e ta b o lic r a t e . In th e p r e s e n c e of th y r o id s u b s ta n c e , c e l l s m etabo l i z e a t a f a s t e r r a t e . I t i s u se d t h e r a p e u t i c a l l y in myxadema (h y p o th y ro id ism ) t o com pensate f o r th e d e f i c i e n t n a t u r a l p ro d u c tio n of th e horm one. 21A • ^ P e rs o n a l o r a l com m unication w ith D r. S pencer Brown, P e d i a t r i c i a n , U n iv e r s it y H o s p ita l, U n iv e r s ity o f M innesota. ^^^W right and M ontag, op. c i t . . p . 200. T. Zimmerman, B. B. B u rg e m e is te r, and T racy J . Putnam , "A Group Study o f th e E f f e c t o f G lutam ic A cid Upon M ental F u n c tio n in g i n C h ild re n and A d o le s c e n ts ," Psycho- so m a tic M e d ic in e . 9 :1 7 5 -1 8 3 , M ay-June, 1947. . C. P f e i f f e r and A. T. H asegava, "Pharm acology o f G lutam ic A c id ," Modern H o s p ita l. 7 0 :8 0 . 8 2 . 84. 86. A p r i l , 1948. ------------------ -------- 222 G. A. S te p h e n s, Hormones and V itam in s (London: George Hewnes, L t d . , 1 9 4 7 ), pp. 2 1 -3 3 . 94 The l a r g e m a jo r i ty o f th e sam p le, 6 3 .6 p e r c e n t, r e c e iv e d no dru g tr e a tm e n t a t a l l . The l a r g e s t s in g le d ia g n o s tic c a te g o r y to r e c e iv e drug tre a tm e n t was th e a t h e t o i d w ith more th a n 50 p e r ce n t g iv e n d ru g s . P ro s tig m in e was g iv e n a lm o st e x c lu s iv e ly t o a t h e t o i d s . A l a r g e r p e r c e n ta g e o f s p a s t i c s th a n o th e r groups r e c e iv e d g lu ta m ic a c id a lth o u g h th e sm a ll number of s u b je c ts p r e c lu d e s comment upon te n d e n c ie s . D e p re s s a n ts and a n t i - c o n v u l s a n t s w ere a d m in is te re d to a t h e t o i d s more th a n to o th e r ty p e s i n th e a tte m p t to q u ie t in v o lu n ta r y movement. The r e c o r d of drug dosage i s a dynam ic, e v e r-c h a n g in g f i l e o f in f o rm a tio n . T ab le X n eed s to be d a te d June 1948. E x am in atio n o f th e c l i n i c a l c a s e h i s t o r i e s of th e same p o p u la tio n one y e a r l a t e r in June 1949 i n d i c a t e s m ajo r changes in ty p e s o f d ru g s and number of s u b je c ts r e c e iv in g them . P ro s tig m in e was no lo n g e r b e in g a d m in is te r e d . D ila n tin ^ ^ ^ was b ein g c o n s id e re d an e f f i c i e n t drug in th e tr e a tm e n t o f g ra n d m al. The e n th u sia sm f o r g lu ta m ic a c id had been tem pered by e x p e rie n c e and o n ly a few were r e c e i v in g i t . I I . AimiOMETRIC TEST FINDINGS The d a ta d e r iv e d from each e a r s e p a r a t e l y was t r e a t e d 2P3 H enry R. V ie t s , "R ecent Advances in Drug T herapy i n P s y c h ia tr y and N e u ro lo g y ," H o s p ita l Management. 6 5 :8 8 .9 0 9 2 ,9 4 ,9 6 ,1 3 2 , A p r i l , 1948. --------- 95 s e p a r a t e l y . The a d v a n tag e o f a d oubled N by c o n s id e r a tio n o f t o t a l e a r s r a t h e r th a n s u b je c ts was c o n s id e r e d i n s u f f i c i e n t v a lu e to r i s k p o s s ib ly subm erging s i g n i f i c a n t d i f f e r e n c e s betw een th e e a r s . I n t e r - e a r com parisons m ight a l s o c o n t r i b u te to p a th o lo g y d i f f e r e n t i a t i o n . The th r e s h o ld s f o r p u re to n e a i r c o n d u c ted s ti m u li were com pared t o two s e t s o f r e f e r e n c e d a t a . The th r e s h o ld v a lu e s i n d e c ib e l u n i t s of d e v ia tio n from th e c a l i b r a t e d z e ro were a u to m a tic a lly compared t o th e " t o t a l p o p u la tio n " by th e e s s e n t i a l n a tu r e o f modern a u d io m e te r c a l i b r a t i o n . The a b s o lu te mean i n t e n s i t y v a lu e s o f th e 2 ,4 8 4 e a rs w ith a h i s t o r y o f "norm al" h e a rin g f o r speech i n th e U. S. P u b lic H e a lth Survey^^"^ s e rv e a s - t h e c a l i b r a t e d z e ro s f o r a u d io m e te r c o n s tr u c tio n by th e Ü. S. B ureau o f S ta n d a rd s and th e a u d io m e te r i n d u s t r y . Any th r e s h o l d d e r iv e d on th e modern c a l i b r a t e d a u d io m e te r i s s t a t e d i n d e c ib e l u n i t s of d e v i a ti o n from th e p o p u la tio n r e f e r e n c e z e r o , r a t h e r th a n from th e p h y s ic a l P?4 Normal H ea rin g by A ir and Bone C o n d u c tio n . N a tio n a l H e a lth S urvey, H earin g S tudy S e r i e s , B u l l e t i n #4 (W ashington, D. C .: X T . S. P u b lic H e a lth S e r v ic e , 1 9 3 8 ), p . 6 . 225 "R ep o rt o f th e Committee on th e C o n s e rv a tio n o f H earing o f th e A m erican Academy o f O phthalm ology and O to la ry n g o lo g y ," T ra n s a c tio n s o f th e A m erican Academy of Ophthalm ology and O to la ry n g o lo g y . 4 5 :2 5 -2 8 , 1940. 96 z e ro (#0002 dynes p e r sq u a re c e n tim e te r ) . The c a l i b r a t i o n c o n s ta n ts were d e r iv e d from a l a r g e number o f "norm al" e a r s w ith no r e g a r d f o r ag e . But th e same d a t a , a n a ly z e d f o r ,a g e d i f f e r e n c e s , shows s l i g h t l y d i f f e r e n t mean and s ta n d a rd d e v i a ti o n m easu res f o r th e age g roups m ost s i m i l a r t o th e ag es o f th e c e r e b r a l p a ls y g ro u p s i n t h i s s tu d y . A c o n t r o l group o f f o r t y c h i ld r e n and young p e o p le o f ag es s i m i l a r to th o s e o f th e c e r e b r a l p a l s y g ro u p s w ere g iv e n th e a i r c o n d u c tio n t e s t . These c o n t r o l in d iv i d u a ls w ere accom panying r e l a t i v e s t o o th e r c l i n i c s , had no p r e s e n t p h y s ic a l c o m p la in ts , had no p r e s e n t or r e c e n t c o ld s , and had n e g a tiv e h e a rin g h i s t o r i e s . T h e ir th r e s h o ld s were grouped and s t a t i s t i c a l a n a ly s e s w ere done p ro v id in g a second s e t of r e f e r e n c e d a t a . I n summary, th e n , th e a i r co n d u c tio n th r e s h o ld m easu res were com pared (1 ) to t o t a l p o p u la tio n norm s, and (2) t o a c o n t r o l group o f f o r t y "norm al" h e a rin g non h an d icap p e d c h i l d r e n . 226 Normal H earing f o r Speech a t Each Decade of L i f e , N a tio n a l H e a lth S urvey, H ea rin g S tudy S e r i e s , B u l le t in . #6 (W ashington, D. C. : U. S. P u b lic H e a lth S e r v ic e , 1 9 3 8 ), p p . 1 0 -1 3 . 97 P ure Tone A ir C onduction M easures# The mean and m edian th r e s h o ld s f o r a i r c o n d u c tio n m easurem ents w ith th e s ta n d a r d d e v ia tio n s of th e means a re shown in T ab le XI. The v a lu e s f o r t h e two tre m o r s u b je c ts a r e l i s t e d i n t a c t . The minus v a lu e s im ply an a v e ra g e a c u i t y b e t t e r th a n th e c a l i b r a te d zero of th e a u d io m e te r, o r b e t t e r th a n th e p o p u la tio n mean a r b i t r a r i l y d e s ig n a te d by z e ro . F ig u re 2 more g r a p h ic a lly p r e s e n ts th e mean a u d io grams to f a c i l i t a t e co m parison. Means* From T ab le XI and F ig u re 2 , a g r e a t e r h e a r in g l o s s f o r th e a t h e t o i d s a t a l l f r e q u e n c ie s t e s t e d and in b o th e a r s i s o b se rv e d . H earin g lo s s seems t o be p o s i t i v e l y r e l a t e d to fre q u e n c y , ra n g in g in th e r i g h t e a r from a low o f 8 .9 0 db a t 256 c . p . s . to a h ig h of 1 7 .3 2 db o f lo s s a t 4096 c . p . s . I n th e l e f t e a r , a s i m i l a r a lth o u g h n o t ex a c t r e l a t i o n s h i p i s s e e n , w ith a low o f 6 .3 8 db a t 256 c y c le s t o a h ig h l o s s o f 1 6 .2 2 db a t 2048 w ith a s l i g h t drop to 1 5 .6 1 db a t 4096 c y c le s . The o th e r t h r e e c l i n i c a l su b -g ro u p s seem t o bunch around th e norm al z e ro . The l e f t e a r o f th e combined group seems to show a sm a ll a v e ra g e im pairm ent i n th e low er f r e q u e n c ie s b u t w ith b a r e l y th e m agnitude o f th e a t h e t o i d g ro u p . Nine o f th e p o s s i b l e te n mean v a lu e s f o r th e 98 M X I g < a* I 4 3 « H © A a I • H 1 0 O ) o • S C Q ^ • o A02 « o O O n H © g . © 0 3 F i rH to lO 03 to O $ • C O m • O A03 O >)03 § S © & © 03 F h rH Â L O to to 03 G O 0 to 03 pH 0 to rH 0 C O to 03 0 C O to 0 ï> to 0 0> 't S t to 0 03 03 rH 0 0 5 00 0 • « • * • • . • # # « • • • • • • • 0 to 0 0 C 5 > to to to H 0 to 0 to rH 0 0 3 1 rH 03 1 rH 03 0 0 0) 03 0 to to 0 C O to 00 0 C O rH 0 ^ A 0 03 C O 03 0 rH to to 0 05 03 rH 0 " 4^ 00 • • ♦ • • • • • • • « • * • • • • * 0 to 0 0 to to to 03 ^ 0 0 to 10 to C O 0 0 3 1 rH 03 1 rH C O 0 to £S to 0 to y I> 0 C O to to 0 to to Ô o> rH 0 0 to IS 0 rH IS to 0 0 rH 0 c - IS • • . • • • • • • • • • • « 0 10 0 0 IS 0 to C O to rH lO y t 1 — 1 to to to 0 03 1 I — 1 1 — 1 1 1 03 to 0 C 5 > 05 00 0 00 to to 0 0 IS 0 C O C O 0 0 rH 0 to 0 to 0 O D 0 to 0 C O 03 to 0 IS IS • • • ♦ • ♦ • • • • • • • » • • • • 0 to 0 0 C O 0 - 10 10 to 0 to to 0 to to 0 1 rH 1 1 rH rH rH to 0 0 to G O 0 £>- 0 5 rH 0 IS C D 0 0 C O 05 0 0 to 0 05 ^ to 0 03 03 iH 0 to rH 0 C O 0 • • • • • • • • • • . • • • • • * • 0 to 0 0 00 to lO 03 to rH to C O 0 to - tM A rH rH rH t 1 rH I — 1 rH 05 0 05 to 03 0 to 0 3 0 3 0 to 0 C O 0 0 IS to 0 05 03 to 0 03 0 0 3 0 C O C O 0 0 0 0 • • • ♦ • ♦ • • • • • • • • • « • • 0 to 0 0 C O 0 10 I> * 4* 03 to IS 0 0 0 0 to ( — 1 03 1 1 to 0 IS to 03 0 to IS to 0 00 to : 0 0 5 0 0 03 0 rH 03 0 0 0 10 0 0 5 03, 0 0 to 0 • * • ♦ • # ' • • • • • • « • • « 0 to 0 0 to £> to to -H * 0 to to 0 0 0 0 Is rH 03 1 1 to 0 03 IS 0 ^ to 03 0 C O G O to 0 03 C O 0 0 ) rH 0 to rH 0 0 to IS 0 3 0 C O iH IS 0 05 t> • • • • • • • • • • # #' • # • • ♦ • 0 to 0 0 to 03 to 0 C O 03 10 to rH to to 03 0 rH rH 03 1 1 rH to 0 0 to 05 0 IS 0 5 to 0 to IS to 0 0 05 0 0 rH 0 IS A " S h 0 0 C O 10 0 03 rH C O 0 rH C O » * • • • • ■ • • • • • • • 0 to 0 0 G O 0 to IS C O 0 to 0 5 0 to rH 0 rH 1 rH rH t 1 rH rH rH rH 0 IS 03 0 0 to to 03 0 tn rH C O 0 0 3 0 0 0 rH 0 to to 05 C to C O 03 0 C O Is IS 0 to 0 • • • ». # • • • • • • • . • 0 to 03 0 05 iH C O to " 4 * to 03 0 C O to . to 03 0 C O rH 1 rH rH rH 43 iH a © w « rH 4 3 c 5 g * © A © • C O ! > a © t > U P q C J • o aJ oJ A *H © * H *43 ^ » d C O a s © c« Î 2 Î C O W 43 •H O fH 43 T O O S ■8- E H g S à © «H • - X 5 C O © s T O f4 T O O 43 © 43 43 C t J A E H g g . o o S T O A * H © * H ' • 4 3 ts to T O © ç r ü f3 î E H 4 3 A -ri •H bO -H 03 rH 03 4k 4k I I F, o Î & g d • T O A © -H a Tf C O © T S © d o •H 4 3 T O 03 I A VO VO CO s CM s CM s g o o o o o I i # # A t> § § gj Q ) A W W < tj c e ; M ^ ts O (O (O VO o I o CM o o o o o o o o & C D A I •H c c ; WwcrtM^crj 4 - 9 O C O U ] eg 100 r i g i d i t y group a r e l e s s th a n z e ro . In th e much l a r g e r sample of s p a s t i c s , s i x o f th e t e n v a lu e s a re l e s s th a n z e r o , w ith a th r e s h o ld more th a n two d e c ib e ls l e s s th a n z e ro a t 256, r i g h t e a r . The com bined group shows n o t one mean n e g a tiv e v a lu e a lth o u g h 2048 and 4096, r i g h t e a r , a r e e x a c tly a t z e ro . V a r i a b i l i t y . D if f e r e n c e s i n s ta n d a rd d e v i a ti o n s in T ab le XX a re n o ta b le . The s p a s t i c s and r i g i d i t i e s a r e r e l a t i v e l y s i m i l a r and n o t u n lik e th o s e o f th e N a tio n a l H e a lth S urvey. The r i g h t e a r of th e com bined group te n d s t o be somewhat l a r g e r and th e l e f t e a r S .D .* s ra n g e from 1 1 .8 3 t o 22.76 d e c ib e ls i n d ic a t in g a wide d i s t r i b u t i o n around th e mean. The a t h e t o i d s ta n d a rd d e v i a ti o n s a re th e l a r g e s t and th e y i n c r e a s e , as do th e mean t h r e s h o l d s , from low t o h ig h e r f r e q u e n c i e s . In a l l i n s t a n c e s , th e s ta n d a r d d e v ia tio n s a r e l a r g e r th a n th e means and in some th e d i f f e r e n c e i s o f a sub s t a n t i a l m ag n itu d e . T his r e l a t i o n s h i p i s p a r t l y a f u n c tio n o f th e n e g a tiv e th r e s h o ld v a lu e s w hich d e p re s s th e mean b u t do n o t th u s a f f e c t th e s ta n d a rd d e v i a t i o n , s in c e th e n eg a t i v e v a lu e s become p o s i t i v e when sq u a re d i n th e s ta n d a rd d e v i a ti o n d e r i v a t i o n . I t can be c o n je c tu r e d t h a t i f th e d a ta were c o r r e c t e d 101 f o r t h i s in c o n s is te n c y , ( f o r exam ple, coding h e a rin g lo s s v a lu e s 30 t h a t th e lo w e st p o s s i b le i n t e n s i t y m e asu re, minus to n , w ere c o n v e rte d t o z e ro ) t h a t th e m agnitude o f th e r e l a tio n s h ip would be s u b s t a n t i a l l y d e c re a s e d . M edians.- B xam ination o f th e m edian v a lu e s a r e i n t e r e s t i n g . S p a s tic and combined groups f in d t h e i r m edians a t z e ro f o r a l l f r e q u e n c ie s in b o th e a r s . The a t h e t o i d m edian sc o re i s a lo s s o f f i v e d e c ib e ls f o r a l l of th e f re q u e n c ie s and b o th e a r s . Seven of th e te n m edian v a lu e s f o r th e r i g i d i t y s u b je c ts a r e f i v e . d e c i b e l s b e t t e r th a n th e norm al mean a v e ra g e . S ig n if ic a n c e o f th e Means. A com parison o f th e s e d a ta w ith th e t o t a l p o p u la tio n p erfo rm a n ce shown i n pp>7 B e asley * s N a tio n a l H e a lth Survey was made. The means and s ta n d a rd d e v ia tio n s f o r th e 2 ,4 8 4 a i r c o n d u c tio n t h r e s h o ld s of th e N a tio n a l H e a lth Survey a r e g iv e n a t th e to p o f T ab le X I. The means a r e , of c o u rs e , z e ro s in c e t h e i r a b s o lu te i n t e n s i t y v a lu e s a t each fre q u e n c y c o n s t i t u t e th e c a l i b r a t e d z e ro of t h i s a u d io m e te r. 227yj^illig B e a s le y , C h a r a c t e r i s t i c s and D i s t r i b u t i o n o f Im p aired H earin g i n th e P o p u la tio n o f th e U n ite d S t a t e s , ’* J o u rn a l o f th e A c o u s tic a l S o c ie ty o f A m erica, 1 2 :1 1 5 , J u ly , 1940. 102 The s ig n i f i c a n c e o f th e d i f f e r e n c e s betw een th e group p erfo rm a n ce and th e norm was t e s t e d w ith * * S tu d e n t * s " p p Q t r a t i o d e s c r ib e d by F ish e r# S in ce com parison in e v e ry in s ta n c e i s made w ith z e r o , th e a b s o lu te mean h e a rin g l o s s i n d e c ib e l s i s a ls o th e m easure o f th e d if f e r e n c e betw een th e group mean and th e p o p u la tio n norm# The t r a t i o s a r e p r e s e n te d i n T ab le XI and a g a in i n T able X II where th e p e rc e n ta g e of s i g n i f i c a n c e i s c o n sid ered # The t r a t i o s o f th e a t h e t o i d groups a r e seen t o be th e o n ly s i g n i f i c a n t v a lu es# They a re a l l s i g n i f i c a n t w e ll beyond th e one p e r ce n t l e v e l i n d i c a t i n g l e s s th a n one chance in one hundred t h a t th e o b se rv e d d i f f e r e n c e s c o u ld o ccu r by chance# W e can be h ig h ly c o n f id e n t, t h e r e f o r e , i n r e j e c t i n g th e h y p o th e s is t h a t th e a t h e t o i d sam ple and th e H e a lth Survey sam ple b elong to i d e n t i c a l p o p u la tio n s . The m ost s i g n i f i c a n t d i f f e r e n c e s o ccu r a t 2048 and 4096 c . p . s # , c o n s is te n t w ith t h e i n t e r - f r e q u e n c y r e l a t i o n s h ip s o f th e o b se rv e d means# The s p a s t i c , r i g i d i t y , and combined g ro u p s, on th e o th e r h an d , d e m o n stra te n o t one s i g n i f i c a n t or n e a r s i g n i f i c a n t t r a t i o w ith th e norm. A part from th e a t h e t o i d v a lu e s . A# F i s h e r , S t a t i s t i c a l Methods f o r R e se a rc h W orkers (E d in b u rg h : O liv e r and Boyd, 1 9 4 2 ), p p . 164-168. 103 M M X I g s ië |2 i ë ê o M cg P q C Î 3 M M § Q H m ^ H ïi I b ". iî & § Q M il P h O o p 4 C O H s g E n S i m-" to 02 O O lO T O T O to o o to O p C D ^ |2 5 02 © © ^ Js; O • * M H # • ♦ C O 05 o O to T O T O to o o rH O o m C O to © © 03 îs; C D îs; • O « • M N ♦ * P ^ C V J U • c d o % > O o T O T O to o o to p O Oî to ^ % ' . Z > rH O + > ! ^ 0 m * H > H ; * * < Î h o rH to rH O d ' - . a Q ) d 03 05 o o to T O T O 0- o o 00 p O 0*rH O % )2: o ^ O 03 0- ^ ÎS i ( D L O • M H # » d to P h - to to o o 05 T O T O 00 p o 05 O O L O ^ % 02 © © o ^ Iz; 02 .# • ÎH ÎH « # to rH <o to o o 02 T O T O \ o p o O o o 05 02 ïe; d ; O © © C D O ^ ÎS î O * H H • • T h • '00 to o o I > T O T O to o o o o o T O - V j l 03 O © © 02 f e - o î s ; ^ • o * • H M • • d 0^02 c d • M o 03 o o tO T O T O cô o o to o o -p 03 rH ^ îz; I> rH Î2î ^ % > Iz; ^ P ^ O # t > H > H « # w ü rH to rH » H d < D d 02 to o o 05 T O T O O p o 05 o p C T ’rH 05. ^ % C O © © iH îs; to % ^ m L O • • H M • • u to C D 03 o o lO T O T O 1 — 1 p o o o p L O to Î 2 5 00 < D © £> o Î2i ^ 03 • « H M • rH to rH rH rH r i rH rH 1 — 1 rH rH © © © © © © © © > > > î> > > > > © © d o © © d o © © d o © © g O p 3 f-9 -H f-9 H * r H d -H #4 U * H o +5 O +5 O 45 O 4^ X c d ^ c d Î2 Î a ^ O S L O rH •• M lO t— 1 •• (i; lO I — \ • C d L O rH • Ç c ^ T i o •H E H -p E h © E h « H en d G o d d 4n d d d d d -P b O b O 4J b O b O *d b O b O •H b O b O T O •H « H © •H * H •H •H « H •H * H W C Q C O .d C O C O b O C O C O n C O C O A 4^ o C Q < C d Q 104 th e b e s t s ig n i f ic a n c e found i s a 1 .3 2 t r a t i o f o r s p a s t i c s a t 256 c . p . s . , on th e r i g h t e a r . The s i g n i f i c a n c e i s on ly s l i g h t l y b e t t e r th a n th e 20 p e r c e n t l e v e l . I n s p e c tio n of T able XI r e v e a ls t h a t th e mean v a lu e in v o lv e d i s a n e g a tiv e one. The o th e r t r a t i o s r a p i d l y d e c re a s e i n v a lu e and th e r e s e a r c h e r can be h ig h ly c o n fid e n t t h a t d i f f e r e n c e s n o te d betw een th e norm and each o f th e s p a s t i c , r i g i d i t y , and combined groups af% ^only chance d i f f e r e n c e s . Com parison W ith C o n tro l Group. The f o r t y non h a n d icap p e d in d iv i d u a ls who com prised th e c o n t r o l group of norm al e a rs ra n g e d i n age from f i v e y e a rs to tw e n ty y e a r s , one m onth, w ith th e m edian age d ro p p in g t o e i g h t y e a r s , n in e m onths. The s ta n d a rd d e v i a ti o n o f th e mean age was two y e a r s , te n m onths. These d a ta can be compared t o th e age in fo rm a tio n r e g a r d in g th e c l i n i c a l g roups p r e s e n te d e a r l i e r i n T able I I . The age ra n g e o f th e c o n t r o ls i s s l i g h t l y w id e r th a n f o r th e g rand t o t a l c e r e b r a l p a l s y sam ple e x te n d in g out a t each end. But th e t o t a l d i s t r i b u t i o n shows more bu n ch in g below age t e n , and t h i s d i f f e r e n c e i s e v id en ce d i n th e con t r o l mean d e p re s s e d some m onths below th e means f o r th e c l i n i c a l g ro u p s. The mean t h r e s h o l d s f o r e a c h fre q u en cy and b o th e a rs o f th e c o n tr o l group a re shown i n T able X I I I . W ith th e 105 n M I — I X H + 5 A H 3 Sll § T O O • C V 2 A • O œ p ^ c > O rH § O' ( Û 0 2 ^ rH t o ÎD to 0 2 u m + 5 w ) •H <o C D § T O O * 02 A O 02 MO § © &■ © 02 F -i rH f X 4 lO to t o 03 t o 1 — 1 t> î> • # ÎO ^ 1 C D to to C D • # 1 05 •s^ l o t> • • to iH rH Hi to 02 # # LO to rH C V 2 t o 02 t o rH • • C D lO 1 — 1 rH C O rH C D • • rH to 1 Hi t o to t o # ' * 02 rH 02 C O CO Hi » # 1 — 1 02 rH O ' lO LO 02 • • t o C N 2 CO to O D C O • # 1 to 02 O CD C \2 # # 1 to V» O O * ' * 02 H i 02 rH # *. t o 02 1 — i 02 O M rH M # * O ' Hi rH to 00 to 05 • * 1 lO 03 iH to 02 ' • # CO C O rH Hi • # t o 02 rH t o M O M • • Hi rH to o t o 05 • # 1 t o C O to 1 — 1 o 1 p - lO to ^ to ♦ • to t o M rH . * • O C D rH rH t o M t o C O • # rH to rH M 00 to o # # rH Hi 1 Hi O O M • # i — 1 LO to Hi O ' • # t o 03 02 G O 02 O CO * # to rH LO 02 02 to 02 ^ I to 05 rH to • • 1 C O 02 05 i— 1 to • # 02 rH C O C O t o 00 • # LO rH to 00 05 M • # CD t o t o o LO C O # • 1 to 05 LO to CD • • rH Hi C D t o t " to H» rH C D Hi CO rH ■ • • t o 02 to 02 M O • • 02 1 t o o t o 05 # « C O rH 00 sM rH « • t o 02 C O M to 02 • • t o 02 rH to to rH Hi # # CD Hi rH O- I — 1 LO rH to 1 Hi lO to C D • • rH o to rH C O # #. Hi rH 1 — 1 to O C O O t o to LO C O 02 to « • îO ^ 1 CD 05 t o 05 • • oo t o ^ 02 * * t o 02 02 to t o 02 O M 1 — 1 02 O ' t o LO to • # O Hi 02 02 t o 02 00 # • 02 1 tO 1 — I o t o # • rH t o o o • # O to t o M 02 C O • # t o iH to C O 02 LO # • rH LO 1 t o M 02 1 — 1 • • to rH to LO rH • • 1 — 1 1 — 1 02 to O Hi • # M to rH 02 O ' Hi 02 02 # * 00 Hi rH t o 00 t o C D • # 1 LO C D to t o to • • C D O to • * O O ' t o M 02 LO • « ( — 1 O M tO ( — 1 • • t o ^ t — 1 to • • to M rH to to # * 0 - Hi o t o # # 02 O rH 02 r> rH t o LO « # rH t o 1 — 1 02 to 02 to 02 LO 1 02 CD • # 02 rH t o 02 05 * # 02 rH rH t o to 02 C D 02 t o V» t o 02 • * rH 'sM 1 t o o rH J> • # I 00 O LO LO 05 • rH CD O ' Hi O • # o r> rH rH 02 to 1 — 1 to * • 02 Hi rH to to t o 02 # • 1 C D % > rH o to • • rH to o to rH • • 1 — 1 rH 1 — 1 C D to C D to 02 rH C O o C O C O • • 1 to rH % > rH to 02 05 1 to G O 02 to • • rH o to 05 to # . # CO Hi rH C O o O ' C D ; # * CD to 02 to 02 C O * * 02 C O O o rH 02 • • tO rH to 03 O' to • # 02 C O rH O to to # # to rH Ü • * 3 • « H to q # # -— - CD a A < 5 -4 O ' «H CD C A O cd O « H M cd A < 5 -1 O ' as < 5 h H i cd A < 5 h H i © • © • •H II © • -H I © • •H II © • •H II ^ CO S CO A • ^ CO A • ^ CO A • CO A • U «M « n o 'C S nd M - P 4 h T O •H rH p j o o T O o 45 O © o O rS •H O •H •H f l •H u O - P &4 45 45 Eh 45 T? EH 45 •H E H 45 P 4 T O gS © cd •H C d cd § o C Î3 Cd a m <d P h bC •H Cd n p Cd 106 e x c e p tio n of 1024 c y c le s , r i g h t e a r , a l l o f th e v a lu e s a re n e g a tiv e . The means a t 4096 c y c le s f o r b o th e a r s a r e th e m ost h y p e ra c u te v a lu e s , w ith th e means a t 1024 c y c le s p iv o tin g c l o s e s t to th e c a l i b r a t e d zero o f th e audiom eter* The e f f e c t o f th e n e g a tiv e v a lu e s was to in c r e a s e th e m agnitude o f th e d i f f e r e n c e s betw een th e means o f th e c l i n i c a l g roups and th e c o n tr o l group where th e c l i n i c a l groups means w ere p o s i t i v e . E s p e c ia l ly in f lu e n c e d w ere th e r e l a t i o n s h i p s a t 4096 c y c le s , b o th e a r s , and a t 256 and 512 c y c le s , l e f t e a r , s in c e th e mean t h r e s h o l d s f o r th e c o n tr o l group ra n g e d from - 2 .2 5 t o - 3 .7 5 a t th e s e fre q u e n c ie s * A lthough some e f f e c t i s o b se rv e d , th e i n c re a s e d d i f f e r e n c e s a r e n o t a s s t a t i s t i c a l l y s i g n i f i c a n t as m ight be e x p e c te d . T able XIV shows th e t r a t i o s of th e d i f f e r e n c e s b e tw een su b -g ro u p and c o n t r o l means w ith a d e t a i l e d c h a r t o f s i g n i f i c a n c e a t f i v e and one p e r c e n t l e v e l s . T h is t a b l e i s com parable to T able X II w hich p r e s e n te d th e same com pari so n , b u t w ith th e t o t a l p o p u la tio n c a l i b r a t e d z e ro norm. The d i f f e r e n c e s o f 3 .4 6 db and 3 .4 1 db betw een s p a s t i c s and c o n t r o l s a t 4096, r i g h t e a r , and 256, l e f t e a r , r e s p e c t i v e l y , a re now seen to be s i g n i f i c a n t a t th e f i v e p e r cen t l e v e l . But th e r e s t o f th e d if f e r e n c e s rem ain chance v a r i a t i o n s . 107 B I <si E H C O M G O T O T O ^ A G G M A T O 0 05 O M ^ {z; ^ rH © (D CD A îs; îz; A 02 © ^ O • # 05 M M # # ♦ # M H tO 1 — 1 rH IP 02 H A 03 • — 'C O H< o o O M T O T O 02 H G G CD M 0 G • H O O f2î îz; rH M © © A 02 % ÎZ! 0 % > ^ . m o • • # # M H . • • • 03 H* iH A 1 — 1 P t * cd O M H* to to G G C D £> T O T O 0 G 0 CO 02 G G -P MCV3 H to X îz; to C O t® r® 0 M % ^ 0 O D ^ % À O O • *. • « M M « # © P rH rH to H A H A © rH u< 02 05 o o t o 00 T O T O A A G G A T O 0 © 03 rH to s ^ 05 M P jD A A A H © ^ P rH • • • # M M « # ÎH 02 rH 05 to H A 02 rH C O T O o to to T O T O A G 0 A 0 T O T O C D sH rH © a rH H4 © © A A C O 0 © ,© lO # • M # • M M • « • # M M 02 t o 02 05 H* H A A C O C D T O G i> C O T O T O A H G G A M G G 05 H 02 © t o C D © © 0 A ^ îz; 02 00 % % O . # H M M • # # • H< t o 02 O H» H A i — 1 02 C O I — ! bO o G % > T O T O A A 0 G A O' G G « H* tO rH ^ Î2Î 02 02 © © A A ÎZÎ ^ 02 A ^ a m o • • M M # # • * • 02 1 — 1 rH 00 H» H A H « M M rH G G M rH T O T O 02 A G 0 A A G G 02 tO to % îz; lO lO © G M O ' 02 A îz; Î2 5 -p M O # • • • M M #. # • # & o rH Ü rH tO rH 02 H 02 •r4 © D* o lO G O 02 tO T O T O M H 0 0 A A 0 G © 02 lO 05 % % « — 1 tO G G 0 A A A îz; îz; îH rH * • • * M M . * # # # & H lO 1 — 1 02 H 02 H iH tO C D ■ G G C O o T O T O 0 M G G H 0 0 G C D 02 C D M 05 © © H 02 ^ îz; A A % îz; to # # # • M M * # - - « • . 02 rH 05 t o A H A H • iH rH 1 — 1 rH 44 -— ' H H 4 - 4 ) —1 rH W C D © © < + H 05 © © 44 o - © © 44 A © © «H J> > > •H O > > •H vt» î> > •H H* > > II © © A n © © A N © © 0 II © © A * f-9 (-4 A A A A 4-4 1 — 1 4-1 1 — 1 A H 4h rH • O • 0 • 0 • O ’ t o iH U t j A 1 — 1 J H T J A H d ^ A H Î - » — " 45 ' 4 5 ‘ — 4 5 * — ' 40 • # P # # d » '• d • • P O C a G G P Ü 0 0 d d 0 G d d O *H bO bO O H bO bO 0 H bO bO Q H bO bô O 4a •H *H •• 4 3 •H vH •• 4 5 • H H •* 4 3 •H *H •• Cd m C O T O Cd C O C O M c d A A Tf C d A A o ( n •H Cd 4 a p; © p4 •H G •H d 45 H 4 5 E H TJ EH •H Eh T O © •r-i A Cd x i bO S A 4 5 •H 0 C O m P 108 The d i f f e r e n c e s betw een th e r i g i d i t y c l a s s and th e c o n t r o l group rem ain sm a ll and i n s i g n i f i c a n t . The l e f t e a r of th e com bined g ro u p shows a one p e r c e n t s ig n i f i c a n c e a t 256 c y c le s , and a d i f f e r e n c e s i g n i f i c a n t a t th e f i v e p e r c e n t l e v e l a t 512 and 4096 c y c le s . The a t h e t o i d group a g a in shows th e v e r y l a r g e s t d i f f e r e n c e s , a l l f re q u e n c ie s g a in in g a d d i t i o n a l d e c ib e l s o f d e v ia tio n as t h e r e f e r e n c e d a ta becomes n e g a tiv e . The s i g n i f i c a n c e l e v e l s a r e a g a in w e ll beyond th e one p e r c e n t l e v e l d e m o n stra tin g t h a t th e d i f f e r e n c e s in mean th r e s h o ld s betw een a t h e t o i d s and c o n t r o ls co u ld n o t have o c c u rre d by chanc e . P re v io u s c a n p a ris o n s w ith th e p o p u la tio n norm had found no d if f e r e n c e s t o be s i g n i f i c a n t a t th e f i v e p e r c e n t l e v e l e x c e p t a l l t e n a th e to id :n o rm m e a su re s. In t h i s second com parison w ith th e c o n t r o l s , th e s p a s t i c : c o n t r o l d i f f e r e n c e s a t two f r e q u e n c ie s a r e s i g n i f i c a n t a t th e f i v e p e r c e n t l e v e l , t h r e e c o m b in e d :c o n tro l d i f f e r e n c e s f o r th e l e f t e a r a r e s i g n i f i c a n t a t f i v e p e r c e n t l e v e l demands, and one o f th e l a t t e r a t one p e r c e n t. In a d d i t i o n t o th e s e new n o n -ch an ce d i f f e r e n c e s , th e o th e r d i f f e r e n c e s w ere g e n e r a lly in c r e a s e d as were th e t r a t i o s . P o ly g o n s. F ig u re 5 shows th e fre q u e n c y polygons o f o > o I— I o * L O to Q o o > o to C O to H^ococo ^ M to 0 » o L O < D L L O lO. o L O C O o > t o o tr{ W < 3 5 C C Î M S O I^otoco E S 110 th e a i r co n d u c tio n th r e s h o ld s f o r each fre q u e n c y f o r s p a s t i c s u b j e c t s . S e p a ra te f i g u r e s have been drawn f o r each e a r w ith c o n n e c tin g l i n e s b etw een th e means o f each fre q u e n c y . S im ila r polygons a r e shown f o r th e a t h e t o i d sub j e c t s in F ig u re 4 , f o r r i g i d i t i e s in F ig u re 5 , and f o r th e combined d ia g n o s is group i n F ig u re 6 . In te r -c o m p a r is o n s f a c i l i t a t e d by th e superim posed p o lygons i n F ig u r e 7 m ani f e s t th e d i f f e r e n c e in th e c h a r a c te r of th e d i s t r i b u t i o n s su g g e ste d b y th e means and e s p e c i a l l y th e s ta n d a rd d e v ia tio n s in T able X I. The s p a s t i c and r i g i d i t y groups te n d to d i s t r i b u t e th e m se lv e s r a t h e r n o rm a lly around th e m ean. A s l i g h t te n d ency to r e s t r i c t t h e n e g a tiv e end o f th e d i s t r i b u t i o n i s in h e r e n t i n t h e e x p e rim e n ta l m ethod, s in c e th e low er l i m i t o f i n t e n s i t y o u tp u t i s a r b i t r a r i l y -1 0 d e c ib e l s by a u d io m e te r c o n s t r u c t i o n . W hether th e t h r e s h o l d o f -1 5 o r -2 0 d e c ib e l s co u ld be d e r iv e d i f th e au d io m eter were so c a l i b r a t ed i s , of c o u rs e , unknown, b u t c l i n i c a l e x p e rie n c e w ith o th e r equipm ent s u g g e s ts t h i s p o s s i b l i t y f o r a few s u b j e c t s . There i s no re a s o n to b e l ie v e t h a t h y p e r a c u ity would e x i s t any more in one g roup th a n i n a n o th e r . The a t h e to i d polygons d i f f e r from th o s e f o r o th e r g ro u p s. They a r e m ark ed ly skewed a c c o u n tin g f o r th e i n c r e a s e d s ta n d a r d d e v i a ti o n s . T h e ir ra n g e i s c o n s id e r a b ly o » O o co L D O c 3 H C Q t O ^ W M ^ 0:; M 5 Ü t-^O cacO L D o a > in I - ( - > 3 E -4 o A ‘ g & H C . H m •H L O ' O ü) o O LO o o rH o o M M < p; M c h ^ ocqw to o> o C Q K M < Pi H O to o o O o O O C O C O in XJ to £^ C O C T > to r o > a H s | + » 1 0 $ o C\3^ tn a. o § § & 0 £ i§ C Q lO I HI C O O ■ W ii lO lO •H I — I in W W <1 Pi M 5^ O h^OCQCO A + > •3 lO If) o o If) o c O iH O J W t i J ^ p c J M S O h^ O cOCQ o < £ > S CO S' C j •g& iH C o o * lO o o o If) W W «a; pc5 M ^ Ô h^ O cocO ( 3 4 •H p H O H I a o iH N rH v-l (D H i O t H C O 4 3 f H Ex w'i ^ o 1 « — 1 ------- 1 - 8 o c n 2 o VP» a 8 R ü > O CO CO - I o C J f-H vr» V O â I CO O t * Ü I ? I Q > & f I O s t) tj v H « 4 4 » 0 ) X 44 0 < H 0 0 T O 4 3 T f T O C l L T O .M ^ A -*î« O S { D W - 4 « i - i Î 2 J c !> CM S O o ^ \f V M O CO CO o o to * Ü & 6 I :8 115 w id e r th a n f o r o th e r g ro u p s b u t th e e x te n s io n i s o n ly i n th e downward d i r e c t i o n on th e o r d i n a t e . Her© w© see g r a p h i c a l l y d e t a i l e d th e e f f e c t o f th e g r e a t e r h e a rin g lo s s v a lu e s as th e y te n d to d i s t o r t th e norm al b e l l- s h a p e d d i s t r i b u t i o n . A verage o f th e Speech F re q u e n c ie s , A n o th er m ethod o f d e s c r i p t i o n f o r a u d io m e tric d a ta i s th e av e ra g e o f th e .th r e e m id d le f r e q u e n c i e s , 512, 1024, and 2048 c y c le s p e r seco n d . A c u ity f o r th e s e th r e e f r e q u e n c ie s seem t o be more r e s p o n s i b le f o r th e th r e s h o ld a c u i t y o f a h e a rin g f o r sp e e c h , and t h e i r a v e ra g e i s o f te n em ployed a s a d e s c r i p t i o n o f th e h a n d i cap * When th e a v e ra g e o f th e th r e e f r e q u e n c ie s i s ta b u l a t e d a c c o rd in g t o c a t e g o r i e s o f l e v e l s o f h e a r in g l o s s , th e d a ta groups i t s e l f a s in T able XV, :, More th a n t h i r t y - s i x p e r c e n t of th e a t h e t o i d sub j e c t s showed lo s s e s o f more th a n t e n d e c ib e ls on each e a r , w hereas o n ly h a l f o f t h a t p e rc e n ta g e o f com bined c a te g o ry p a ls y av e ra g e d below te n d e c ib e ls in th e sp eech r a n g e . The l a r g e s p a s tic group h ad o n ly 5 ,3 p e r c e n t and 2 ,6 p e r c e n t 2 2 9 ^ a lte r Hughson and Eva Thompson, '^ C o rre la tio n s o f H earing A c u ity f o r Speech w ith D is c r e te F req u en cy A udiogram s," A rc h iv e s o f O tolaryngoloC T . 3 6 :5 2 6 -5 4 0 . O cto b e r, 19421 ----- 1 1 6 H m g T f S § * 3 0 5 • H S S < 5 5 o b Û P ' H 5 ' P 4 E 4 H » c / ] 4 a : « H C D < J L , © ^ $ l f o 1 ^ 0 3 O ^ a 0 3 , © + ) < u s i 0 3 E h b O Ü P Q • H c d M P h K : • * W o 0 3 4 5 O M > ^ 0 - 4 r H O 4 5 © • H p j 0 5 T S 0 3 • H 4 5 r H C C I b O j : 1 0 0 m O • H b O * — 'fx P 4 . H « C D • % M g O K 4 5 s p OT 4 4 ^ C D • H © C O t D < 4 W P I 0 3 O M S +5 ^ © C O r C b Û 0 3 O 4 5 » r 4 m H < 4 c d i l M P S i > % , 4 5 4 5 4 4 o • H © o 0 P 3 t o o •H K P P H w © Si t o C Î 3 < 4 cri b û t o < 4 % P ^ » H M 0 C O M > o < 4 E 4 M © © 0 ro P I m P 4 © b û P d 4 4 v 4 0 J 4 0 Cri rH © X» © w 'O > © 0 P H 03 S a è -p © u o î§ o 03 © O X ) X* X3 nO T f 'O 0 0 ' 0 0 t o tO co S i 0 P: G cri c ri cri (ri X3 X I X î X î P P P 4 5 © © © © S4 u P h 0 0 0 0 S s g S 117 f o r th e r i g h t and l e f t e a r , r e s p e c t i v e l y , below th e t e n d e c ib e l m a rg in a l l i n e . When a more c o n s e r v a tiv e m a rg in a l l i n e of h an d icap i s a r b i t r a r i l y chosen a t tw en ty d e c i b e l s , th e r e a re s t i l l more th a n o n e - f o u rth of th e a t h e t o i d group t h a t f a l l i n to th e h an d icap p ed g ro u p . E xcept f o r one s u b je c t in th e combined group and one e a r in th e s p a s t i c s , th e r e a r e no o th e r e a r s t h a t a re below th e tw en ty d e c ib e l l i n e when th e th r e e speech f r e q u e n c ie s a r e a v e ra g e d . Spondee Word T e s t M easures. S ix te e n o f th e 101 s u b je c ts who w ere a b le to m eet th e demands o f th e p u re to n e t e s t s i t u a t i o n were u n a b le t o re sp o n d r e l i a b l y t o th e re c o rd e d spondee w ords. The m ajor demand o f th e exp erim en t a t t h i s p o in t was th e a b i l i t y t o u n d e rs ta n d and r e p e a t th e words h e a rd . S u b je c ts who c o u ld nod o r shake t h e i r h e a d s , say "Yes" o r " F o ,” or b y some means make known a p o s i t i v e o r n e g a tiv e judgm ent w ere n o t alw ays a s s u c c e s s f u l w ith word r e p e t i t i o n . F iv e of th e s p a s t i c s , e i g h t a t h e t o i d s , and th r e e o f th e combined c a te g o r y f a i l e d when th r e s h o ld o f word u n d e r- s t a n d a b i l i t y was r e q u i r e d . In some i n s t a n c e s , th e p a r a l y s i s pro v ed to o u n y ie ld in g t o a llo w r e p e t i t i o n o f such words as " b a s e b a l l ," "e y e b ro w ," " s c h o o lh o u s e ," " c h u r c h b e ll," e t c . , so t h a t th e y 118 co u ld b e re c o g n iz e d by th e ex am in er. Two of th e com bined group spoke S p an ish w e ll b u t c o u ld n o t u n d e rs ta n d o r r e p e a t th e " f o r e ig n " la n g u ag e of th e spondee t e s t . T hree of th e group w ere s u s p e c te d to h av e a s tr o n g em o tio n a l w ith d ra w a l b a s ic to a s p a r s i t y o f e x p r e s s io n th ro u g h sp eech . In a t l e a s t one s u b j e c t , a la n g u ag e d i s a b i l i t y r e s u l t i n g from th e se v e re h e a r in g lo s s p lu s an e d u c a tio n a l h i s t o r y in s c h o o ls f o r th e d e a f where o n ly th e s ig n lan g u ag e was t a u g h t, h a n d icap p e d lan g u ag e u n d e r s ta n d in g . I t was f e l t t h a t two o r more o f th e s e background f a c t o r s w ere o p e r a tin g in a t l e a s t f i v e o f th e s ix te e n s u b je c ts i n q u e s tio n . The i n te r - g r o u p com parisons of spondee m easures can be exam ined in T ab le XVI. A gain, th e means o f th e a t h e t o i d e a r s a r e seen to d e v ia te s i g n i f i c a n t l y from th e norm. W ith th e F re d u c e d t o t h i r t y - t h r e e s u b j e c t s , th e mean th r e s h o l d h e a rin g im p airm en ts f o r spondee m a t e r i a l a r e s l i g h t l y o v er t h i r t e e n d e c ib e ls f o r each e a r . These v a lu e s a re a g a in c o n s id e r a b ly h ig h e r th a n c o u ld o ccu r by chance as in d ic a t e d by th e t r a t i o s of 4 .1 1 0 and 4 .8 9 5 f o r r i g h t and l e f t e a rs r e s p e c t i v e l y , w e ll beyond th e one p e r c e n t l e v e l o f s i g n i - f ic a n c e . The means o f th e o th e r g ro u p s a r e a l l above z e ro , ra n g in g from 2 .7 3 d e c ib e ls f o r th e s p a s t i c r i g h t e a r to 119 g I m o p t o o t o 0 3 4-1 P o 0 3 c o T ) < D # ' # • • © ■ — * » P | C O l O 0 3 p Si 0 0 •H I I . l O o 1 — 1 0 5 c o o b O 0 3 o 0 5 O * H ■ • • • • A t o l O t o 0 3 P 4 4 P H ~ © 0 C V 2 P î 1 ^ P Î 4 - - ^ X ! E - t bO •H K P o p l O p t o o p o c o t o 44 p o C D o >> © • # • • P P < o L O l O t o v4 '— " d o > •H 1 P b O % X î o c o o •ri « w bO o 05 p . • • # c q s j j l O t o 0 3 4 ^ t O o p O © 44 t o o 05 •H - — ' © « • # W î O P t o o t o O t O p p p p II © p X Î ' - ^ X Î 0 0 o 0 3 P p bû p o p P « a l " H • • * M t o t o c o P p t o o p t O t o P 44 t o o o % > P ^ © « • • • O Î O P t o t o t o t o •H t O P I I © îs; P cri — x î t o o t o t o A bO o 0 3 05 C D * H • # • 0 3 t o t o 0 3 Si § •H A O P P cri « cri © © a C D E4 120 8 .1 3 f o r th e com bined c a te g o r y l e f t e a r . These d a ta d i f f e r from th e p u re to n e m easu res where means c lo s e t o z e ro and even some n e g a tiv e v a lu e s were seen (T ab le X I, Page ). F o t o n ly a re th e mean l o s s e s f o r spondee words h ig h e r v a lu e s , b u t th e means ap p ro ach c l o s e r to s i g n i f i c a n t d e v ia tio n s when the t t e s t i s a p p lie d th a n do th e p u re to n e m easures f o r th e same c a t e g o r i e s . B oth s p a s t i c e a r s a r e beyond th e one p e r c e n t l e v e l o f s i g n i f i c a n c e . The r i g i d i t y e a r s a r e betw een t h e t e n t h and f i f t h p e r c e n t and betw een th e second and f i r s t p e r c e n t s i g n i f i c a n t l e v e l s f o r r i g h t and l e f t e a r s , r e s p e c t i v e l y . The combined g roup r i g h t e a r h a s b e t t e r th a n a f i v e p e r c e n t l e v e l o f s i g n i f i c a n c e , th e l e f t e a r o n ly betw een tw en ty and te n p e r c e n t. P o s s ib le re a s o n s f o r th e d i s p a r i t i e s betw een p u re to n e and spondee d a ta ca n be c o n je c tu re d o n ly . The spondee word t e s t was s ta n d a r d iz e d w ith a d u l t s ; no a tte m p t was made t o g ra d e v o c a b u la ry down t o th e age l e v e l s o f th e av e ra g e o f o u r sam ple (a p p ro x im a te ly te n y e a r s o l d ) . A m atched sam pling of "norm al" c h ild r e n w ere u n f o r tu n a te ly n o t a v a i l a b l e f o r a com parison of spondee d a t a . The p o s s i b i l i t y rem a in s t h a t th e d a ta may be demon s t r a t i n g a sm all s y s te m a tic la n g u ag e h a n d ic a p i n t r i n s i c to th e " s i t u a t i o n " and environm ent o f most c e r e b r a l p a ls y c h i l d r e n . I f th e r e i s such a h a n d ic a p , th e spondee word 121 t e s t does n o t seem b a s i c a l l y d e s ig n e d t o be s e n s i t i v e enough f o r i t s d e s c r i p t i o n . A lth o u g h th e o th e r groups show sm a ll l o s s e s , th e com parison w ith .th e a t h e t o s i s c a te g o ry s t i l l rem a in s c o n s is t e n t w ith t h e p u re to n e d a t a . Hot o n ly a re th e a t h e t o i d mean v a lu e s and " t" r a t i o s c o n s id e ra b ly l a r g e r th a n th o s e f o r o th e r g ro u p s , b u t a t r a t i o com parison o f th e d i f f e r e n c e betw een a t h e t o i d and s p a s t i c means r e v e a ls "v e ry s i g n i f i c a n t " v a lu e s o f 3 .1 3 and 3 .4 9 f o r r i g h t e a r and l e f t e a r , r e s p e c t i v e l y . The 3p a s t i e s were chosen f o r th e com parison f o r t h e i r l a r g e r sam pling and p o s s i b ly g r e a t e r s t a b i l i t y w ith a some w hat s m a lle r s ta n d a r d d e v i a t i o n . These r a t i o s c l e a r l y i n d i c a t e t h a t th e a t h e t o i d and s p a s t i c sam ples c o u ld n o t have b een drawn from th e same p o p u la tio n . R ight B ar- L e f t E ar C o r r e l a t i o n s . A lthough th r e s h o l d s of th e two e a r s w ere d e r iv e d s e p a r a te ly and h a n d le d s e p a r a te l y to a llo w in d e p e n d e n t d e s c r i p t i o n s o f each e a r , th e d e g re e o f c o r r e l a t i o n betw een th e two e a r s was o f i n t e r e s t . ' T ab le X Œ d e ta ils th e means of th e th r e s h o l d s f o r each e a r de r i v e d from t h e e n t i r e 101 s u b je c t sam ple, th e s ta n d a r d de v i a t i o n o f th e d i s t r i b u t i o n s , and th e P earso n p r o d u c t- moment c o r r e l a t i o n b etw een th e two e a r s a t each fre q u e n c y . D if f e r e n c e s betw een th e two means a r e o f th e m ag n itu d e of l e s s th a n one d e c ib e l a t a l l f re q u e n c ie s 122 TABLE XVII COMPARISON OP RIGHT EAR A M D LEFT EAR FOR AIR CONDUCTION PURE TONE AND SPONDEE DATA F requency M g lit E ar Mean 8.D# L e f t Mean E ar 8 .D . P ro d u c t- Moment r 256 5 .2 0 11.9 6 5 .6 1 1 1 .0 1 .7 9 512 4 .5 5 1 3 .9 6 .'5 .6 6 1 5 .0 6 .8 7 1024 5 .1 5 1 5.40 4 .6 0 1 5 .1 2 .8 7 2048 7 .0 5 19.47 6 .4 9 1 7 .2 5 .8 4 4096 7 .0 8 2 0 .5 4 6 .2 8 1 8.40 .8 1 Spondee 7 .4 1 12.8 4 8 .0 6 1 1 .7 4 .9 0 123 e x c e p t 256 c y c le s where 1 .5 9 d e c ib e ls s e p a r a te s th e two m eans. The s l i g h t l y l a r g e r d i f f e r e n c e betw een th e means a t 256 c y c le s may r e f l e c t an i n e q u a l i t y o f u n i l a t e r a l im p a irm e n ts t o one e a r o r th e o t h e r . The g r e a t v a r i a b i l i t y o f a b s o lu te th r e s h o ld s seen i n th e s i z e o f t h e s ta n d a r d d e v i a ti o n s i s a f u n c tio n o f th e l a r g e r a t h e t o i d v a lu e s w hich d i s t o r t th e t o t a l d i s t r i b u t i o n . The product-m om ent c o r r e l a t i o n s ra n g e from .7 9 to .8 7 f o r th e p u re to n e s and r i s e t o a h ig h o f .9 0 f o r th e spondee m e asu res. C o n sid erin g th e number and d eg ree o f h e a rin g l o s s e a r s and th e p o s s i b i l i t y o f u n i l a t e r a l im p a ir ment w ith norm al h e a rin g in th e o p p o s ite e a r , th e m agnitude o f c o r r e l a t i o n i s alm ost s u r p r i s i n g , a lth o u g h th e h ig h p o s i t i v e r e l a t i o n s h i p was e x p e c te d . The c o r r e l a t i o n o f .9 0 f o r sp eech r e c e p tio n th r e s h o ld s s l i g h t l y s u rp a s s e s th e p u re to n e c o r r e l a t i o n s . A lthough th e d i f f e r e n c e i s s m a ll, t h i s may d mi ons t r a t e th e i n t e g r a t i v e c h a r a c te r o f th e added c e r e b r a t i v e a c t i v i t y in v o lv e d in th e word r e c o g n i t i o n problem o f th e SRT t e s t . T en sio n v s . Hon- T en sio n A th e t o id s . The h y p o th e s is was c o n s id e re d t h a t h e a rin g a c u ity d if f e r e n c e s o b se rv e d i n th e a t h e t o i d s c o u ld be r e l a t e d t o th e s t a t e of te n s io n in th e a t h e t o i d . The r e l a t i o n s h i p o f t e n s i o n in th e a t h e t o i d to f a c t o r s of a t t e n t i o n and f a t i g u e , a c t i o n o f m e d ia l e a r 124 m u sc le s, and o th e r p o s s i b le p h y s io lo g ic a l adm ustments p e r t i n e n t t o h e a rin g a c u i t y h a s n o t been th e s u b je c t of p r e v io u s stu d y . A b i s e r i a l c o e f f i c i e n t o f c o r r e l a t i o n was u se d t o d e s c r ib e th e r e l a t i o n s h i p betw een te n s io n o r la c k of te n s io n and h e a rin g a c u i t y . I t was f e l t t h a t th e v a r i a b l e of te n s io n i s r e a l l y a co n tin u o u s v a r ia b le t h a t h a s b een a r b i t r a r i l y re d u c e d t o two c a t e g o r i e s . The b i s e r i a l r i s d e sig n e d to d e s c r ib e th e c o r r e l a t i o n betw een two c o n tin u o u s v a r i a b l e s where one i s a r b i t r a r i l y made dichotom ous. T ab le XVIIshows th e mean a v e ra g e v a lu e s f o r te n s io n and n o n - te n s io n a t h e t o i d s w ith th e b i s e r i a l c o r r e l a t i o n s o f each p a i r o f groups th e y r e p r e s e n t . The p r i n c i p l e upon w hich th e fo rm u la f o r a b i s e r i a l r i s b a se d i s t h a t th e l a r g e r th e d i f f e r e n c e betw een means th e l a r g e r th e c o r r e l a t i o n , w ith no d i f f e r e n c e betw een means y i e l d i n g a ze ro c o r r e l a t i o n . The c o r r e l a t i o n v a lu e s shown i n T able XV IIare sm all and do n o t su g g e st a r e l a t i o n s h i p betw een th e te n s io n d e s c r i p t i o n and h e a rin g a c u i t y . P ure Tone Bone Conduct ion M easures. Pure to n e bone c o n d u c tio n t e s t s were a d m in is te re d t o f o r t y - s e v e n e a r s o f tw e n ty -n in e s u b je c ts who showed t h r e s h o l d l o s s e s f o r a i r co n d u cted p u re to n e s o f f i f t e e n d e c ib e ls o r more a t two f r e q u e n c i e s , or tw e n ty d e c ib e ls o r more f o r one fre q u e n c y . 125 H H H m g EH 1 © to 40 p; 0 o> 03 40 rH O' o 0 * 03 • rH O PiTri 03 • C D 1 — 1 rH co 05 to O- ■ o 05 O' O C O . 03 • rH rH !> 1 — 1 # —^ 1 — 1 rH 1 • 00 © vrit C O rH • O o £> - 03 to P i 03 • 03 • rH rH u • o to • cri Ü rH rH 1 m 03 05 î»o O £> O' C O 4h O r— 1 . • 03 • rH o 0 P 05 to • A 0 1 P 03 03 O ' O^iH lO o o ^ 05 0 LO • 03 • rH o U G O to « 1 (O lO 03 O ' 03 03 0- O to • 03 * rH o - l> to • 1 © 1 P 0 O 0 o to P iT j LO 0 - lO o - C D é 03 • rH o 03 • t o rH rH 05 o 05 05 C D (> 03 ^ o • 03 • rH rH C O • • rH ! — 1 1 © 00 • sjH 05 1 — 1 P i O 00 O ' 03 ^ t o A • CJ * 03 ♦ rH rH cri o 00 to • M rH rH 1 x> î> rH o 03 O O' 03 Hi C O bo a O • 03 • iH 1 — 1 •H 0 ) — 1 C O • Pri P 1 — 1 1 C T * 0 C O O- P O ' 40 Hi o (il 03 • 03 ♦ rH rH rH rH CO • lO 1 — 1 1 to O to to O ' O Hi O ' LO * 03 • rH o 03 00 O • rH bO P •H P C r i p p P ^ 0 o cri cri P tP •H © 0 © S rH P P P C ri O © o •H «iH EH •H P © 1 © 0 P P . P © © O © •H Eh S EH m 126 The d i f f i c u l t i e s u s u a l l y e n c o u n te re d i n o b ta in in g v a l i d and r e l i a b l e m easu res o f bone c o n d u c tio n a c u i t y , such as v a ry in g th ic k n e s s o f a d ip o s e t i s s u e , c o n d u c t a b i l l t y o f te m p o ra l bone, l a t e r a l i z a t i o n around th e h e a d , a i r co n d u ct io n d i f f e r e n c e s betw een th e e a r s , e t c . , c o u ld n o t be a v o id ed# Masking o f th e o p p o s ite e a r was u s e d w h erev er i t co u ld be t o l e r a t e d by th e s u b je c t i n an a tte m p t to c o n t r o l th e l a t e r a l i z a t i o n phenomenom. Amount o f m asking fo llo w e d th e recom m endations o f th e Audio Development Company^^^ f o r th e u se o f t h e i r equipm ent* The m asking p ro c e d u re s and a d d i t i o n a l f a t i g u e in v o lv e d i n a second m ajor t e s t i n g sequence made bone c o n d u c tio n m easurem ents d i f f i c u l t f o r some o f th e group* Moments of r e s t came more f r e q u e n t l y and, i n a sm a ll num ber, " r e s t l e s s n e s s " was observed# D e s p ite th e c l i n i c a l problem s in v o lv e d i n th e m easurem ent o f th e h e a r in g of bone co n d u cted s t i m u l i , i t was f e l t t h a t r e l a t i v e l y r e l i a b l e m easu res had been o b ta in e d . U n i l a t e r a l and B i l a t e r a l M arginal L o s s e s . T ab le XIX'r shows t h e number o f s u b je c ts i n each su b-group who y ie ld e d 230 B u l l e t i n No. 38 (M in n e a p o lis: Audio Developm ent Company, 1 9 4 6 ), p . 1. 127 X IH X m g w iH X> cri o 4J 0 O E> C O 1 — I l — 1 rH C D EH X» rH 03 P C O A < m EH '"«H^ f4 , cri H U P Q 0 -P Q cri. tO iH iH O) ^ C D rH iH rH < H fH co m A m O g A EH A <4 ^ x> i 4 Iz; H! fH 1 — i cri 0 CO Oi LO ^ O U A A 04 0 -P fH W cri O rH •H +3 m f l X! p A b O rH C V J iH îs; •H H 0 4 Q M Q jsî H © 'P O *H 43 0 •H O *H p P 43 -P »tS O *H rH © 0 *H P X cri C ri X b O 0 g 43 A -p "H u o o co < ri 0 4 E h o EH 128 a i r co n d u c tio n f in d in g s t h a t i n d ic a t e d bon© c o n d u c tio n e x p lo ra tio n * N in e te e n s u b je c t showed b i l a t e r a l l o s s e s o f a m a rg in a l m agnitude or above f o r bone c o n d u c tio n te s tin g * F o u rte e n o f th e s e w ere a t h e t o i d s co m p risin g th e l a r g e s t g ro u p , th r e e w ere s p a s t i c a , w ith th e trem o r and combined groups y i e l d i n g one each* U n i l a t e r a l l o s s e s were r e v e a le d i n n in e a d d i t i o n a l s u b j e c t s . Four s p a s t i c s , f o u r a t h e t o i d s and one r i g i d i t y t o t a l l e d t h i s g ro u p . Com parison o f th e u n i l a t e r a l and b i l a t e r a l lo s s groups o f f e r s a n i n t e r e s t i n g stu d y . I n a l l p r e v io u s f i n d in g s t h e a t h e t o i d s te n d e d t o d e m o n stra te th e more extrem e d e v i a t i o n s , b u t breakdow n by u n i l a t e r a l vs* b i l a t e r a l im p airm e n t does n o t fo llo w t h i s c o n s is te n t l i n e . The a th e t o i d s show f o u r te e n b i l a t e r a l lo s s e s a s com pared to th r e e b i l a t e r a l in s ta n c e s in th e s p a s t i c g ro u p , b u t th e u n i l a t e r a l l o s s e s a r e e q u a te d a t fo u r l o s s e s ea c h f o r th e s p a s t i c and a t h e t o i d c a t e g o r i e s . I t would seem t h a t , w ith r e s p e c t to number o f u n i l a t e r a l l o s s e s , th e a t h e t o i d s do n o t d e v i a te s i g n i f i c a n t l y from th e s p a s t i c g ro u p . But th e m agnitude o f d e v ia tio n in q u a n t i t y o f b i l a t e r a l l o s s e s i s c l e a r and d e f i n i t e * S p e c if ic BC T e s t R e s u lts . Four o f th e tw e n ty -n in e 129 s u b je c ts i n th e l o s s group w ere u n a b le to o f f e r r e l i a b l e re s p o n s e s to th e bone c o n d u c tio n t e s t i n g p ro ced u res* F a tig u e f o r c e d a h a l t to th e m easurem ents o f th e second e a r o f one a d d i t i o n a l s u b je c t w ith b i l a t e r a l im pairm ent* A ll f o u r of th e above w ere a t h e t o i d s who had b i l a t e r a l lo s s e s * A th e to id s u b je c ts Ho* 10 and Ho* 30 were to o t i r e d to c o n tin u e th e t e s t i n g p ro cess* A lthough A th e to id No. 21 seemed a c t i v e and i n t e r e s t e d , th e t e s t i n g was h a l t e d when sw eat began to b lin d him d e s p i t e e f f o r t s to keep him c o o l and co m fo rtab le* A th e to id Ho* 32 was f o r c e d to d e p a r t b e f o r e bone c o n d u c tio n t e s t i n g co u ld be done, in o rd e r to u se Red C ross t r a n s p o r t a t i o n to h i s home, th e o n ly t r a n s p o r t a t i o n p o s s ib le * Hone o f th e s e s u b je c ts c o u ld be r e t u r n e d f o r f u r t h e r s tu d y due t o h o s p i t a l c l i n i c p o lic y . S in ce th e s i g n i f i c a n c e of th e bone c o n d u c tio n m eas urem ent i s l i m i t e d w ith o u t r e f e r e n c e t o th e a i r c o n d u c tio n t e s t on th e same s u b j e c t , i t was d e c id e d n o t to group th e bone co n d u c tio n data* A ll of th e d i s c r e t e m easures d e r iv e d by bone co n d u cted s t i m u l i a r e i n s t e a d p r e s e n te d in company w ith th e a i r co n d u c tio n v a lu e s f o r th e same s u b je c ts i n T ab le /.X’Xl* The a b s o lu te and r e l a t i v e p o s i t i o n s o f th e a i r and bone c o n d u c tio n m easu res f o r any s u b je c t c o n t r ib u t e s i g n i f i c a n t l y t o th e c r i t e r i a em ployed by th e o t o l o g i s t in th e judgm ent o f p a th o lo g y . T h is r e l a t i o n s h i p w i l l be b a s ic 130 C D P 4 A O B C D C D . O A A .. - 2 5 1 — 1 e g î H o X C D î > 4 M A H X A < P Q > « 4 E h O M A O O M t H < 4 i o P Q C D X X X X X X O X X X X o 05 1 1 03 03 03 1 — 1 X I 03 X X —' O 1 • ^ w • co O o X X X X o o X O o o 03 H rH 1 — l rH «H 1 X X ^ • O 1 1 cri O C \3 T O T O T O T O T O T O X X O O O X X O O O O o o o o ' 43 M (M rH 1 — 1 rH 03 .— i rH «H x ^ (M O O 0 p rH o O o A © % S p 03 D^rH O X O O X o X o O X X X 0 X ( — 1 1 — i 03 X rH 1 iH 03 03 . f n 1 ( H C O X o o X O X O X o X X O O 03 rH 03 03 X rH i 03 rH 43 m 0 O O o o O O O O O p o o P O p o O o Eh P Q < 4 P Q < 4 P Q < 4 P Q < 4 m < 4 P Q < 4 P Q <4 P Q < 4 m <4 t o O O X o O X o o X X o X o X 05 i — i rH 03 03 1 X rH 03 £> X ' - ' O 1 • -çfl T O • C O o X X X X X X X X o O o o o rH 1 03 1 — l rH «H i t o O ' U * o tri ü 03 T O T O W — T O T O O O X O X X X X X O X o o X X X 43 >503 rH 03 iH X rH rH 1 rH 1 X ^ X O O 1 bO P rH O O Ti 0 Î2i M 04 P 03 D^rH O O X O O X o X o o O o X X 0 lO 03 X X 03 rH H rH iH 03 X P 1 1 k m lO X X X O X X X X X o X X o o 03 03 X X rH iH rH rH 1 1 03 rH 43 T O 0 O o O Q o o P P P P P O o o o o O p EH PQ < P Q < 4 P Q < 4 PQ <4 P Q < 4 P Q < 4 m < P Q < 4 P Q < 4 43 O f i O M O 0 SH Eh •f“5 C D H X W P PQ rH t o 0 3 to 03 EH rH X C D C D 0 5 rH 1 — 1 03 X < 4 151 T ri < D H 4 ^ d o ü M 5 § E 4 co i g co co o A A g M e P 4 è co I 0 § M £ - 4 g 0 p R s 1 m < o X o X X O O o O o O o X X O O X 0 5 0 3 0 3 0 3 t M X t O t o X X 0 3 0 2 t M X . X X t M • T r i • T O 0 • C O X X O X P O o o X o X O O o X X O O X X X X b O X t o X X 0 3 r H 0 3 0 3 Si t m d • O • H c r i O 0 2 4 3 o H '-' 0 T O o o o X X V i X X o X X o o O O O O T O 4 3 O 4 3 > 5 0 3 0 3 0 3 1 0 3 X X X r H 0 3 0 2 0 3 r H X t H O 4-10 0 4 3 r H 4 3 0 d r H ■ d d A 0 0 O 0 P 0 3 • H f i O ^ H I O O X o r H O o o O O O X O O X X 1 — 1 O 0 X 0 2 0 3 1 O 0 3 X X 0 2 0 2 0 2 0 2 0 3 r H r H p X d k C D X O O o X O X X O O O X X O X X X 0 2 r H r H r H 1 0 3 X X 0 3 0 2 0 2 1 — 1 r H 1 X 4 3 T O 0 P P O U O O p p O P O P P O P O P O p p E H P Q < 4 P Q < 4 P Q < P Q < 4 P Q < 4 P Q < 4 P Q < 4 P Q < 4 P Q < 4 P Q < 4 C D X O O X X X X X X X X O X X O O 0 5 X < o X t o t o t M X X 0 - O t M X <-^o • T O • 0 0 X X T r i X X X O X X X X O O X O O • X A T M t M X © X t o t o X t M X X t o X X 4 3 X d • O p o c r i O 0 2 b O si k ''- T O • H O O T O 4 3 O X X O X o X o X o X O X o X 4 3 > 5 0 2 0 3 X O c r i X t M X X 0 2 0 2 0 2 X X 0 3 0 2 r H 1 o X h O O r H 4 4 1 4 3 b O d H • H 0 O 4 3 4 3 k P 0 2 d d C M f H X O 0 X X X O O O O X X O O X X 0 o 0 X r H 0 3 • H 0 3 X X X X X 0 3 0 3 X 0 2 0 3 1 1 ' r * f H r H 1— 1 k m P P X O O X o o X o O O X X X X o X X 0 2 I — 1 1 — 1 0 3 0 2 X X 0 3 0 3 0 3 0 3 I — 1 r H r H 1 t M 4 3 T O . 0 P P P P P O o o P P U P O O P O p p O P E H P Q < P Q < 4 P Q < 4 P Q < 4 P Q < 4 P Q < 4 P Q < 4 P Q < 4 P Q < 4 ■ m < 4 4 3 O 0 • i ~ o X p t o 0 0 O r H 0 3 X c o 0 5 O r H X 1 — 1 1 — 1 r H 1 — 1 r H i * H 0 3 0 3 132 T r i 0 g •H 43 d o o X 8 A g tD i g c o C D O A A g î — I O k ë co A g. M I g P M O A m 0 0 0 X X X 0 X X X 0 0 0 5 A m A • 0 2 X TM X 03 0 2 Tri • * t H 0 0 T O d rH • A X X 0 X b O X X 0 X 0 X X q 0 A T M iH X o - •H tM 0 t M tM < 0 1 1 —1 1 —i d • 0 4 3 rH T O cri ü O î T O 0 *H T O f q w T O 4 h cS 0 0 X X 0 0 0 > X . 0 0 rH X X X X + 5 > 5 0 2 iH rH tM 0 4 3 0 0 2 X X rH t—1 C Q t 0 0 d d 0 0 d M 0 0 d A 0 îs ; •H d 0 3 rH 4 3 CMrH 0 X 0 0 P X d 0 0 0 X X X X 0 X X O ' 0 rH 0 3 X rH 0 2 0 3 d . *H k m rH X 0 0 0 0 X P X 0 X X X X X 03 X X rH 0 2 0 2 rH 0 2 02 4 3 T O 0 P p 0 P p p P P P P P P P P P P P P EH A < 4 A < 4 A < 4 A < 4 A < 4 A < A < 4 A < 4 A < 4 t o X X X 0 X 0 X X 0 X X 0 0 X X 05 1 rH TM O ' TM 0 2 TM t o 0 • • tM 0 T O # rH # CO X 0 0 X X Tri X X X X 0 X 0 0 0 0 X A T M 1 — i rH X m 0 TM 0 X TM O ' rH d • 0 d rH 0 0 0 3 b û •H 0 t M 0 X X 0 0 43 0 0 X 0 0 0 X X 0 0 43 > 5 03 t-i A TM 5> 0 rH 0 rH TM t o I —l 1 —i 0 3 A 0 0 A d bO d A d •H 0 4 3 k d 03 d X CMrH X 0 0 0 0 0 X d X X 0 0 0 0 X X 0 0 X I —1 rH rH t M O ' *H 03 0 rH 0 3 TM rH 0 2 rH rH rH d ! — 1 ♦H k t o P rH X 0 0 0 X X 0 P X X 0 X 0 X X X 0 0 3 X 1 — 1 (— i t M X 0 3 0 3 P X rH 0 2 rH rH rH 4 3 T O 0 p 0 P P P P P P P P P P ' P P 0 P P P A A < 4 A < 4 A < 4 A < 4 A < 4 A < 4 A < 4 A < 4 A 43 EH ü M : k 0 A 0 M M A X P k S d X m 0 5 0 02 X t— 1 t M k 0 2 0 rH A 02 02 0 2 X X X k E - t P 133 t o th e p a th o lo g y d is c u s s io n i n a l a t e r s e c tio n * I n s p e c tio n o f th e l a r g e T able /.XQCl.I r e v e a l s n o ta b le te n d e n c ie s . The s u b je c ts w ith m arked b i l a t e r a l l o s s e s by a i r c o n d u c tio n seem t o have s i m i l a r l y m arked bone c o n d u c tio n im p a irm en ts. A th e to id s Nos. 5 , 6 , 11, 12 , 1 3 , 1 8 , 19, 29, and 35 show t h i s r e l a t i o n s h i p betw een a i r and bone c o n d u c tio n d a t a . The u n i l a t e r a l l o s s e s r e v e a l no s y s te m a tic te n d e n c ie s . S p a s tic s No. 9 and No. 36 and A th e to id s No. 1 and No. 20 show l i t t l e o r no bone c o n d u c tio n im pairm ent a lth o u g h th e AC m easure d e n o te s d e f i n i t e im pairm ent* A th e to id No. 8 and S p a s tic No. 31 on th e o th e r h an d , show l o s s e s i n b o th a i r and bone c o n d u c tio n v a lu e s . These r e l a t i o n s h i p s become s i g n i f i c a n t f o r p a th o lo g y d e s c r i p t i o n alo n g w ith o th e r p e r t i n e n t c r i t e r i a * Agreement i s high^^^» that a l o s s f o r a i r c o n d u cted s t i m u l i unaccom panied by any d e v i a t i o n from th e A. G. Pohlman and P. W. K ranz, " I s a D i f f e r e n t i a l D ia g n o s is Between M iddle and I n t e r n a l E a r.D e a fn e s s P o s s ib le ? " The L ary n g o sco p e. 3 6 :2 8 9 -2 9 8 , A p r i l, 1926. ^^%)ean M. L i e r l e and S c o tt H. R eg er, " C o r r e la tio n s Between Bone and A ir C onduction A c u ity M easurem ents Over Wide F requency Ranges in D i f f e r e n t Types o f H ea rin g Im p a ir m e n ts ," The L a ry n g o sco p e> 5 6 :1 8 7 -2 2 4 , May, 1946* ^^^George E. Shambaugh, "The I n t e r p r e t a t i o n o f Bone C onduction and O ther T e s ts o f H e a rin g ," A nnals o f O tology. R h in o lo g y . and L ary n g o lo g y . 4 5 :7 7 9 -9 7 , S eptem ber, 1936. 134 norm f o r bon© cond u cted sound is u s u a lly th e m a n if e s ta tio n o f a c a u s a tiv e p a th o lo g y in th e m iddle o r e x t e r n a l e a r . Where th e l o s s f o r bone co n d u cted sound i s c lo s e to o r th e same a s f o r a i r co n d u c tio n s t i m u l i , th e p a th o lo g y i s u s u a l l y s u s p e c te d a s b ein g somewhere i n th e c o c h le a - e ig h th n e rv e - c o r te x p o r t i o n of th e sy stem . Where some l o s s i s o b ta in e d f o r bone c o n d u c tio n , b u t th e m ag n itu d e o f th e lo s s i s n o t l a r g e e n o u ^ to ap p ro x im a te th e a i r co n d u c tio n f i n d i n g s , th e d a ta a r e c o n s id e re d to r e f l e c t p a t h o l o g i c a l p r o c e s s e s i n b o th th e co n d u c tin g and p e r c e p tiv e p o r tio n s o f th e a u d i to r y sy stem . Judgment o f p a th o lo g y l o c a t i o n on th e b a s i s o f a u d i t o r y t e s t d a ta a lo n e , how ever, i s r a r e l y done. The p r a c t i c e of m ost o t o l o g i s t s i s to su rv e y th e a u d ito r y t e s t d a ta in c o n ju n c tio n w ith t h e i r m e d ica l e x a m in a tio n f in d in g s r e g a r d in g t h e e a r , n o se , and t h r o a t , and th e m e d ic a l and h e a rin g h i s t o r y of th e c l i e n t and h i s fa m ily . The im p o rtan ce o f an o to r h in o la r y n g o lo g ic a l e x a m in a tio n in th e i n v e s t i g a t i o n i s s u b s t a n t i a t e d on th e s e grounds a s w e ll as f o r th e d e s c r i p t i v e d a t a i t a ffo rd s * Summary. These d a ta seem to i n d i c a t e t h a t a t h e t o i d s have a l a r g e r mean and m edian h e a r in g im pairm ent f o r p u re to n e s t i m u l i when compared t o n o rm al r e f e r e n c e d a ta and when com pared t o o th e r p a l s y d ia g n o s is g ro u p s. T h is 135 r e l a t i o n s h i p i s c o n s i s t e n t a t a l l f r e q u e n c ie s and f o r b o th e a rs and th e d i f f e r e n c e s seem to in c r e a s e w ith fre q u e n c y . When th e d i f f e r e n c e betw een each g r o u p 's p erform ance and th e norm i s t e s t e d f o r s i g n i f i c a n c e , th e t r a t i o s o f th e a t h e to i d s a t a l l f r e q u e n c ie s f o r b o th e a r s a re s i g n i f i c a n t w e ll beyond th e one p e r c e n t le v e l# The o th e r g ro u p s d e m o n stra te n o t one s i g n i f i c a n t o r n e a r s i g n i f i c a n t t r a t i o w ith d i f f e r e n c e s betw een th e a t h e t o i d s and th e norm c o u ld n o t h av e o c c u rre d by ch an ce, w hereas th e o th e r groups a re d e m o n s tra tin g o n ly chance d e v i a t i o n s . When th e mean t h r e s h o l d d a ta w ere compared t o th e a v e ra g e t h r e s h o l d s f o r a c o n t r o l group of f o r t y in d iv i d u a ls o f s i m i l a r a g e s , m ost o f th e d i f f e r e n c e s betw een c l i n i c a l and r e f e r e n c e d a t a were in c r e a s e d due t o th e n e g a tiv e v a lu e (b e t t e r - t h a n - a v e r a g e ) mean th r e s h o ld s o f th e r e f e r e n c e group, The d i f f e r e n c e s betw een a t h e t o i d s and c o n tr o l group were th e l a r g e s t , w ith c r i t i c a l r a t i o s s i g n i f i c a n t w e ll beyond th e one p e r c e n t l e v e l . E ig h t o f th e te n d if f e r e n c e s betw een s p a s t i c and c o n t r o l group mean th r e s h o ld s rem ain ed chance v a r i a t i o n s . The d if f e r e n c e s a t th e o th e r two f r e q u e n c i e s , - 256 c . p . s . , l e f t e a r , and 4096 c . p . s . , r i g h t e a r , - were now s i g n i f i c a n t t o f i v e p e r c e n t demands. D if fe r e n c e s betw een r i g i d i t i e s and c o n tr o ls were s t i l l sm a ll and i n s i g n i f i c a n t . Seven o f th e te n d if f e r e n c e s 136 betw een th e combined group and c o n tr o l th r e s h o ld s c o n tin u e d to be chance v a r i a t i o n s ; th e d i f f e r e n c e s f o r th r e e of th e f r e q u e n c ie s were l a r g e enough to y i e l d f i v e p e r c e n t s i g n i f i cance and one o f th e l a t t e r was s i g n i f i c a n t a t th e one p e r c e n t l e v e l . When th e th r e e m id d le f r e q u e n c i e s , 512, 1024, and 2048, a r e a v e ra g e d , o n e - t h i r d of th e a t h e t o i d s show av erag e l o s s e s o f more th a n 10 db com pared to o n ly fo u r p e r c e n t o f th e s p a s t i c 3» A ll of t h e g ro u p s d e m o n stra te some d e v i a ti o n from th e c a l i b r a t e d z e ro f o r spondee word s ti m u li w hich a r e l a r g e r th a n th e p u re to n e m easu res f o r c o rre sp o n d in g g ro u p s , w ith some d i f f e r e n c e s ap p ro a c h in g s ig n if ic a n c e # But th e a t h e t o i d m easu res p ro v e t o be o f c o n s id e ra b ly l a r g e r m agnitude and a s i g n i f i c a n c e w e ll beyond th e one p e r c e n t l e v e l i s re a c h e d when th e mean o f a t h e to i d spondee m easu res i s com pared to th e s p a s t i e s . The d i f f e r e n c e s betw een th e means o f th e two e a r s f o r e a c h fre q u e n c y were l e s s th a n 1 db ex c ep t a t 256 c y c le s where 1 .5 9 db s e p a r a te d th e r i g h t e a r from th e l e f t . H igh, p o s i t i v e c o r r e l a t i o n s from .7 9 to *87 f o r th e p u re t o n e s , and .9 0 f o r spondee w ords, w ere o b ta in e d betw een th e m easures o f th e two e a r s . B i s e r i a l c o e f f i c i e n t s o f c o r r e l a t i o n betw een te n s io n 137 d e s c r i p t i o n and h e a r in g l o s s i n th e a t h e t o i d sample i n d i c a te d no r e l a t i o n s h i p betw een th e two f a c t o r s . The a t h e t o i d s do n o t seem to d e v i a t e s i g n i f i c a n t l y from th e s p a s t i c s w ith r e g a r d t o number o f u n i l a t e r a l l o s s e s , b u t th e a t h e t o i d s show more th a n fo u r tim e s a s many b i l a t e r a l im p a irm e n ts. Marked b i l a t e r a l a i r co n d u c tio n l o s s e s seem t o show s i m i l a r l y m arked bone c o n d u c tio n im p a irm e n ts, w h ile th e u n i l a t e r a l l o s s e s r e v e a l no s y s te m a tic te n d e n c i e s . I I I . TEST-RETEST RELIABILITY The r e l i a b i l i t y o f th e m ethod o f p u re to n e m easure ment was s tu d ie d by a com parison o f th e r e s u l t s o f th e t e s t w ith th e f in d in g s of th e second t e s t . A lth o u g h th e r e l i a b i l i t y o f a u d io m e tric m easurem ent h a s been p r e v io u s ly d e s c r ib ed by W ittin g and Hughson,^^*^ i t was d e c id e d to examine t e s t r e l i a b i l i t y h e re b ec au se of th e p e c u l i a r n a t u r e o f th e c l i n i c a l p o p u la tio n s tu d i e d . F o u rte e n o f th e s u b je c ts were b ro u g h t i n f o r a second round o f p u re to n e th r e s h o l d s , tw e n ty -e ig h t to n i n e t y - t h r e e days a f t e r th e f i r s t t e s t . These s u b je c ts in c lu d e d f o u r s p a s t i c a , n in e a t h e t o i d s , and one r i g i d i t y . E . G. W ittin g and W a lte r Hughson, " I n h e r e n t Acc u ra c y o f a S e r ie s of R e p eated C l i n i c a l A udiogram s," L ary n g o sco p e. 5 0 :2 5 9 -2 6 9 , M arch, 1940. 138 A combinat i o n o f f a c t o r s r e s t r i c t e d any l a r g e r f r e quency o f r e t e s t s : d is ta n c e of th e s u b je c t from th e h o sp ita l, u n a v a i l a b i l i t y o f th e t e s t i n g l a b o r a t o r y d u rin g th e g r e a t e r p a r t o f th e week, and r e s t r i c t e d o v e r a l l tim e a llo w e d f o r r e s e a r c h b y th e h o s p i t a l . S ix o f th e group were h o u sed a t a n e a rb y C o n v a lescen t H o s p ita l and were more r e a d i l y a v a i l a b l e . A lthough f o r t u t i o u s f a c t o r s o p e ra te d i n t h e i r s e l e c t i o n , th e f o u r te e n r e t e s t r e l i a b i l i t y s u b je c ts were n o t u n r e p r e s e n ta ti v e of th e t o t a l sample* T h e ir ages a t th e f i r s t t e s t ra n g e d from s ix y e a r s , s i x months to s i x t e e n y e a r s and e le v e n m onths, w ith a mean age o f n in e y e a r s , n in e m onths. T hree o f th e group w ere below seven y e a rs o f a g e . E leven o f th e group were q u a d r ip le g ic s , two were h é m ip lé g ie s , and one s p a s t i c showed th r e e e x t r e m i t i e s in v o lv e d . F iv e of th e group were m o d e ra te ly h a n d icap p e d w ith f o u r m ild and f o u r s e v e r e ly h an d icap p e d in d iv id u a ls # One s p a s t i c q u a d r ip le g ic had s e v e r e ly in v o lv e d le g s w ith th e arms m ild ly h a n d ic a p p e d . T ab le XXXHin th e A ppendix^^^ d e t a i l s th e ag e , e x t e n t , and s e v e r i t y f o r each o f th e f o u r te e n s u b je c ts of th e r e l i a b i l i t y g ro u p . 235 See A ppendix, p* 243. 139 Seven had norm al h e a r in g ; sev en showed m a rg in a l l o s s e s o f f i f t e e n d e c ib e ls a t two f r e q u e n c ie s o r tw en ty d e c ib e ls a t one f re q u e n c y . Of th e seven w ith m a rg in a l l o s s e s , f i v e w ere b i l a t e r a l l y im p a ire d , two had u n i l a t e r a l l o s s e s . The means of th e d i f f e r e n c e s betw een th e f i r s t and second th r e s h o l d s were o b ta in e d f o r each fre q u e n c y and each ear* The means a r e shown along w ith th e s ta n d a rd d e v i a ti o n e x p re s s io n o f th e v a r i a b i l i t y o f t h e i r d i s t r i b u t i o n in T ab le ÆKIm , • The m e ^ d i f f e r e n c e s w ere o b ta in e d w ith o u t r e g a rd to s ig n so t h a t r e a l d if f e r e n c e s would n o t be sub merged b y c a n c e l l a t i o n of p o s i t i v e and n e g a tiv e . The a v e ra g e d i f f e r e n c e betw een T e st #1 and T e s t #2 ra n g e d from 1*79 d e c ib e ls a t 2048 c . p . s . , r i g h t e a r , to 5*36 d e c ib e ls a t 1024 c . p . s . , r i g h t e a r . The mean d i f f e r en ces a t 256 and 512 c y c le s seemed l e s s s t a b l e i f th e s l i g h t l y l a r g e r s ig n a s a r e i n d i c a t i v e . The l a r g e s t d i f f e r e n c e a t any th r e s h o l d p o in t in any s u b je c t was one of te n d e c i b e l s . C o n sid e rin g th e number of changes from t e s t t o t e s t , 2048 c y c le s , r i g h t e a r , show ed o n ly fo u r d i f f e r e n c e s , te n s u b j e c t s r e p e a tin g th e t h r e s h o ld o f th e f i r s t t e s t when th e second t h r e s h o l d was ob ta in e d * T h ir te e n changes in f o u r te e n r e t e s t s were found a t 4096 c y c le s f o r b o th e a r s . I n r e s p e c t to c h a n g e a b ilit y , th e s e f re q u e n c ie s m ig h t be c o n s id e re d m ost s t a b l e and l e a s t s t a b l e , r e s p e c tiv e ly * 140 M I gas B § E 4 E h I g C O i ^co r o o • 03 a S O M '— 03 + 5 }> > 0 • i n O f H g . Q > 03 to CO lO 03 C O O § ^co • ro O • 03 A â o 03 -P p^G ^ O H W (3 •H < D PI P < D 03 ^ M k lO C O lO 03 O O • to I — I 03 # to lO to t o C O * 03 C O C O o o « lO o> ?> t o C O • to (H 03 « to o o c - C O 03 LO O ' • to t o 03 to to O I — j 03 'cM ; —I to « 03 LO o to < J> 03 03 05 lO to G O £> L O to to 05 CD 03 • • 03 ^ rH r — i 0 5 to 03 • • I — I rH ^ to H C O • • 03 03 rH iH iH t o 0 3 t o O) rH 03 • • 0 5 H 0 5 0 3 * # to 03 03 to 05 05 03 # • C O 05 rH rH 05 0 - 0- lO 0 0 (D to 1 — 1 iH O î> lO o • # 03 I — I I — I I — I C O N j» 0 5 to * ' • C O 0 5 to 03 0 0 iH rH to O to C O • # 03 05 0> LO # * CO 05 CO iH r-i to 0 5 rH C O . . • # 03 iH 03 ^ ^ t o rH rH to I — I CO C O ■ ♦ * 03 03 03 C> $ * to to t o l O ^ o • • rH iH iH O C- t o • 0 m to < D © 1 — 1 03 o o f i Ü © © 43 4 3 u n m m © © © © t o A -4 E"( Eh O rH «M <H •H II *H fH # » * * 4-5 # q A • « fl a * * H cd A h a A © cd A h pcj • © An * (H © © *H ^ O CO o Â Eh 141 The mean t h r e s h o l d s f o r T e s t #1 and T e st #2 a re shown In T able XXT* The d i f f e r e n c e s betw een th e two t e s t means a r e g iv e n and may be com pared to th e means o f th e a b s o lu te d i f f e r e n c e s , o b ta in e d w ith o u t r e g a r d to s ig n , to o b se rv e th e e f f e c t of th e l a t t e r s t a t i s t i c a l p ro c e d u re . As a m easure of t e s t - r e t e s t r e l i a b i l i t y , th e s i g n i f i c a n c e o f th e d i f f e r e n c e betw een th e means a t each fre q u e n c y was s tu d ie d w ith th e t t e s t . S in ce th e m easu res t h a t made Up th e r e s p e c t i v e means would be e x p e c te d t o be c o r r e l a t e d , th e t r a t i o fo rm u la f o r s i g n i f i c a n c e o f d i f f e r e n c e in means 236 o f r e l a t e d m easu res was u se d . T h e .r a t i o s a r e shown in T able XXTK. D e s p ite means o f d if f e r e n c e s o f f i v e d e c ib e ls a t fo u r fre q u e n c y p o i n t s , t h e t r a t i o s o f th e d if f e r e n c e s b e tw een th e means o f T e s t #1 and T e s t #2 g e n e r a lly d id n o t p ro v e s i g n i f i c a n t . Most o f th e v a lu e s f a i l e d t o m eet th e t r a t i o demands o f 2 .1 6 and 3 .0 1 a t f i v e p e r c e n t and one p e r c e n t l e v e l s of s i g n i f i c a n c e , r e s p e c t i v e l y , f o r t h i r te e n d e g re e s o f freedom . The d i f f e r e n c e s betw een th e means a t 1024 c y c le s r i g h t e a r , was s i g n i f i c a n t a t th e one 236i E. P. L in d q u is t, S W tis t^ io a l A n a ly s is in Educa- t i o n a l R e se a rc h (B o sto n : Hought'on M i f f l i n Company, 1 9 4 0 ). p p . 58-39% 142 p e r cent l e v e l . R easons f o r t h i s i s o l a t e d s i g n i f i c a n t d i f f e r e n c e a r e o b sc u re . The c o u n te r p a r t f o r th e l e f t e a r i s o n ly a chance d i f f e r e n c e d e s c r ib e d by a t r a t i o o f .5 9 . No p a r t i c u l a r p a t t e r n o f d i r e c t i o n o f d if f e r e n c e s i s o b se rv e d i n th e means where f i v e f r e q u e n c ie s show i n c r e a s e s and a s many show d e c re a s e s i n mean th r e s h o l d s . W ittin g and Hughson^^*^ have p r e v io u s ly o b se rv e d t h a t ex p e c t a t i o n would be somewhat h ig h e r f o r d e c re a s e i n t h r e s h o ld a c u i t y when norm al s u b je c ts were r e t e s t e d . In th e s c a t t e r diagram of F ig u re 8 , a l l o f th e v a lu e s of th e f i r s t t e s t f o r a l l f r e q u e n c ie s a r e p l o t t e d a g a in s t th e c o u n te r p a r t v a lu e s of th e r e t e s t . The h ig h , p o s i t i v e , r e c t i l i n e a r r e l a t i o n s h i p betw een th e two t e s t s i s g r a p h i c a l l y d e m o n stra te d . I t would seem from th e s e d a ta t h a t , a p a r t from th e one fre q u e n c y of 1024 c y c le s , r i g h t e a r , th e d if f e r e n c e s o b se rv e d betw een th e f i r s t t e s t and r e t e s t th r e s h o ld s a r e d i f f e r e n c e s t h a t co u ld e a s i l y have o c c u rre d by chance sam p lin g . When c o n s id e re d as m easures o f r e l i a b i l i t y , th e t v a lu e s and th e s c a t t e r diagram i n d i c a t e r e l i a b l e r a t h e r th a n u n r e l i a b l e m ethod. P 3 * 7 W ittin g and Hughs on, l o c . c i t . X: Thresholds in F i r s t Test -10 0 10 V 20 30 40 50 60 70 ^0 90 F )( 90 O 0 so 1 F / / O 16 1 F I I I I I 1 7 6 1 F 1 F 1 F 3 60 - 1 F 1 Iff B " 1 F 4 50 1 F I I I 1 7 1 rr 5 1 F 1 F Z 40 I F « 1 F — — - - 5 I I rs 2 30 ' o I I I F 1 F 1 F 5 20 I I 1 ? 1 F I I I F I I F g M f* F nil It 1 F - fO 1 fr I I (z I I R I f F 1 F 8 nil nil -H tr 4 M * R 4 M * 1 7 I I P 25 0 1 rr ■ m * - ■ III R III! II" 1 7 1 F /? I I K m + - w * + 4 M * 4 M * P 5 If F 30 40 ♦ 4 H - 1 p I rr 1 F S Fx 22 25 27 5 S Z / 2 6 o 4 y 3 / 4 2 o 7 0 4 3 a « I a 5 I « N FIGURE 8 SCATTER DIAGRAM OF THRESHOLD VALUES IN TWO^PURE TONE AIR CONDUCTION THRESHOLD TESTS 144 IV. OTORHINOIARYNGOLOaiCAL EXAMINATION FINDINGS P h y s ic a l and p a t h o lo g ic a l c o n d itio n s w hich may a l t e r a u d io m e tric o b s e rv a tio n s a r e numerous. Congenital an o m alies sh o u ld f i r s t be c o n s id e re d and th e f a c t t h a t th e b a s ic p a th o lo g y , c e r e b r a l p a l s y , m ight have had a c o n g e n ita l background in c r e a s e s th e i n t e r e s t in c o n s id e r in g t h i s ty p e o f c o n d i tio n . A t r e s i a or s te n o s is of th e e x t e r n a l e a r c a n a l c o u ld c e r t a i n l y a l t e r h e a r in g a c u i t y as c o u ld con g e n i t a l m a lfo rm a tio n o f th e tym panic membrane, m id d le e a r , o r c o c h le a . M aishaped n a so p h a ry n g e s, r e s u l t i n g from con g e n i t a l c o n d i tio n s , co u ld a ls o a l t e r th e f in d in g s by up s e t t i n g th e dynam ics o f th e m iddle e a r and e u s ta c h ia n tu b e . O ther p a t h o lo g ic a l c o n d itio n s c a p a b le o f a l t e r i n g th e a u d i to r y a c u ity in c lu d e r e p e a te d m id d le e a r i n f e c t i o n s , eu sta c h ia n - tu b e p a th o lo g y , o v erg ro w th o f lym phoid t i s s u e in th e n aso p h ary n x from c h ro n ic i n f e c t i o n o r a l l e r g y , o to s c l e r o s i s , and d is e a s e s o r anom alied o f th e e ig h th c r a n i a l n erv e and a u d i t o r y c o r te x . E a r, n o se, and t h r o a t ex a m in a tio n s o f each s u b je c t w ere done b e f o r e th e a u d io m e tric s tu d y . When im pacted ^ ^ ^ P ra n c is L. L e d e re r, D is e a s e s o f th e E a r. Nose. and T h ro a t ( f o u r t h e d i t i o n ; P h ila d e lp h ia : P. A. D avis Company, 1 9 4 5 ), p p . 3 -2 9 2 . 239 Edmund P. F o w ler, e d i t o r . M edicine o f th e E ar (New York: Thomas N elson and Sons, 1 9 3 ^ ), p p . 9 3 ^ 6 8 . 497-558. 145 cerumen was o b serv ed In th e e x t e r n a l m eatu s, i t s p re se n c e was n o te d and th e n removed from th e e a r c a n a l. The a d v a n t age of a t e s t o f t h r e s h o l d a c u ity b e fo r e and a f t e r rem oval o f cerumen was c o n s id e re d o f f s e t by th e a d d i t i o n a l f a t i g u e p o s s i b i l i t y . The e x t e r n a l e a r was examined f o r p re s e n c e o f eczema t o h e lp e s t a b l i s h p re s e n c e o r ab sence of a l l e r g i c s t a t e s . Cerumen i n th e e a r c a n a l was n o te d a s to c o lo r and c o n s is te n c y . S t r u c t u r a l m a lfo rm a tio n s were r e c o r d e d . The tym panic membrane was exam ined by means o f an e l e c t r i c o to sc o p e t o d eterm in e p re s e n c e o f p e r f o r a t i o n or s c a r t i s s u e . M o b ility o f th e e a r drpm was t e s t e d w ith th e S e ig le oto sco p e and th e f u l l n e s s of th e drum was n o te d . The membrane*s c o lo r and c o n s is te n c y w ere o b se rv e d . The Toynbee T e st w hich c o n s i s t s o f th e p a t i e n t sw allow ing w h ile th e exam iner o b se rv e s th e membrane, was co n d u c ted . Movement o f th e drum a t t h i s tim e i n d i c a t e s th e p o te n c y o f th e e u s ta c h ia n t u b e . The e x t e r n a l nose was o b se rv e d aé to ty p e , d e fo rm ity , s i z e o f n o s t r i l , p re s e n c e o f c r u s t in th e v e s t i b u l e , and 240 Lawrence R. B o ie s, e t a l . . F undam entals o f Oto- lapynaoloK y ( P h il a d e l p h ia : W . B. S aunders Company. 1 9 4 9 ). p p . 2 6 ,2 8 . ' ^^^dm und P. F o w ler, J r . , e d i t o r . M edicine o f th e E ar (New Y ork: Thomas N elson and Sons, 1 9 3 9 ), p . 146 s t r a i g h t n e s s o f th e septum . The l a t e r a l n a s a l w a ll was exam ined f o r c o lo r o f m ucosa, c o n s is te n c y o f th e m ucosa, anatom y o f th e t u r b i n a t e s and p re s e n c e of absence o f p o ly p i. The n a tu r e o f th e n a s a l s e c r e tio n s and th e s iz e of th e a i r m y w ere n o te d . O ral h y g ie n e , d e n t i t i o n , th e h e ig h t and c o lo r o f th e p a l a t e , b u c c a l m ucosa, and c o n d itio n o f th e s a l i v a r y d u c ts w ere o b se rv e d . P re se n c e o r ab sen ce o f th e t o n s i l s was o f i n t e r e s t and where th e t o n s i l s were p r e s e n t, th e c o n d itio n of th e t o n s i l s was n o te d . Where t o n s i l s had been rem oved, th e c o n d itio n of th e t o n s i l l a r f o s s a e and p i l l a r s was n o te d . The p re s e n c e o r ab sen ce o f lym phoid t i s s u e i n th e ph ary n x was re c o r d e d . The n aso p h ary n x was exam ined f o r s iz e and sh a p e, th e p re s e n c e o f a d e n o id t i s s u e and th e amount and l o c a t i o n o f any o th e r n aso p h a ry n g e a l lym phoid t i s s u e . F ig u re 8 shows th e check s h e e t u se d by th e o t o l o g i s t and h is a s s i s t a n t i n re c o rd in g e x a m in a tio n f in d i n g s . I n th e judgment of th e two o to la r y n g o l o g is ts ask ed to e v a lu a te th e ex a m in a tio n data,^*^^ th e t o t a l sam pling o f c e r e b r a l p a l s y p a t i e n t s p r e s e n te d p h y s ic a l c o n d itio n s w ith 2 ^ % r. W arren Line and D r. V ic to r G o o d h ill o f th e h o s p i t a l D epartm ent o f O to la ry n g o lo g y e v a lu a te d t h i s d a t a . 147 no o u ts ta n d in g v a r i a t i o n s from th o s e n o te d in th e g e n e ra l p o p u la tio n . The same o b s e rv a tio n s were made by th e c o n s u lt in g o t o l o g i s t in W o l f e s t u d y i n r e f e r e n c e t o t h e f i f t y Iowa s u b j e c t s . The m e d ic a l c o n s u lta n t s o f t h i s i n v e s t i g a t i o n judged th e d i f f e r e n c e s betw een r i g h t and l e f t e a r s to be c l i n i c a l l y i n s i g n i f i c a n t and recommended t h a t r i g h t and l e f t d a t a b e grouped t o g e t h e r . The p h y s ic a l f in d in g s f o r each group were e s t a b l i s h e d s e p a r a te ly and th e f in d in g s p re p a r e d i n p e rc e n ta g e s f o r t a b u l a r p r e s e n t a t i o n . The E a r. T able XXCEpresents th e p h y s ic a l f in d in g s o f th e e x a m in a tio n o f th e e a r . No c o n g e n ita l m a lfo rm a tio n s of th e e x t e r n a l e a r w ere found i n any o f tïrie su b -g ro u p s. Hard cerumen was found i n 1 3 .8 p e r c e n t o f th e e n t i r e sam ple. The c o lo r of th e cerumen was y e llo w in o n e - h a lf t o t h r e e - f o u r t h s o f th e s u b je c ts in th e g ro u p s , e x c e p t f o r th e a l l - y e l l o w f in d in g s in th e a t a x ic and trem o r g ro u p s. Brown cerumen was o b se rv e d in h a l f of th e r i g i d i t i e s , and in a p p ro x im a te ly o n e - f o u rth o f th e s p a s t i c and th e a t h e to i d g ro u p s ♦ None o f th e 101 s u b je c ts h ad drum p e r f o r a t i o n s . W i llia m W olfe, "A C l i n i c a l E v a lu a tio n o f F i f t y C e re b ra l P a ls ie d I n d i v i d u a l s ," (u n p u b lish e d D o cto r» s d i s s e r t a t i o n , S ta te U n iv e r s ity o f Iowa, Iowa C ity , 1 9 4 7 ), p . 47. 148 TABLE XXri FINDINGS OF MEDICAL EXAMINATION OP THE EAR T o ta l Pop. A th. S p a s t. R ig . A tax. Trem. Comb. M alfo rm atio n 0 0 .0 0 0 .0 0 0 .0 0 0 .0 0 0 .0 0 0 .0 0 0 .0 Cerumen Hard S o ft C olor Y ellow Brown W hite 1 3 .8 8 6 .3 7 1 .3 2 6 .3 2 .5 2 0 .5 7 9 .5 6 6 .7 2 8 .2 5 .1 8 .0 9 2 .0 ^ 7 6 .0 2 4 .0 0 .0 1 0 .7 8 3 .3 5 0 .0 5 0 .0 0 .0 0 .0 1 00.0 1 00 .0 0 .0 0 .0 0 .0 100 .0 1 00 .0 0 .0 0 .0 0 .0 1 00 .0 8 5 .7 1 4 .3 0 .0 Tympanic Membrane I n t a c t P e r f o r a te d 1 00 .0 0 .0 1 00.0 0 .0 1 0 0 .0 0 .0 1 0 0 .0 0 .0 1 00.0 0 .0 1 00 .0 0 .0 1 0 0 .0 0 .0 R e tr a c te d Normal F u l l 4 9 .5 5 0 .5 0 .0 5 0 .0 5 0 .0 0 .0 6 0 .0 4 0 .0 0 .0 5 8 .3 4 1 .7 0 .0 5 0 .0 5 0.0 0 .0 1 0 0 .0 0 .0 0 .0 1 4 .3 8 5 .7 0 .0 C o lo r Gray O p a le sc e n t Normal Red P ink D u ll W hite 1 7 .7 5 3 .1 2 2 .9 0 .0 6 .3 2 7 .5 4 7 .5 2 0 .0 0 .0 5 .0 8 .0 56.0 24.0 0 .0 1 2 .0 0 .0 6 6 .7 3 3 .3 0 .0 0 .0 0 .0 100.0 0 .0 0 .0 0 .0 0 .0 0 .0 0 .0 0 .0 1 00 .0 2 8 .6 4 2 .9 2 8 .6 0 .0 0 .0 C o n s iste n c y T ra n s p a re n t Opaque T ra n s lu c e n t A tro p h ic 1 6 .5 1 3 .6 68 .9 1 .0 8 .3 1 4 .6 7 7 .1 0 .0 2 0 .0 ■ 2 0 .0 5 6 .0 4 .0 1 6 .7 1 6 .0 8 3 .3 0 .0 0 .0 0 .0 1 00.0 0 .0 0 .0 0 .0 1 00 .0 0 .0 4 2 .9 1 4 .3 4 2 .9 0 .0 Toynbee Drum Moves 5 3 .4 6 0 .0 7 2.0 3 3 .3 5 0 .0 0 .0 1 4 .3 S p e c ia l F in d in g s * A ll v a lu e s a r e p e rc e n ta g e s 149 A pproxim ately f i f t y p e r cent of th e t o t a l g ro u p , f i f t y p e r cen t o f th e a t h e t o i d s , and s i x t y p e r cen t of th e s p a s t i c s showed r e t r a c t e d e a r drums. The o to la r y n g o l o g is ts r e p o r te d t h a t a p p ro x im a te ly h a l f o f th e norm al i n d i v i d u a l s in th e g eo g rap h ic a re a from w hich t h i s smaple was drawn show r e t r a c t e d membranes. An o p a le s c e n t c o lo r of e a r drum was n o te d in th e l a r g e s t number of th e s u b j e c t s . The 2 7 .6 p e r c e n t o f a t h e to i d s w ith g ra y drums seems somewhat l a r g e . Only one- f i f t h to o n e - t h i r d o f th e s u b je c ts showed "norm al" c o lo r . The drum proved t r a n s l u c e n t i n mos t . o f t h e s u b je c ts , w ith a sm a ll number t r a n s p a r e n t . More th a n t w o - f i f t h s o f th e com b in e d s u b je c ts had t r a n s p a r e n t drums w hich i s somewhat u n u su a l b u t n o t n e c e s s a r i l y a p a t h o lo g ic a l s ig n . Toynbee movement was n o te d in s i x t y p e r c e n t o f th e a t h e t o i d s and a lm o st t h r e e - f o u r t h s of th e s p a s t i c s , b u t o n ly o n e - th ir d o f th e r i g i d i t y and only f o u r te e n p e r c e n t of th e combined g ro u p s. Toynbee movement i s n o t alw ays d i s c e r n i b l e and f i f t y p e r c e n t w ith o u t t h i s c l i n i c a l sig n i s not c o n s id e re d u n u s u a l. The Nose. The f in d in g s o f th e ex a m in a tio n o f th e nose a r e d e t a i l e d in T able XXÎEE JJo o u ts ta n d in g v a r i a t i o n s were found in th e e x t e r n a l n o se . Average shape and s iz e were n o te d in th e g r e a t m a jo r ity o f th e s u b je c ts exam ined. 150 M H H I "d O O O O tO î> O 'M i o C D Hi to to © • • • • * * * • • • * • • O O O O 'c M lO O rH o C O rH Hi Hi o o rH C D C- C M IN rH rH n rH rH s o o ■ - o o o O O O O O O O O O O p4 # • • • # q O O O ’ O O O O O O O o O O . ' . g o q O O o © H rH rH 1 — 1 rH ÎH EH a S O O O O O O O o o o O O O •H • ♦ :• « • # # * M O O O O O O O o o o O O O cd o o I p Ip o o p rH rH rH rH P •H r - t o O O o tp £> O cO O O o 'O • • • • • • • • # • ♦H C O co O O O to C D C D O C O O o o bO rH CO lO O Ip to rH C D C O o * r - | ; ' r-i rH (Z: Ü •H P O O O O o o o O O O O o o W • • • • • • « • • • C d C D U O O Ci W C D C M o C D C O 00 Hi P i iH CQ C O O C O IP rH IP Hi G O C O rH nd •H m G O O iP tp 05 C O 05 O O CQ rH O C M • • • é * * • P • * ■ ♦ • • CD C O O rH G O C D JN iH © C- O ÎO O o LO rH îO ÎN iH rP rH £> O rH P rH < P O iH *H O» C D O 'C H C N J C M IN rH CD C M £ n ai P # # # # • • • • • • • • ♦ P © O î > rH O ip sii O i p Hi O îO rH C O O rH 1 — Il— 1 C Q O C M C D rH IP Hi C O 1 — 1 EH P rH P i O P M c ! © o C T •H fl O © p o X p - i © © to © p P © p *H © P > rH P > O o O P rH g to * * H * H rH g d © © *H tO*H o *H © cd o as P P •H rH p © p P A P R »H P rH P *iH C © P P P C d p rH 'H to •H W Ü © P © © m © > U © T O P © o to P © T î p P P © to p ^ 4J « H P © © P ,*H © > < P © © Ë © © © P «H © A P d A © A -P © îs; O m ^ A © O A îs q © C D < C o E h A ja; > C D A W © b O a J ■ P cî © o © A © S CD § H a i > — i — I g 151 C O c b * d * 2 5 © M § i •H M p G O O O 1 — ! H E H ■ M M M X % m H A m A < EH C O g nd © G * H A S o o p o s © P E H © •H % © P <4 Î N , P ♦ H 'd • r H b û •H M ü • H P W © P C O Tj •H O P © A p < 4 G O rH *H © P P © O 1 — i E H d p o p EN CO o o o » • • • • l O H * O O O C D r H O O O O O • . • « » O O O O O o o o o o o • • « • • o o o o o LO t o o o o o o • * • • * o to H i o o 00 rH 0 5 A CO O » • # # • «O rH EN A O EN rH r H 0 > lO L O o ♦ . • • • • 05 H i H i rH O EN rH © O © O m rH d O i — 1 a Î2Î © « M rH o © rH G© p © P P o G rH ©© rH •H © P P O P P G © O M H CO IN O O O O H i LO o o o o H 0 0 O O O O O O O o o o o o o s o o o o o o o o o o o o t o t o o o o o o # # # # # o o o o o s o o o o o $ # # # # o o o o o o rH o o o o o • • • • • o o o o o s o o o o o o . • • • • • • CD H i H i CO o o rH CO rH o o o o o o « • ' • # # * CO CO o CM o o r H IN I — I CD EN o CO o o © • • • • • • © CO CM CO O O O o rH EN I —I O 0 A-t O O O O O O • * • • • C O o C M o o C D I— 1 t o O t o O O 00 o rH o o 00 rH « 0.0 © • • © 05 O P 00 H i S ÎN . O © G H © © A P b O © to © M •H © •H GP o Tf P d © •H © •H ©© 1 — 1 G A >A P4 > C Q o EH <4 EH m Id 'd O "H P |N> O © rH P i SA K © A rH »d © O O © G ir | A P d . O EH •H © A > A P •H o o o P © p O Î N b O A P o d S © p p "H p O P O © P p p © P P W O T © > P ÎN, ÎN>A d <4 <4 ÎG p o <4 \ rH 05 O O O • • • • « E N C M O O O A H i O O O O O ♦ • • ♦ • o o o o o o rH O O O O O ♦ • • • ♦ o o o o o o I — I A E N O O O O A t - O O O A O O O O A O O E N * # . # # • • • • • • # # # # * • • • • • A «O O O O O A C O O O O A O A O O O A O O A 00 rH C O rH 00 rH A A o o o o o « « • • # o CO o o C M CD CO o o o o o # # # # * 00 C M Hi o to Hi C O rH O A EN A o A # • . • • ♦ O CO C M iH O EN H i CM CM m P O •H P P © rH P d G Î N » * © © P p *H rH Pi © © O d O © p P O P O a o © d d d o © ^ ^ p, ^ ^ m p d 152 p o E H A © G •H A a o p p o a © p E-î © •H M © p < 4 ÎN P •fH A •H bO *fH A - O ' •H P (0 © P i CO A •rH O O o P • $ © o o A o p rH "© G o p p O O © # * rH O o d o P i rH O ( P P G P •H © d Pi © © ÎN © © rH P A O f P < 4 f P A E n O A E n O A Hi O A H* O • • • • • • • ♦ • . * • H» A O Hi A O 00 rH O 00 rH O rH CO rH CO CM IN C M EN O O O O O O O O O O O O • « • • ♦ . • • ♦ • • « O O O O O O O O O O O O o O o O . o 1 —1 1 —1 rH H 1 —1 o o o O O O O O O o o o • « « « • • • • • o o o o o o O O O O O O A A A A A A A A A E N O A E N O E N A O E N A O • • • • • • • • • # # # A A O A A O A A O A A O A A A A H 00 rH C O Hi 00 05 Hi 00 05 rH rH 05 rH 1 — 1 05 • • • • # # r# # ♦ • • • A O A A O A A A A A A A rH 00 H C D C M E N . C M E N rH 05 O C M E N O rH 05 O C M E N O • • « . • « • • • A A O 05 O O A A O 05 O O C M E n rH 00 C M E N rH C O A 05 A rH Hi A A O A rH A A * # # # # # ■ * • # # # # O E N rH 05 05 1 — 1 A A rH C M A rH C M E N rH E N C M E N C M E N ( D P m { n p i â G P »H <4 •H IG <4 P o •H H P rH P O • iH © P rH bO © P P © © ^*-9 0 Ph ■ H © A © © rH »H bO © A P Ë © © P C O ^ l A « P © (-9 P 0 •H p © 1 H P H *H © A © P O •H © P bO © P P © w S A O P P rH d rH *H © A g © C O S © bû P A to © S’ P G © o S A © P © © © d rH © > rH H g 153 Those w ith v a r i a t i o n s showed e i t h e r wide o r narrow n o s e s , b u t none were found to have d e f o r m iti e s . The n o s t r i l s w ere medium in s i z e i n th e m a jo r i ty , w h ile d e v ia tio n s te n d e d i n th e d i r e c t i o n o f s m a lle r r a t h e r th a n l a r g e r n o s t r i l s . These f in d in g s were e :^ e c te d s in c e more narrow n o ses were found th a n w ide. C ru s ts w ere n o te d in th e v e s t i b u l e s f o r th e l a r g e p e rc e n ta g e o f s u b je c ts w ith on ly a few , 1 1 .5 p e r c e n t o f th e a t h e t o i d s , showing d is c h a rg e i n t o th e v e s t i b u l e . The septum was o b serv ed to be s t r a i g h t in alm o st a l l of th e s a u r i e s . In th o s e who d id show d e v i a t i o n s , a n t e r i o r d i s l o c a t i o n s w ere more common th a n p o s t e r i o r d e v ia tio n s # The l a t e r a l n a s a l w a ll f o r more th a n t h r e e - f o u r t h s t o f th e sam ple was th e u s u a l p in k c o lo r found in th e av e ra g e h e a lth y n o se . Almost f i f t e e n p e r c e n t showed a p a le c o lo r r a t h e r e v e n ly d i s t r i b u t e d th ro u g h th e fo u r l a r g e r su b -g ro u p s Average c o n s is te n c y o f th e mucosa was o b se rv e d in th e m a j o r i t y . The anatomy of th e t u r b i n a t e s was n o t found to b e u n u su a l w ith a sm a ll p e rc e n ta g e o f th e h y p e rtro p h y o b se rv e d . S e c re tio n s were n o te d t o be w a te ry o r mucoid i n alm o st a l l s u b je c ts w here s e c r e t i o n s were p r e s e n t . No p o ly p i w ere o b se rv e d and th e airw ay was found to be medium s iz e d i n th e v e ry l a r g e p e rc e n ta g e o f a l l g roups s tu d i e d . 154 The Mouth. The f in d in g s of th e e x a m in a tio n of th e m outh, th e p h ary n x , and th e naso p h ary n x a r e d e t a i l e d in T ab le XXIV* O ral h y g ie n e and d e n t i t i o n were a v e ra g e ; th e b u c c a l mucosa a v e ra g e i n c o lo r and a p p e a ra n c e ; and th e s a l i v a r y d u c ts were open i n a l l o r n e a rly a l l c a s e s s tu d ie d . The h e ig h t o f th e p a l a t e was judged to be h ig h e r th a n av e ra g e f o r 16*7 p e r c e n t o f th e t o t a l g ro u p , w ith th e r i g i d i t y , com bined, and a t h e t o i d groups showing m ost o f th e h ig h e r p a la te s * T o n s ils were p r e s e n t in o n e - h a lf to tw o - th ir d s of th e f o u r l a r g e r g ro u p s . Most o f th e t o n s i l s ap p e a re d p a l e , f ir m , w ith n o th in g i n th e c r y p t s , i n d i c a t i v e o f a r a t h e r h e a lth y anatom y. Edematous t o n s i l s w ere o b se rv e d in more th a n tw e n ty - s ix p e r c e n t o f th e s p a s t i c s and f o u r te e n p e r c e n t of th e a t h e to i d sam pling* True ta g s w ere p r e s e n t in a few of th o s e w ith o u t t o n s i l s , w ith some showing p a le fo ssa e * P harynx* The p h ary n x ap p e ared "norm al" i n th e m a jo r i t y , b u t a s u r p r i s i n g number e x h ib ite d lym phoid h y p e r p la s ia . N asopharynx* The s i z e o f th e naso p h ary n x was con s id e r e d av erag e i n m ost o f th e s u b je c ts o f each g ro u p , w ith a few c o n tr a c te d an ato m ies o b se rv e d . A p art from th e combined c a te g o ry , th e ad e n o id was p r e s e n t i n f i f t e e n p e r c e n t o r more of each group* The aden o id had been removed i n tw o - th ir d s o f th e combined JLOO > H s I m o g M P h § M E H g M '© © O O O EN o A EN A A O O O O o o o G * « • * $ # • • # 0 • • * * • • • •H o o o A O V A A A H* O O O o O O O A o CO rH A A CO rH O G H rH o o G o o o O O O O O O O O O O o o o o O • • • • « # # * * * * • « • • Ë o o o O O O O O O O O O O o o o o © o O O O o G rH rH rH H rH 54 © o o o O O O O O O O O O O O o o o •H • • • * • * • # * • • • « • • • • M O O O O O O O O O O O O O O o o o © o O o o o 4-5 rH H H H H <4 ÎN •H O O O O O O EN A o o o o O o o o o • » * # • * • • • • * # # *H o o o O O O A A o o o o g o o o o fcO O o A A o q •H rH rH rH 1 — t CG o •H O O O O O O O O o o o o o o o o o 4J . * # • • • * « * 0 0 0 • ♦ • O T o o o A O H4 A o o o o o o o o o © o 05 05 o o A rH rH rH CD H •H O o o o A O EN 05 H A EN o O O o o o o 4-5 • • • • « « • « • * « • * © o o o CM O EN A A CM EN O O O o o o o \ G o 05 CM 05 o •P rH rH G O — 4 H o © 44> o o o O O O A EN A A O O o o o o o P © ♦ • ♦ • ' # * • • • • • O H o o o O A A A A O O O o o o o H G o 05 œ H 05 g P4 rH rH O A p m G © P © bO m m o G P P •rH O © P , © G G © O •fH P ' •H © © G © © ( G © G © P G bO bO H O bO H bO ^ bO H ÎN T5 © ^ © rH H ©rH H © © © G ^ © ' I - H M G © G P .G © G © G G rH G © Ë © G © © p ÎG © o O •H © Ü O P © bO © © H ‘G G > © O «G rH A 5-1 H > X O P > H O © > «H O > © © 0 •H A rH © © © q © <4 M A G <4 A A rH *© Î G O <4 A A G H o O A A A o G © © P A G S O ' P A Ç Q < 4 CD O T © W © G < û o U © A © G © © © G y > HI I — I 156 C D G G © ÎG P H G Q •H P M G Ph O o ÎG O H H > E h H <4 ÎG i— I ÎS; m < A p X P Q H <4 E H P <4 A O G EN A O O O o o o A O O EN O A O A A E n O O © * # # » » • • • # # # # • # • ■ • » • • • G CO A O A A O O O A O O to O y O A A O O G G A EN CM O A to G G 00 G A G G o o G O O O O O O O O O O O O O O O O O o o o O . * * • • # # » # # # . • * # # s O o O O O O O O o o o o O O o o o o o o © o o o o o G G G G G G Eh © O O o o o o o o O O O O O O O O o O o o •H « • • * • • • • • • • • # * * # X o o O O O O O O o o o o o o o o o o o o © A A O O O A o p G G G G <4 N P •H G O O A EN O O O O O O O O o o EN EN A O O G • ♦ ♦ * • * • • • * * * * # • « * bO O o A A O O O O O O O O o A O A A A O O G . A A A A O O G G A A A G G o O O A A A A C-O EN EN O EN O A O ^ O O O O G * # # * # # # # # # » # # # # » # • • « P O O A A A A A O A A O A O G O A CM O ^ m zû G EN G CM CD G G A © O. CD G G o A A O O O C '-A O O C O O C M . O O O O O A O EN P # •. • • • • • • • • • • • • • • * • • • © I —1 CO A A O A TM O O O A O A O A O CM O en A A A CM EN CD G 05 CM <M A 'th p <4 G O G G © P <£> Tji A O A A CM A A A O O O EN O C M A 05 O A P © * * • • • • • • * • • • • • • « # • • ♦ O r4 O 05 EN A EN 05 CD C M G O O O O A b- EN o ^ EH p to A G EN E n G © 05 G G A A A P O A A ÎN •» © 1 G © G G O © © © © G A G ÎN 0-4 © G © ÎN G ü © O © O G © W © G P O G Ph fH G © CQ © o G © Gi bO O bO T) © P P p ^ p ÎN G G î^ © © G G G G G P © © A G © © ^ G © EH bû O © © © W © G G © ^ G g g o © © b O G ; G G rG X © rG © G G G © © O G G G © G © G P © G © P G © A © R G P hG P © G © © G © © G G G G P G A G G O G "G G Ë G >H © a A G P ra G A O fG fH p L , o p3 M <4 O P fH ^ © © © F) G P4 > Ç> © © a A < © o Q p G pM fH G Eh < < 4 G P G O o td EH EH 157 , — \ A t J P © P M G A G P G g fH O '—■ O G > G G 8 g i m <4 Eh G g G 0 G •H 1 O o ooo O O ooo o o o o o o ooo Ë • • • • * • • . . . © ooo O o ooo o o o o o o ooo G o q o o o G G 1 —1 G G G © OOO o o OOO o o o o o o ooo •H . * • • • • • • • • • • • • X OOO o o OOO ooo o o o ooo © o A A o o A p G 1—1 1 — i IN P *1 — 1 G * r H b O •H 0 4 ü •H P 5 G G O P © G P < 4 g G G © P P © O G E H G g- P h I E N A o E N A E N A O C- A O O O O 0 5 O O • * • • • • • • • • • • • A A O A A A A O A A O C M O o C M o o G C O A A A A A A A EN o O O • • « • • A A O O o A A A A O O O G o # # # « ' • C O C M O O O 05 ^ A C M 05 C O A EN 05 A A C M EN G EN . Tjl A C M A A 05 G • * • A A G C M EN G C O N f» A nf © P O © © b O G © P G © 0 4 © Ü © 'P p4 (4 O > G O G Q <4 ^ CD A 0 P g T S P G Ü O G © A G •G « S ; fH < 4 © © © © OOO OOO O O O OOO • • • # e e • • « o o o OOO O O O OOO o O A A 1 — 1 G A E N o A E N O G 05 O E N O O • • • • • • A A O A A O A A O E N O O A C M C M A C M C M C M EN O A EN O A G A A 'M i CM A G 05 O EN CM O A CM CM G A A CM A G 05 O • • • E N CM O A CM C M A O EN * • • CM O EN A A 05 * # * A G CM A © G A •G © G G O P G G © © •G P <4 1 — I G G bû © © G G G Ë ® © © A ^ p . f3 © G G p G G © T î © © bO © a © p © x n G E h G G P G © G G O G © G S - G o en • • • A O EN CM G O 05 • • • CM O C M CM © a G © G P G © O G p © G G bû © Tf G Ë © © A S G © bû © P G © o G © A © © P G © J> G I —I 158 g ro u p ’ s s u b je c ts . The s i z e of th© ad en o id was sm all o r medium i n m ost of th e s u b j e c t s , b u t as many a s tw en ty p e r c e n t o f th e a t h e t o i d s showed l a r g e ad e n o id m a sse s. The lymph t i s s u e ab o u t th e e u s ta c h ia n tu b e opening was sm all in m ost p a t i e n t s exam ined, a lth o u g h alm o st e i g h t p e r c e n t of th e a th e to i d s showed la r g e amounts of l a t e r a l lym phoid t i s s u e , w hich co u ld be c o n s id e r e d s u s p e c t o f o b s tr u c t iv e c h a r a c te r . Summarizing th e p h y s ic a l f in d in g s o f th e groups o f c e r e b r a l p a l s i e d s u b j e c t s , th e o t o la r y n g o l o g is ts judged t h a t th e e x a m in a tio n s y ie ld e d a v e ra g e e a r , n o s e , and t h r o a t f in d i n g s , in c lu d in g th e "norm al v a r i a t i o n s " found in th e random ly s e le c te d p o p u la c e . They c o n s id e re d th e v a r i a t i o n s among th e v a rio u s s u b d iv is io n s to be m in o r. D is c u s s io n . I n t e r p r e t a t i o n o f th e e x a m in a tio n f i n d in g s m ust rem ain c o n s e r v a tiv e . P o s s e s s io n o f s p e c i f i c " p a th o lo g ic a l" symptoms, f o r exam ple, lym phoid t i s s u e n e a r th e e u s ta c h ia n tu b e or a g ra y c o lo re d tym panic membrane, need n o t be accom panied by d im in ish e d h e a rin g a c u i t y . P o lv o g t and B o rd iey ^^^ have d e m o n stra te d t h a t many c l i n i c a l L. M. P o lv o g t and J . E. B o rd le y , " P a th o lo g ic Changes i n th e M iddle E ar of P a t i e n t s W ith Normal H earing and of P a t i e n t s With a C onduction Type o f D e a fn e s s ," A nnals of O to lo g y . R h in o lo g y . and L ary n g o lo g y . 4 5 :7 6 0 -7 6 9 , Septemb e r , "Ï936V ' 159 f in d in g s u s u a lly a s s o c ia te d w ith im p a ire d a c u i t y need n o t be accom panied by im p a ired a c u ity . C l i n i c a l e x p e rie n c e would te n d t o co n firm t h i s r e s e a r c h f in d in g . The p re s e n c e of a lymph t i s s u e n e a r th e e u s ta c h ia n tu b e i s a good ex ample o f a p o t e n t i a l l y p a th o lo g ic s i t u a t i o n which u s u a l l y does n o t d ev elo p i t s p o t e n t i a l . The lymph t i s s u e m ust o b s tr u c t th e e u s ta c M a n tu b e c r e a t i n g im balance o f p r e s s u r e s on e i t h e r s id e o f th e drum w ith p o s s i b ly i n i t i a t i n g a r e s u l t a n t s t i f f e n i n g o f th e o s s i c u l a r system b e fo re any e f f e c t on th e h e a rin g a c u i t y w i l l be r e a l i z e d . The mere p re s e n c e o f ad en o id t i s s u e may be s u s p ic io u s b u t need n o t i n i t s e l f e f f e c t a h e a rin g im p a irm en t. C lie n ts w ith h e a rin g im pairm ents r e s u l t i n g from a d e n o id a l involvem ent u s u a lly show an h y p e rtr o p h ie d and i n f e c t e d a d e n o id , o r an o b s t r u c t i v e ad e n o id , w ith th e i n f e c t i o n t r a n s m itte d i n t o and th ro u g h th e tu b e . , Some show ce ro u s f l u i d i n th e tym panic c a v i t y a lth o u g h t h i s more advanced s ta g e o f o t i t i s m edia need n o t be e v id e n t. Y et th e c l i e n t who h a s n o t undergone adenoidectom y may r e t a i n th e ad en o id m asses f o r an e n t i r e l i f e t i m e w ith o u t t r o u b l e . The p r a c t i c e h as d e v e lo p e d , t h e r e f o r e , o f f in d in g and d e s c r ib in g th e c h i l d w ith h e a rin g impaiimient and th e n rem oving th e ad e n o id s i f th e y seem o b s t r u c t i v e o r i n f e c t e d , r a t h e r , th a n i n d i s c r i m i n a t e l y p e rfo rm in g ad e n o ïd ec to m ies on th e t o t a l p o p u la tio n , r e g a r d l e s s o f h e a rin g c o n d itio n o r 160 h e a l t h o f th e a d e n o id . V. INTERRELATIONSHIPS OF AUDITORY TEST, MEDICAL, AND CASE HISTORY DATA Type o f L o s s * The p u re to n e a i r c o n d u c tio n m a rg in a l l o s s e s were e v a lu a te d w ith r e g a r d t o p ro b a b le l o c a t i o n of u n d e rly in g p a th o lo g y . Two r e s id e n t o to la r y n g o l o g is ts of 245 th e h o s p i t a l s t a f f made in d e p en d en t e x a m in a tio n s o f and b a se d in d e p en d en t judgm ents on th e fo llo w in g d a ta : a . A ir c o n d u c tio n p u re to n e audiogram s. b . Bone c o n d u c tio n p u re to n e m e a su re s. c . The r e l a t i o n s h i p of a i r and bone c o n d u c tio n m easures d. E a r, n o se , and t h r o a t ex a m in a tio n f i n d i n g s . e . D ise a se and a c c id e n t h i s t o r y of each s u b je c t. f . D ise a se and a c c id e n t h i s t o r y o f th e m other d u rin g th e p reg n an c y . g . H is to r y of h e a r in g im pairm ent i n th e f a m ily . h . R ecord o f any c o n g e n ita l d e fo rm ity in th e s u b je c t and in h i s f a m ily . i . The cc m p le te h o s p i t a l m e d ica l r e c o r d . On th e b a s i s o f th e t o t a l and co m b in atio n s o f a u d i t o r y t e s t d a ta w ith m e d ic a l e x a m in a tio n and c a se h i s t o r y ^ ^ % r . W arren Line and Dr. V ic to r G o o d h ill. 161 m a t e r i a l , each p h y s ic ia n made a Judgment c o n c e rn in g th e lo c a t i o n o f th e p a th o lo g y r e s p o n s ib le f o r th e h e a rin g im p a irm e n t. T h is Judgment i s one t h a t i s r e p e a te d l y made by th e e a r s p e c i a l i s t i n h i s r o u tin e c l i n i c a l p r a c t i c e and p a r t l y d e l i m i t s and d i c t a t e s m e d ic a tio n and tre a tm e n t p ro c e d u re s . Judgment C r i t e r i a and P ro c e d u re . Pour Judgment c a t e g o r ie s were a r b i t r a r i l y chosen as a l t e r n a t i v e s : 1 ) c o n d u c t iv e , 2) p e r c e p tiv e , 3) b o th c o n d u c tiv e and p e r c e p tiv e , o r m ixed, and 4) u n d ec id ed . The " c o n d u c tiv e " ty p e was th e d e s ig n a tio n f o r a Judgment o f p a th o lo g y e x i s t e n t i n th e e x te r n a l e a r o r tym p a n ic c a v i t y , w ith no in v o lv em en t o f any p o r tio n o f th e c o c h le a - e ig h th n e r v e - c o r te x system . The " p e rc e p tiv e " Judgment c a te g o r y was r e s e r v e d f o r th e h e a rin g l o s s th a t a p p e a re d to be th e r e s u l t of p a th o lo g y i n some p o r tio n of th e c o c h le a - e ig h th n e r v e - c o r te x sy stem , w ith th e ab sen ce of any m iddle e a r o r e x t e r n a l e a r a b e r r a t i o n . C r i t e r i o n f o r th e assig n m en t t o th e " c o n d u c tiv e - p e r c e p tiv e " or "mixed" c a te g o r y was a Judgment o f p a th o lo g y s im u lta n e o u s ly e x i s t e n t in th e c o c h le a - n e r v e - c o r te x p o r t i o n of th e a u d ito r y system and in th e tym panic c a v ity o r ex t e r n a l e a r . Where th e s e d a ta d id n o t seem to in d ic a t e a c l e a r 162 p a th o lo g y fo c u s in e i t h e r d i r e c t i o n , th e "u n d ecid ed " c a t e go ry v/as u se d . Unanimous judgm ents were n e c e s s a r y f o r p lacem en t in c a te g o ry . Judgm ents a t v a r ia n c e w ith each o th e r were r e so lv e d by r e c o u r s e to th e "undecided" c a te g o ry . Judgment R e s u l t s . T able XXFiT p r e s e n ts th e judgm ents of th e u n i l a t e r a l and b i l a t e r a l im pairm ents of each p a ls y su b -g ro u p . The s p e c i f i c Judgm ents made by each Judge may be in s p e c te d i n T able XXXIII of th e A ppendix. The Judgm ents of ty p e of p a th o lo g y were made of f o r ty - s e v e n e a r s in t h i r t y - o n e s u b j e c t s . Agreement i n Judg m ents were found ,in f o r t y - f o u r in s t a n c e s . T h is i s an un u s u a lly h ig h c o r r e l a t i o n i n a Judgment p ro c e d u re In v o lv in g two Judges and .th re e a l t e r n a t i v e c a t e g o r i e s . D isagreem ent in Judgments was o b ta in e d i n t h r e e in s ta n c e s and th e s e were a s s ig n e d t o th e "u n d ecid ed " c a te g o ry . . Seven o f th e n in e u n i l a t e r a l l o s s e s were Judged to be m iddle e a r p a th o lo g y m a n if e s ta tio n s . Only one e a r was a f ra n k p e r c e p tiv e ty p e w ith d isa g re e m e n t r e g a r d in g th e p a th o lo g y fo c u s f o r th e l a s t s u b je c t. The Judgm ents of b i l a t e r a l im pairm ents showed a m arkedly d i f f e r e n t c h a r a c t e r . Only te n o f th e t h i r t y - e i g h t e a r s in t h i s g ro u p in g were Judged p u r e ly c o n d u c tiv e in p a t h o lo g ic a l n a t u r e , w ith f i v e a d d i t i o n a l e a rs h av in g b o th 163 B I E -* m f t § o w g lb O fh < D * u a m \ = > © > •p A © rH o pd cd Î H U © < 0 A m -P cd p ) rH © C Q «H pel m © !> •H •P p | Ü PJ no Od C Î o o o © T O Ü pd (3 © î> *H A rH © cd o Pd U U Q ) © 4 J cd rH no M ♦H © c H PD "H K > * H 4 ^ Ü S K O o r 4 rH O rH 03 to to rH to H to iH I — I rH I — I t > s 4 - 3 tù * H • H > n > T O Ü W 4 5 © fH O • H u G 4 5 45 T O o • H I — 1 m © •H .o c d c d bO © g 45 A 45 •H A o o C O < Pd E H o E -i M rH rH rH O rH 03 to lO m rH to 164 m id d le e a r and in n e r e a r in v o lv e m e n t. On th e o th e r h a n d , f u l l y tw en ty -o n e of th e s e e a rs w ere judged to be r e s u l t a n t o f p a th o lo g y somewhere w ith in th e c o c h le a - e ig h th n e rv e - c o r te x p o r tio n o f th e a u d i to r y sy stem . T h is number o f in n e r e a r p a th o lo g ie s i s a l s o augm ented by th e f i v e e a rs r e c e i v ing a "mixed" judgm ent. A t o t a l o f tw e n ty -s ix e a rs out of a p o s s ib le t h i r t y - s i x e a r s , a p p ro x im a te ly tw o - th ir d s o f th e b i l a t e r a l im pairm ent g ro u p , were th u s judged to have some involvem ent of th e e ig h th n erv e system . These d a ta need t o be i n t e r p r e t e d w ith some c a u tio n . A lthough th e judgment c o n s id e r a tio n in v o lv e d a la r g e mass of a u d ito r y t e s t , m e d ica l e x a m in a tio n , and c a se h i s t o r y f i n d i n g s , th e judgment p ro c e d u re m ust s t i l l be re g a rd e d as l a r g e l y s u b je c ti v e . A p art from a u to p sy ex a m in a tio n and th e l i m i t e d o p p o r tu n ity o f o b s e rv a tio n d u rin g some s u rg e ry , th e o t o l o g i s t can n e v e r a c t u a l l y o b se rv e th e in n e r e a r system . S ince no membrane p e r f o r a t i o n s were d is c o v e re d , th e m id d le e a r c o n d itio n was n o t d i r e c t l y o b serv ed e x c e p t a s i t m odi f i e d th e ap p earan ce o f th e tym panic membrane and o f f e r e d o th e r m a n if e s ta tio n s to be i n t e r p r e t e d by th e o t o l o g i s t . C e rta in te n d e n c ie s ap p e ar i n th e d a ta even when c o n s id e re d c o n s e r v a tiv e ly . I t w ould seem from th e s e d a ta t h a t when th e p e r c e p tiv e im pairm ent o c c u rs , i t i s c o n s id e r a b ly more l i k e l y to occur b i l a t e r a l l y in t h i s c l i n i c a l 1 6 5 p o p u la tio n . On th e o th e r hand, th e u n i l a t e r a l im pairm ent of h e a rin g a c u ity i n th e p a ls y group i s l i k e l y to be r e s u l t a n t from m id d le e a r p a th o lo g ic a l p r o c e s s e s . The m id d le e a r im pairm ent may s t i l l , how ever, be found in th e b i l a t e r a l group as w itn e s s th e more th a n f i f t y p e r c e n t who r e c e iv e d a c o n d u c tiv e o r m ixed Judgm ent. A n a ly s is o f th e s e d a ta b y p a ls y su b -g ro u p r e v e a ls i n t e r e s t i n g te n d e n c ie s . An ab sen ce of any s p a s t i c e a r t h a t r e c e iv e d a p e r c e p tiv e ty p e Judgment i s n o ta b le . The th r e e s p a s t i c b i l a t e r a l im p airm en ts w ere judged to be of m id d le e a r c h a r a c t e r , a s w ere th e f o u r u n i l a t e r a l l o s s e s . The d e s c r i p t i o n a g a in d i f f e r s f o r th e a t h e t o i d s . T h irte e n o f th e b i l a t e r a l l y im p a ire d a t h e t o i d s u b je c ts were judged to have b i l a t e r a l in n e r e a r p a th o lo g ie s . F iv e e a rs of th e tw e n ty -s ix in v o lv e d were judged to have an a d d i tio n a l problem in th e tym panic c a v ity , r e c e iv in g th e m ixed ty p e d ia g n o s is . Only one of th e f o u r te e n b i l a t e r a l l y im p aired s u b je c ts were judged t o be f r e e from in n e r e a r in v o lv em en t. The im p a ire d l e f t e a r o f one a t h e t o i d showed p e r c e p tiv e ty p e c h a r a c t e r i s t i c s and b o th ju d g e s s u s p e c te d c o n g e n tia l lu e s a s o t i o l o g i c a l l y im p o rta n t. The o th e r e a r in t h i s s u b je c t was w ith in norm al d e s c r i p t i o n and p erfo rm an c e. I f one can g e n e r a liz e from th e s e d a t a , i t would 166 a p p e a r t h a t when a b i l a t e r a l h e a r in g l o s s i s d is c o v e re d in th e c e r e b r a l p a ls y g ro u p , th e chances a r e good t h a t th e c h i ld i s of a t h e t o i d d i f f e r e n t i a l d ia g n o s is and th e h e a r ing im pairm ent i s o f p e r c e p tiv e o r in n e r e a r n a tu re * When th e u n i l a t e r a l lo s s is fo u n d , i t te n d s t o be o f m iddle e a r n a tu r e and d o e sn ’t seem t o be a s s o c i a t e d w ith any one c e r e b r a l p a ls y ty p e r a t h e r th a n another* VI* ETIOLOGY AND HEARING IMPAIRMENT Background F a c t o r s * A tte n tio n g iv e n in th e p re v io u s s e c tio n to th e ty p e s o f h e a rin g l o s s found i n c e r e b r a l p a ls y c h ild r e n i s p e r t i n e n t t o a c o n s id e r a tio n o f f a c t o r s p o s s ib ly r e s p o n s ib le f o r th e h e a rin g l o s s . The a n a ly s is of th e d a ta a c c o rd in g t o th e fo c u s o f p a th o lo g y somewhat d e l i m i t s th e many p o s s i b le e t i o l o g i c a l b a s e s . The p re v io u s breakdown o f th e d a ta ac c o rd in g t o u n i l a t e r a l o r b i l a t e r a l im pairm ents a l s o te n d s to illu m in a te th e e q u iv o c a l n a tu r e of th e r e s p o n s ib le p a th o lo g y . The h y p o th e s is t h a t th e h e a rin g pathology i n c e r e b r a l p a ls y c h i ld r e n i s r e l a t e d to th e o r i g i n a l p a th o lo g y b a s ic to th e p a l s y i t s e l f rem ain ed to be t e s t e d . In th e c l a s s i c a l an im al ex p e rim e n ts of S ten g e r^ ^ ^ ^^^S* V. S te n g e r, "D ie o t i t i s c h e n Symptôme d e r B â s is - f r a c t u r , " A rch iv f u r K lin is c h e C h i r u r g i e . 6 8 :1017-1030, 1902 167 and Brunner, wherein r a t s and g u in e a p ig s w ere su b m itte d to h ead traum a of I n s u f f i c i e n t f o r c e to f r a c t u r e th e s k u l l , hem orrhages o f v a ry in g d e g re e s were prod u ced in th e i n t e r n a l e a r . By a n a lo g y , and a ls o from th e a u to p sy r e p o r t s of Tiieodore,^^® Manasse,®^® A l e x a n d e r , U l r i c h , S a k a i , ® ® ® and L a n g e , a s i m i l a r p a th o lo g ic change can o cc u r in h u m an -ears. G rove*3^^^ c l i n i c a l r e p o r t in 1947 o f 127 f r a c t u r e d ^^'^Brunner, H ans, and Leopold Schoiibauer, "Zur Bhand- . lung d er S c h a d e lb a s is f r a k tu r e n ," A rch iv f u r K lin is c h e C h i r u r g ie . 1 1 6 :2 9 7 -3 3 1 , 1921. 248g^ Theodore, " B e itr a g e z u r P a th o lo g ie d e r L a b y rin - th e r c h u tte r u n g ," A rc h iv f u r Q h re n h eilk u n d e. 6 1 :2 9 9 -3 0 7 , ^^^P aul Manasse , "Zur P a th o lo g is e b e n Anatomie d e r T ra u m atisch e n T am b h eit." V irchow ’ s A rchiv f u r p a th o lo g is c h e Anatomie und P h y s io lo g ie und f u r K lin is c h e M ed izin , 182-209, 1907. " " : ^^^G. A lex a n d er, "Die H i s t o l i g i e d e r in d e re k te n trauma- t is c h e n L a b y rin th v e r1etzu n g d urch S c h a d e ls c h u s s . F e s t s c h r i f t f u r U r b a n ts c h its c h ," A rc h iv f u r O h re n h eilk u n d e, 5 9 :1 3 -1 8 , A u g u st, 1903. 251 K* U lr ic h , "V erletzu iig des Geh or o rgan s b e i S ch ad e l- b a s i s f r a k t u r e n ," A cta G to la ry n g o lo g ic a (S u p p le m en t), 6 :1 -1 7 0 , 252j^^ S a k a i, "Anat omis che Befunde am n e u s c h lic h e n G ehororgan nach B a s i s f r a k t u r , "A rchiv f u r Q h re n h eilk u n d e, 8 5 :1 8 8 -1 9 7 , May, 1911. " ---------------- 253 W . Lange, "A n atom ische Befunde am G ehororgan nach B a s i s f r a k t u r ," Z t s c h r i f t f u r Q h re n h eilk u n d e, 5 3 :3 7 -4 2 , 1906* E. Grove, "H earing Im pairm ent Due t o C ranio c e r e b r a l Traum a," A nnals o f O tology, R hin o lo g y . and L ary n g o lo g y . 5 6 :2 6 4 -2 7 1 , Ju n e , 1947. 168 and 700 n o n - f r a c tu r e d s k u l l s a f t e r c r a n i o c e r e b r a l traum a con firm ed th e t h e s i s t h a t h e a rin g im pairm ent may o ccu r a f t e r a se v e re c r a n i o - c e r e b r a l a s s a u l t . S in ce c r a n i o c e r e b r a l a s s a u l t i s , from one p o in t of view , e s s e n t i a l l y what o cc u rs as th e c h i l d p a s s e s th ro u g h th e b i r t h c a n a l d u rin g l a b o r , a p o s s ib le e t i o l o g i c a l r e l a t i o n s h i p betw een h e a rin g lo s s and c r a n i o c e r e b r a l trau m a among c e r e b r a l p a ls y c h i ld r e n may be c o n je c tu r e d . C h ild re n w ith c e r e b r a l i n ju r y s u s ta in e d d u rin g th e b i r t h p ro c e s s were g iv e n th e " b i r t h in ju r y " e t io lo g y c l a s s i f i c a t i o n . Gregg^^^ o b serv ed i n 1941 t h a t m a te rn a l r u b e l l a d u rin g th e e a r ly p reg n an cy c o u ld r e s u l t in c o n g e n ita l d e fo rm ity o r d e f o r m itie s o f th e c h i ld . Gordon and I n g a l l s , F a b e r a n d o th e r w orkers have r e p e a te d ly warned r e g a r d in g f a c t o r s i n p r e n a t a l and p a r a n a t a l l i f e r e s p o n s ib le f o r ' d e a th , d e f e c t and d i s a b i l i t y . R u b e lla i s now w ell re c o g n iz e d H. M. G regg, " C o n g e n ita l C a ta r a c t F o llo w in g Ger man M easles in M o th e r." T r a n s a c t i ons of th e O phthalm oloR i- c a l S o c ie ty o f A u s t r a l i a ~ ( 1941)'. 5 :5 5 -4 6 . 1942. ^'^'^John E. Gordon and Theodore H. I n g a l l s , "D eath, D e fe c t and D i s a b i l i t y I n P r e n a ta l L i f e ." A m erican J o u rn a l , o f P u b lic H e a lth . 3 8 :6 6 -7 4 , J a n u a ry , 1948. ^^*^Harold K. F a b e r, " C e re b ra l Damage i n I n f a n ts and In C h ild re n : Some O b se rv a tio n s o f I t s Causes and P o s s i b i l i t i e s o f I t s P re v e n tio n ," A m erican Jo u rn a l o f th e D iseases^ o f C h ild re n . 7 4 :1 -9 , J u ly , 1947. 169 among o t o l o g i s t s a s r e s p o n s ib le f o r some c o n g e n ita l d e a f- ness#^^® H opkins and G u ild e r^ ^ ^ w ere r e c e n t l y fo rc e d to r e v i s e t h e i r a n a ly s e s o f th e p e d ig re e d a t a , w hich ap p e ared in th e f i r s t monograph o f S tu d ie s - H e re d ity i n D eafness p u b lis h e d by th e C larke School R esearch S t a f f , due to th e d is c o v e ry of some r u b e l i a s i n t h e i r sam pling* The i l l n e s s e s o f th e m other d u rin g g e s t a t i o n and e s p e c i a l l y d u rin g th e f i r s t t r i m e s t e r th u s m e r its a t t e n t i o n . C o n g e n ita l a b n o r m a litie s a re o f te n fo u n d in more th a n one a r e a o f th e o r g a n i s m . T h e p o s s i b i l i t y of a c o n g e n it a l l y d e f e c tiv e h e a r in g mechanism in company w ith a c o n g e n it a l l y abnorm al c e n t r a l nervous system p r e s e n ts i t s e l f . F e b r i l e d is e a s e s in th e c h i ld have been re c o g n iz e d as e t i o l o g i c a l l y im p o rta n t f o r c e r e b r a l p a ls y .^ ^ ^ They have a ls o been c o n s id e re d r e s p o n s ib le f o r some in s ta n c e s o f a d v e n tit io u s deafnes s . E i g h t of th e t o t a l sample were 258q. ^fesse X h o eft."M edical P r o g r e s s ; R u b e lla (German M e a s le s )," New England Jo u rn a l o f M e d icin e. 2 3 6 :9 7 8 , Ja n u a ry 26, 1947. O K Q Ruth P. G u ild e r and Louise A. H opkins, C larke School S tu d ie s Concerning th e H e r e d ity of D e a fn e ss. Mono- g ra p h i V P e d ig re e D a ta . 1930-1940 (N ortham pton; C larke School f o r th e D eaf, 1 9 4 9 ), p . i i i . ^^^Gordon and I n g a l l s , l o c . c i t . ■^Faber, op. c i t . . p . 4 -6 . ^^% dw ard P. F o w ler, "The E f f e c t o f S evere I l l n e s s Upon th e H e a rin g ," A nnals of O to lo g y , R hin o lo g y . and L aryn g o lo g y , 4 3 :3 8 7 -4 0 1 , Ju n e , 1934. ----- 170 judged to have s u s ta in e d a c e r e b r a l l e s i o n r e s u l t i n g i n th e p a ls y a f t e r a se v e re i l l n e s s * These r e p o r t s and o p in io n s seem to in d i c a t e t h a t some f a c t o r s c o n s id e re d r e s p o n s ib le f o r th e c e r e b r a l p a ls y c o n d itio n have been co n firm ed by o th e r in d ep en d en t w orkers a s c a u s a tiv e f o r h e a rin g im pairm ent i n norm al in d iv id u a ls * Three h y p o th e se s m ight b e a r c o n s id e r a tio n : 1. The h e a rin g im pairm ent i n th e c e r e b r a l p a l s y sub j e c t i s th e r e s u l t o f th e same p ro c e s s t h a t i s r e s p o n s i b le f o r th e p a r a l y s i s • 2* The h e a rin g l o s s and c e r e b r a l p a ls y a r e cau sed b y s e p a r a te and d i s t i n c t p a t h o lo g ic a l p r o c e s s e s . 3 . Both #1 and #2 above may occur in th e la r g e c e re b r a l p a ls y p o p u la tio n . The r e l a t i o n s h i p betw een th e judged e tio lo g y o f th e c e r e b r a l p a ls y and th e in c id e n c e and d eg ree o f h e a rin g im pairm ent was s tu d ie d . I t was f e l t t h a t s y s te m a tic c o e x is te n c e of h e a rin g im pairm ent w ith p a r t i c u l a r c e r e b r a l p a ls y e t i o l o g i e s m ight i ll u m in a te th e p o s s i b l e i n t e r - r e l a t e d c a u s a tio n s . The l a c k of c o r r e l a t i o n betw een th e s e f a c t o r s , on t h e o th e r hand, would te n d to n e g a te th e h y p o th e s is o f sim u lta n e o u s o r r e l a t e d c a u s a tio n . C o r r e l a t i o n s . The p o i n t - b i s e r i a l c o e f f i c i e n t o f c o r r e l a t i o n was u sed t o su rv ey th e p u re to n e d a t a . C e re b ra l 171 p a ls y o tlo lo g y , p r e s e n t o r a b s e n t, was d e s ig n a te d as th e diehotom ous v a r ia b le w ith th e oontinuton of h e a rin g lo s s th e c o n tin u o u s v a r i a b l e . The p o i n t - b i s e r i a l c o e f f i c i e n t o f c o r r e l a t i o n was chosen fo r i t s p e c u l i a r s e n s i t i v i t y n o t o n ly to th e in c id e n c e of h e a rin g lo s s b u t a ls o to th e m agnitude of th e im p airm en t. The p o i n t - b i s e r i a l c o e f f i c i e n t s o f c o r r e l a t i o n b e tw een c e r e b r a l p a ls y e t io lo g y and h e a rin g l o s s a re shown in T able XXJI. S in ce only one s u b je c t was judged by Dr. Jones to have s u s ta in e d damage due to a p r e n a t a l a c c id e n t, th e c o r r e l a t i o n f o r t h a t e tio lo g y c l a s s i f i c a t i o n was n o t done. The one c h i l d w ith t h a t c l a s s i f i c a t i o n was n o t in th e m a rg in a l l o s s g ro u p . A part from th e e r y t h r o b l a s t o s i s e t io lo g y c l a s s i f i c a tio n , th e c o r r e l a t i o n s betw een h e a r in g l o s s and th e c e r e b r a l p a ls y e tio lo g y c l a s s i f i c a t i o n s a re n e g a tiv e , r e l a t i v e l y s m a ll, and n o t s i g n i f i c a n t . The c o r r e l a t i o n v a lu e s f o r th e elev en e r y t h r o b l a s t o s i s s u b j e c t s , on th e o th e r hand, show a d i s t i n c t l y d i f f e r e n t r e l a t i o n s h i p . A ll of th e r v a lu e s f o r th e e r y t h r o b l a s t o t i c s a r e v e r y h ig h , p o s i t i v e and v e ry s i g n i f i c a n t . The c o r r e l a t i o n s in c r e a s e w ith fre q u e n c y from m id d le C to 4096 c y c le s , w ith o n ly a s l i g h t d e c re a s e a t 4096 c y c le s . The c o r r e l a t i o n s a re te n t o sev en te e n p o in ts h ig h e r f o r th e r i g h t e a r th a n f o r th e l e f t a t th e v a r io u s f r e q u e n c i e s , b u t even th e s m a lle r group of l e f t 172 > m •3 Î 54 e n en o ^ fb t o M C Î 5 il o o Q co S l§ H M M m is P < fH ^ * 1 *aj ( _ I J M ^ o Q m M P m m M il I lO tO rH to <o to L O Oi O C V i rH to rH O O ♦ # • • $ * y* 1 1 I 1 1 • 00 y rH y* rH C Q y rH (M rH rH o • O * * # # • • A 0 3 I 1 1 1 1 u • cS o M O ca 03 to 03 o 45 WCQ t— î rH O lO O 1 — i O O * # • • « * © d iH i 1 1 1 1 © 0 © < M 1 — 1 G) o 03 C" to iH rH O rH lO 1 — f o {x,lO • « « • « • 1 1 1 1 1 iO tO O C Q lO 03 lO O O rH rH rH C V Î • « * # # » 1 1 1 1 1 <0 1 — 1 O O O 03 y o> 1 — i < M rH 00 rH rH O • * ♦ ♦ * 1 1 1 1 1 • 00 rH O H to rH . 00 m xfi rH to rH 00 rH o • O • • • • • • ftCvJ 1 i 1 1 1 ÎH « cd O H rH to rH 03 03 rH 45 î>iC V 2 rH rH rH to rH rH h O O • • # • • • bO t— 1 1 1 1 1 1 •H © fU 0 , Q < © (M lO to O) 03 03 Pi rH rH rH o to rH 1 — 1 P4 tO • * • * 4 4 1 1 1 1 1 co lO 03 !0 00 O 03 to O rH rH to ( — I O C V Î # «. • # « 4 1 1 1 1 1 ,— s I — 1 to -— ' 00 il î>»tO to rH 00 1 1 u II II 1 rH n 45 03 0 J2; rH Î2; © « 0 to oJ —^ H J2Î 1 — 1 " — " © II rH rH 45 0 45 rO . g S 0 03 © T O oS S U M •H O 45 T O © 45 T O 45 © 0 rH u T O d T O «H cj © «S *0 © oj 0 H 0 © «H TS 0 S U S U -ri 45 bO g 45 T O 45 0 •H © © H © O 0 O W O T O rH 'd 45 ÎH rH U O •H o 0 0 45 O 1 — 1 0 P4 M (U < m Q < M T O fH M t> -H 173 e a r v a lu e s ru n s from .4 8 to .74* F u rth e r R e l a t i o n s h i p s . With th e e r y t h r o b l a s t o s i s s u b je c ts th u s s e t a p a r t , f u r t h e r r e l a t i o n s h i p s w ith p re v io u s d a ta w ere exam ined. I t was found t h a t a l l e le v e n w ith e r y t h r o b l a s t o s i s e t io lo g y were a t h e t o i d s , a l l e le v e n had b i l a t e r a l h e a rin g im pairm ent s , and a l l e le v e n had r e c e iv e d a p e r c e p tiv e o r m ixed judgment f o r each e a r . The e x te n t t o which th e y had form ed th e b u lk o f th e p e r c e p tiv e o r mixed judgm ent c a te g o r ie s i s shown i n T ab le X X V II.R etabulated in T&hle'MQftl3.re th e judgm ents o f fo c u s of p a th o lo g y - c o n d u c tiv e , m ixed, p e r c e p tiv e - w ith th e e r y t h r o b l a s t o s i s s u b je c ts ex clu d ed from th e d a t a . The numbers o f p e r c e p tiv e and m ixed judgm ents a r e seen to be re d u c e d from a t o t a l o f tw e n ty -s ix e a rs t o a t o t a l o f f o u r e a r s . Two s u b je c ts th u s rem ain w ith b i l a t e r a l Involvem ent o f th e c o c h le a - e ig h th n e r v e - c o r te x system w ith one e a r h a v in g an a d d i t i o n a l m iddle e a r c c m p lic a tio n . The u n i l a t e r a l d a ta as w e ll a s th e p u r e ly c o n d u c tiv e judgm ents a r e u n a f f e c te d s in c e no e r y t h r o b l a s t o t i c ap p e ared w ith o n ly a u n i l a t e r a l h e a r in g d e v i a t i o n , nor could any be found w ith a s t r i c t l y m id d le e a r p a th o lo g y . When th e a t h e t o i d s of e r y t h r o b l a s t o s i s e t io lo g y a re grouped to g e th e r and compared w ith th e a th e to id s o f o th e r e t i o l o g i c a l c a t e g o r i e s , t h e i r means and s ta n d a rd d e v ia tio n s 174 I g • o 0 3 1 —i rH < D 0 P U rH rH p ; < D > •H • p 0 3 02 02 A © rH O PU 1 —i rH c d 0 0 < ù © 04 45 Q j '0 0 3 rH © •fH M P U - H P U rH 1 —1 © > #H 45 0 3 C O 1 — 1 rH lO o 0 t 5 p u tO rH rH lO 0 O O # o 0 3 © • d 0 P U rH rH îz» © > • H 45 0 3 rH rH p4 © p H ü P U d 0 0 © © P4 45 c d 'd 0 3 rH © •H X ' 0 PU 0) S © î> • H 45 0 3 CO H O 0 d P U rH 1 — 1 rH CO 0 o Q l>^ 45 03 m H d K d O 03 45 © *H O *H 0 0 4 5 4 5 d d - H rH 03 © - H S 0 (d © 0 bO © a 4 5 1 A 45 ‘H 0 O O 1 C O < U P U E 4 O EH 175 a p p e ar as in T able XXVIJI.The d if f e r e n c e s betw een th e means of each fre q u e n c y f o r each group a t each fre q u e n c y were t e s t e d f o r s ig n i f ic a n c e w ith th e t r a t i o m easu re. The means o f th e r i g h t e a r f o r th e e r y t h r o b l a s t o s i s group ru n from 2 5.00 d e c ib e ls a t 256 c . p . s . to 54.55 d e c ib e ls a t 2048 c i p . s . w ith’ b u t a s l i g h t d e c re a s e a t 4096 c y c le s to 53.6 3 d e c i b e l s . The t r a t i o v a lu e s a re a l l w e ll beyond th e one p e r ce n t le v e l* The l e f t e a r m easures a r e from 6 .3 6 to 12.72 d e c ib e ls l a r g e r th a n th e v a lu e s f o r th e r i g h t e a r , b u t th e r i g h t e a r m easu res rem ain la r g e and s i g n i f i c a n t w e ll b e y o n d .th e one p e r c e n t l e v e l . T here can be l i t t l e chance t h a t th e d i f f e r e n c e s shown betw een th e e r y th r o b l a s t o s i s group of a t h e to i d s and th e norm al c o u ld have o c c u rre d by chan ce. D e s p ite th e in c lu s io n in the t h i r t y n o n - e ry th ro b l a s t o s i s s u b je c ts o f th e two re m a in in g b i l a t e r a l im p a ir ment s u b je c ts p lu s th e one b i l a t e r a l and f o u r u n i l a t e r a l c o n d u c tiv e in s t a n c e s , th e means o f th e n o n - e r y th r o b l a s to s is group more c l o s e l y ap p ro ach th e mean v a lu e s o f th e o th e r groups p r e v io u s ly shown in T able X I . O n l y one t r a t i o v a lu e i s s i g n i f i c a n t a t th e f i v e p e r cent l e v e l , t h a t o f 2048 ( c . p . s . ) f o r th e r i g h t e a r . The rem ain in g v a lu e s d id ^^^See page 98. 176 > I g 0 w 1 E H O Q I — I O Q 0 S 1 1 g P q e n I g % 0 H E H 1 Q P U â B hM ^ K g 0 EH. H B g < O K g ' MW C O ‘ îs; CO O < M : M 01 > . g g . (b M o s j - U E 4 O M k EH O « g g § è ë ib C O M A C O M g g Q te C O ^ EH M < :< g (O rH o 00 o o C- o> 0» O rH o 03 02 o ’"• « • • • • O C O O* t o fH H *H CO 0 rH O H* tO ^ C O iH CO to lO iH C O • • « • T O O to lO C O to Ht iH • 02 H* fH iH A 0 • cd o CD l> tO 0~ 1 — i to 0 ) O- CO t o H^ 02 • • • • • « 45 î> sO 00 G O to 02 fH 44 © r4 02 H H © 0 03 © 0 C O t o 03 O 03 to O* H 03 C O lO rH H^ © 02 • • • 0 H CO to ^ 02 03 iH A d 02 fH to C O O CO C O tO rH O 00 to (O « • • • • • uo 00 C O CO 03 M 02 1 — 1 1 — 1 CO C O y l> iO 02 to <0 C O 03 to C O 03 0 ) • • • • • • o C O to 03 to lO fH lO fH rH 1 — 1 t o 03 O- O to to C O tO tO lO o Hi 02 $ H* • • • $ # # T O o H4 02 C O to Hi 03 • 02 tO rH rH H A 0 • c S ü rH O C O O 03 C O 03 02 rH lO £> 02 • « « -P f> iO lO C O to H i 02 fH 0 O rH tO fH H b Û 0 •H © P U 0 lO ( — 1 O C O C O a* 02 to fH o 03 03 © 02 • • • • « • 0 iH 02 Ht 0 - 02 o A lO C O rH H O rH H< O Hi O O C O 03 to LO m (O • • • • • « lO lO C O iO 02 00 H 02 02 H T O 0 # O 0 • O •H <d P *H (d P 'H 1 TO'--' © * 45 © • 45 O O H S C O cd O ^ M cd 0 45 fH P U T O CO P U 0 T O II 0 II 45 W t e EH © t e E H A 1 --1 -W » 0 0 45 M o 177 n o t r e a c h th e minimum s ig n i f ic a n c e demands. The s ta n d a rd d e v ia tio n v a lu e s rem ain la r g e in t h i s group s e n s i t i v e to th e l o s s m easures s t i l l in c lu d e d t h a t skew th e d i s t r i b u t i o n . T able XXIM i s a r e t a b u l a t i o n o f T able 3CV X I w ith th e e r y t h r o b l a s t o s i s s u b je c ts ex c lu d ed . The av e ra g e l o s s e s f o r the speech f r e q u e n c ie s o f n o n - e r y th r o b l a s to s is s u b je c ts a r e now seen to be c o n s id e ra b ly more s i m i l a r to o th e r types,. The e l im in a tio n of m ost of th e more extrem e lo s s e s o f th e a t h e to i d group when th e e r y t h r o b l a s t o t i c s a re e x t r a c te d i s im m ediately a p p a r e n t. V II. DRUG DOSAGE A N D REARING IMPAIRMENT S ince c e r t a i n drugs have been i d e n t i f i e d as e t i o l o g i c a l l y im p o rta n t f o r h e a rin g Impairment, attention was g iv e n to th e r e l a t i o n s h i p betw een drug dosage and h e a rin g im pairm ent i n t h i s g ro u p . As p r e v io u s ly s t a t e d in th e f i r s t s e c tio n o f t h i s c h a p te r , th e tre a tm e n t r e c e iv e d by t h i s c l i n i c a l p o p u la tio n d id not le a n h e a v ily on drug d o sag e. Only o n e - th ir d o f th e e n t i r e group r e c e iv e d one o r more d ru g s . The p o i n t - b i s e r i a l c o e f f i c i e n t o f c o r r e l a t i o n was C. Howard, J r . , "A u d ito ry Im pairm ent Caused by D ru g s," N aval M edical B u l l e t i n . 4 6 :3 8 7 -3 9 1 , March, 1946. 178 M s ' E 4 - . . C O * — *02 Q 0 0 Hi H i A Qr O ÏP C M 02 0) 'g A M ' . s e HI O O C M 02 O A O H O 0 4 C M - rH A C O 0 O 01 * w * (x , eo o C O «E-I m C O C O 'te te H C O & L ■ ^ g i A 04 o 0 i A H O O H te A Q C O C O te A o u A te H O te A A . HM te te < co â H A H t e E H > O w <EH g: H A te 4 5 © A 0 © *H 02 a i s te b O •H A 45 0 % o 0) § 4 5 |3 w te A. 4 5 A t e 45 © 5 *S )0 te W A te A 45 T O te t e © T O 02 O 45 45 0 b O 4 5 te < 4 A A4 5 4 5 te t e © o 02 te 4 5 4 5 T O 0 d b O A t e C O A 03 T O 0 S te te °g te © © A > 0 03 C J 3 C M C M 03 C Q te C M te C M î> to te to te te C M C M te C M 0 0 0 0 0 0 T O *0 te! A te t e te T O 0 0 0 0 0 0 © te te 0 C M 0 to 0 H § te 0 te 0 0 c ü c s J cd d cd 0 0 0 0 0 0 0 4 5 4 5 4 5 4 5 4 5 4 5 © © © © © © 0 U 0 0 0 0 0 0 0 0 0 0 0 t e S S S S 179 a g a in employed to su rv e y th e p o p u la tio n f o r sy s te m a tic r e l a t i o n s h i p s between drug and lo s s * The d a ta in T able t e s t i f y t h a t no such r e l a t i o n s h i p c o u ld be found. The t r i d i o n e , m e s a n to in , p h é n o b a r b ita l, and d i l a n t i n sub j e c t s along w ith t h e i r combinati o n s w e r e grouped t o g e th e r a s d e p r e s s a n ts and a n t i - c o n v u ls a n ts . The r a t i o n a l e f o r t h i s am algam ation was to p ro y id e a l a r g e r sam ple in one group f o r th e c o r r e l a t i o n re q u ire m e n ts . I t was f e l t t h a t th e e f f e c t on th e organism was s u f f i c i e n t l y s im ila r from one d e p re s s a n t t o a n o th e r to j u s t i f y th e g ro u p in g . S in ce th e m ixed dosage group in c lu d e d in d iv id u a ls who had r e c e iv e d d ru g s somewhat d i s s i m i l a r in n a tu r e , i t was d e c id e d t o ex clu d e th e m ixed dosage group o f seven from th e a n a l y s i s . The one s u b je c t g iv e n t h y r o id e x t r a c t was a ls o n o t am enable to group s tu d y . A ll of th e v a lu e s i n T able 3GCX:nx a r e se en t o be s m a ll, some n e g a tiv e , and none d e m o n stra tin g any r e l a t i o n s h ip te n d en cy . Nor does th e p ro stig m in e group s ta n d o u t from th e r e s t even though the a th e to i d s r e c e iv e d th e m a jo r i t y o f p ro s tig m in e p r e s c r i p t i o n s . I t must be r e i t e r a t e d t h a t o n ly t h i r t y - s e v e n r e c e iv e d 265geg T able X, page 91. 180 MM A M O } = ) M Q E H < A M g § ^ A ë 0 A < 4 £ h g g M A o A M p II p H E - * p m < 1 * M M A A M H S C O p te ^ m ë te o A I 4 5 © P te 0 3 o H -^C D • H T O o • C M A • O C M MO o te g & © C M 0 te A te te te C M te 0 3 o H * *Ç 0 0 3 H * o ACM 0 • « ^ 45 MCM 0 Ü O b O 0 te te © « g. © C M 0 te A te ü te te C M 03 t e H H O 0 0 0 4 1 4 • 4 C M C D C J3 te O 0 0 t e * * 4 1 H 03 t e C D O 0 t e 0 1 # « 1 . %> t e 03 0 0 o 0 0 0 • * 1 4 ' ' 4 1 ■ H < 3 3 O 0 0 0 ■ ■ ■ • • 4 1 t e t e M t e t e t e rH . 0 « 1 4 # • 1 O t e CO t e t e te 0 0 * 1 * * * 1 C M t e H* t e O t e 0 t e 4 4 « 4 1 03 H 0 M O t e t e t e # 4 * 1 t e 0 t e î>- t e te 0 t e 4 A t e Ü 4 © 0 03 4 5 § 03 03 t e 4 5 p 4 4 1 t e ' 0 > O t e 0 t e W 0 N 03 H t e t e bO te 03 0 M bO te 6 II t e II 03 P II 0 n 3 A 45 A © 1 A 0 A 4 5 ' — * 0 03 0 0 t e A 4 5 t e 0 © 0 0 0 A P < î A 181 dru g s and th e q u a n t ity of drug dosage was an u n c o n tr o lle d v a r i a b l e . W hether some o th e r r e l a t i o n s h i p would be d i s co v ered betw een h e a rin g l o s s and drug dosage i f th e amount of th e drug co u ld be in c re a s e d can o n ly be c o n je c tu r e d .. W ithin th e framework o f th e c l i n i c a l tr e a tm e n t program , th e q u a n t ity of drug dosage c o u ld n o t be m a n ip u la te d . The l i t e r a t u r e does not r e p o r t any in s ta n c e s o f h e a rin g im p a ir ment fo llo w in g dosages o f th e drugs used w ith t h i s c l i n i c a l g ro u p . CHAPTER V GENERAL DISCUSSION C o n s id e ra tio n o f th e p u re to n e d a ta as p r o p o r ti o n a ll y r e p r e s e n t a t i v e o f th e t o t a l c e r e b r a l p a l s y p o p u la tio n can n o t be done w ith o u t some r e s e r v a t i o n . A 1thought th e sam pling te c h n iq u e was p ro b a b ly s u f f i c i e n t l y random ized, th e n a tu r e of th e o n e - th ir d o f th e i n v i t e d p o p u la tio n t h a t f a i l e d to r e p o r t and t h e i r e f f e c t on th e f in d in g s o f t h i s stu d y can only be c o n je c tu re d . There i s no re a s o n to b e lie v e t h a t th e y r e p r e s e n t a s i g n i f i c a n t l y d i f f e r e n t sam ple. Yet th e p o s s i b i l i t y t h a t o th e r p e r t i n e n t and v a r i a n t f in d in g s l i e submerged in t h a t group re m a in s. The re a s o n s why a p a r t i c u l a r p a re n t w i l l r e a c t fa v o r a b ly to a r e s e a r c h i n v i t a t i o n and th e n e x t p a r e n t sum m arily r e j e c t th e p ro p o s a l i s a sub j e c t w o rth y o f i t s own i n v e s t i g a t i o n . C e rta in te n d e n c ie s have ap p eared in th e d a ta t h a t b e a r upon th e i n i t i a l h y p o th e se s and g u id in g q u e s tio n s . The s i g n i f i c a n t m agnitude o f im pairm ent f o r b o th a t h e t o i d e a rs when compared to th e norm and to o th e r p a ls y c a te g o r ie s seems c l e a r . The r e l a t i v e l y n e g a tiv e in c id e n c e of h e a rin g im p a ir ment i n th e s p a s t i c , r i g i d i t y , tre m o r, and combined ty p e s i s a s n o ta b le a s th e more anom alous n a tu r e o f a t h e to i d h e a rin g a c u i t y . 183 The u n i l a t e r a l im pairm ent was Judged i n alm o st a l l in s ta n c e s to be c o n d u c tiv e ty p e , r e f l e c t i n g m id d le e a r p a th o logy s in c e no e x t e r n a l o t i t i s or c o n g e n ita l m a lfo rm a tio n was found in th e m e d ic a l e x a m in a tio n of th e e a r . That u n i l a t e r a l h e a rin g im p airm en ts in c e r e b r a l p a l s i e d in d iv id u a ls u s u a lly r e s u l t from p a t h o lo g ic a l p ro c e s s e s in th e tym panic c a v ity would be a c o n s e r v a tiv e c o n c lu s io n . The b i l a t e r a l im pairm ents had a c o n s id e ra b ly g r e a t e r p r o p o r tio n of in n e r e a r e ig h th n e r v e - c o r te x p a th o lo g y th a n ap p e ared i n th e u n i l a t e r a l l o s s e s . The m a jo r ity o f th e s e were found t o be in th e a t h e t o i d c a te g o ry . Some m iddle e a r p a th o lo g y was a ls o found in th e b i l a t e r a l group b u t th e in c id e n c e was sm a ll and was co n fin e d to th e lo s s e s of l e s s e r m agnitude. These d a ta and c o n c lu sio n s h e lp e d to d e s c r ib e th e na t u r e of h e a rin g e f f i c i e n c y i n th e d i f f e r e n t i a l l y d iag n o sed groups of c e r e b r a l p a l s i e d i n d iv i d u a ls , w ith group te n d e n c i e s and in te r - g r o u p d if f e r e n c e s c l a r i f i e d . The lo c a t i o n o f o t i c p a th o lo g y u n d e rly in g th e h e a rin g im pairm ents h a s been d e lim ite d p r im a r il y to th e m iddle e a r in u n i l a t e r a l im pairm ents v /ith a m arked in c id e n c e o f e ig h th n erv e p a th o l ogy i n th e b i l a t e r a l im p airm en ts. The a t h e t o i d group formed th e b u lk o f th e b i l a t e r a l lo s s e s and th e b u lk of th e l a r g e r m agnitude l o s s e s . 184 The n a tu re o f otoJ.pgic p a th o lo g y u n d e rly in g th e m iddle e a r p a th o lo g y in s ta n c e s seemed s i m ila r to th e m id d le e a r p a th o lo g ie s e x i s te n t in an o th e rw ise norm al p o p u la tio n . No c o n g e n ita l d e f o r m itie s w ere b o s e rv e d , and th e c h a r a c te r of m id d le e a r o t i t i s found d id not seem u n u s u a l. W hether th e c e r e b r a l p a ls y group h a s some r e s i d u a l d i a t h e s i s making i t v u ln e ra b le to m iddle e a r p a th o lo g y cannot be co m p letely d e te rm in e d from m é d ic a l ex a m in a tio n and from th e s e d a ta . The m ild in c id e n c e o f c o n d u c tiv e ty p e im pairm ent would n o t su p p o rt an h y p o th e s is t h a t a d i a t h e s i s f o r m iddle e a r p a th o lo g y i s in h e re n t in th e g ro u p . I t i s c o n s id e ra b ly more l i k e l y t h a t th e c e r e b r a l p a ls y group and th e component ty p e s do not d i f f e r from o th e rw ise norm al in d iv id u a ls in in c id e n c e of and v u l n e r a b i l i t y to m iddle e a r i n f e c t i o n . The r e l a t i v e l y weaker p h y s ic a l s t a t e o f th e group m ight make i t l e s s r e s i s t a n t t o d is e a s e p r o c e s s e s , b u t th e fre q u e n c y of m e d ica l a t t e n t i o n and ex a m in a tio n p ro b a b ly c o u n te rb a la n c e s th e fo rm er. The n a tu r e of th e in n e r e a r se n so ry n erv e p a th o lo g y i s even more e lu s iv e s in c e th e anatomy i s l e s s a v a ila b le f o r i n s p e c ti o n th a n th e m iddle e a r . The la c k o f any demon s t r a t e d c o r r e l a t i o n betw een h e a rin g l o s s and p o s t n a t a l f e b r i l e d is e a s e s se v e re en o u g h -to m a n ife s t b r a in damage te n d s to argue a g a in s t any h y p o t h e t i c a l r e l a t i o n s h i p betw een 185 h e a rin g l o s s and f e b r i l e d is e a s e s in th e c e r e b r a l p a l s i e d group* The h y p o th e se s t h a t b r a i n damage, d is e a s e s in th e m o th er, o r c o n g e n ita l m aldevelppm ent were r e s p o n s ib le f o r th e o tic p a th o lo g y f i n d l i t t l e su p p o rt when th e s e f a c t o r s a re c o r r e l a t e d a g a in s t th e h e a rin g lo ss * The c o r r e l a t i o n betw een h e a rin g lo s s and e r y th r o b l a s t o s i s is h ig h , p o s i t i v e , and v e ry s i g n i f i c a n t . The c o r r e l a t i o n betw een e r y t h r o b l a s t o s i s and p e r c e p tiv e type b i l a t e r a l im pairm ent i s even h ig h e r . Yet on th e b a s is of th e s e d a ta a lo n e , th e h y p o th e sis: t h a t th e e r y t h r o b l a s t o s i s r e s p o n s ib le f o r th e c e r e b r a l p a ls y was a l s o r e s p o n s ib le f o r th e p a th o lo g y o f th e h e a rin g im pairm ent i s n o t j u s t i f i e d . A l o g i c a l case, may, how ever, be d ev elo p ed to su p p o rt such a c o n te n tio n . To do so makes i t n e c e s s a ry to c o n sid e r b r i e f l y th e dynamics of e r y t h r o b l a s t o s i s . E r y t h r o b la s to s i s * S ince Dr. W iener’ s^^® r e p o r t on r h in c o m p a ta b ility i n 1939, e x c e l l e n t p r o g r e s s has been made in th e d is c o v e ry , d e s c r i p t i o n , and p r e v e n tio n o f ^^^K* L a n d s te in e r and A. S. W iener, "A g g lu tin a b le F a c to r i n Human Blood R ecognized by Immune S era f o r Rhesus B lo o d ," P ro c e e d in g s o f th e S o c ie ty f o r E x p erim e n tal B iology and M e d icin e. 4 3 :2 2 3 , Ja n u a ry , 186 e r y t h r o b l a s t o s i s . L a n d s t e l n e r W i e n e r , 268 b e v i n e , 269 P o tte r,2 '^ 9 L an d e,2” ^^ D o c to r,2"^^ Leonard,2"^6 ly a lle r s te ln ,^ '^ ^ Z i m m e r m a n , ïannet,2'^6 s t i l l e r , 2 '7 ’* ' and o th e r r e s e a r c h e r s L a n d s te in e r and A. S. W iener, l o c ♦ o l t . S. W iener, "Rh F a c to r s In C l in ic a l M e d ic in e ,” J o u r n a l o f L a b o ra to ry and C l i n i c a l M edicine * 3 0 :9 5 7 -9 7 6 , November, 1945. L ev in e , e t a l . , "The R ole o f Iso im m u n iza tio n in th e P a th o g e n e sis of E r y t h r o b l a s t o s i s F e t a l i s , ” Am erican J o u rn a l o f O b s te tr ic s and G ynecology, 4 2 :9 2 5 -9 3 7 , December, 1941. ^"^^Edith L. P o t t e r , "The P re s e n t S ta tu s o f th e r h F a c t o r , ” American J o u rn a l o f D is e a s e s o f C h ild r e n , 6 8 :3 2 -5 8 , J u ly , 1944. ^ L o ttie Lande, " C l i n i c a l Signs and Development o f S u rv iv o rs o f K e rn lc te ru s.D u e t o r h S e n s i t i z a t i o n , ” J o u rn a l o f P e d i a t r i c s . 3 2 :6 9 3 -7 0 5 , Ju n e , 1948. ^"^^Jack M. D o c to r, " K e r n lc te r u s : N e u ro lo g ic a l S eq uelae o f E r y t h r o b la s to s i s F e t a l i s , ” J o u rn a l o f P e d i a t r i c s , 27: 3 2 7 :3 3 4 , O ctober, 1945. r ^"^^Martha F. L eo n ard , "H em olytic D ise a se of th e Newborn ( E r y th r o b la s to s is F e t a l i s ) , " J o u rn a l of P e d i a t r i c s , 2 7 :2 4 9 -2 6 5 , S eptem ber, 1945. ^*^^Harry W a lle r s t e in , ” S u b s t i t u t i o n T ra n s fu s io n : A New T reatm ent f o r S evere E r y t h r o b la s to s i s F e t a l i s , ” A m erican J o u rn a l of D ise a se s of C h ild re n , 7 3 :1 9 -3 3 . J a n u a ry , 1947: 275 H. M. Zimmerman and H. Y annet, " C e re b ra l S equelae o f I c t e r u s G rav is Neonatorum and T h eir R e la tio n to K e r n ic t e r - us, ” Am erican J o u r n a l o f D is e a s e s of C h ild re n , 4 9 :4 1 8 -4 3 0 , F e b ru a ry , 1935. 276 H. Yàhnet and R. L ieberm an, " C e n tra l Nervous System C o m p lic atio n s A s s o c ia te d w ith K e r n l c t e r u s ,” J o u rn a l o f th e Am erican M edical A s s o c ia tio n . 1 3 0 ;335-339, .F e B r u a r 'y ,~ Ï 9 l^ 277 R alph S t i l l e r , "A Follow-Up Study o f T h ir ty -F iv e E r y t h r o b la s to s i s I n f a n t s , ” Am erican J o u rn a l of D ise a se s of C h ild re n , 73:651-662, J u n e ,“ 1947. ; ------------------------ 187 d e se rv e c r e d i t f o r c o n s id e r a b le tim e and e f f o r t on a g ra d u a l l y y ie ld in g problem . Known v a r io u s l y a s e r y t h r o b l a s t o s i s f e t a l i s , ja u n d ic e o f th e newborn, h e m o ly tic d is e a s e o f th e new born, i c t e r u s neonatorum , k e r n l c t e r u s , — th e p a th o lo g y r e s u l t s i n one case among tw en ty r h n e g a tiv e b lo o d f a c t o r fem a les Tsho m ate w ith an r h p o s i t i v e m a le . The r h incom pat ib le m a rria g e seems to occur i n only tw elv e p e r ce n t o f th e p o p u la tio n and e r y t h r o b l a s t o s i s o cc u rs i n o n e -tw e n tie th o f th e iss u e of th e s e m a rria g e s . E r y t h r o b la s to s i s i s th e n p r e s e n t a t b i r t h in about one out o f ev ery th r e e hundred b a b ie s . The r h f a c t o r i s a b lo o d a g g lu tin o g e n , s im ila r to th e f a c t o r s A, .B, .and C, d e te rm in in g th e f o u r m ajor b lo o d groups w hich n a t u r a l l y o cc u rs i n th e r e d b lo o d c e l l s o f ap p ro x im a te ly e i g h t y - f i v e p e r c e n t o f w h ite p e rs o n s . W hether th e r e d c e l l s have o r la c k t h i s f a c t o r de pends e n t i r e l y upon the; g e n e tic -c o m p o s itio n o f th e p a r e n t s . I f a p e r s o n ’ s r e d blood c e l l s c o n ta in th e r h f a c t o r , he i s s a id t o be r h p o s i t i v e . I f h i s re d c e l l s la c k th e r h f a c t o r he i s r h n eg a tiv e d D uring th e f i r s t pregnancy r e s u l t i n g from th e u n io n o f r h p o s i t i v e m ale and r h n e g a tiv e fe m a le , th e m other d ev e lo p s a n tib o d ie s a g a in s t th e f o r e ig n a g g lu tin o g e n o f h e r own c h i ld i n u t e r o . The f i r s t c h i ld i s u s u a lly s a fe from grow ing a n t ib o d ie s . 1 8 8 In th e second o r any su b seq u en t p regnancy o f th e woman s e n s i t i z e d by a p re v io u s r h p o s i t i v e p reg n an c y , th e a n tib o d ie s in th e plasm a o f th e m o th e r’ s b lo o d r i s e in re s p o n s e to f u r t h e r s tim u la tio n to p r e s e n t r h p o s i t i v e p reg n an cy . These a n tib o d ie s may p a ss over i n t o th e c i r c u l a t i o n o f th e f e t u s and h e re cause d e s t r u c t i o n of th e f e t a l r h p o s i t i v e b lo o d . The b lo o d d e s tro y in g d is e a s e p ro c e s s in th e f e t u s r e s u l t i n g from r h f a c t o r in o omp a t ab i l i t y i s th e e r y th r o b l a s t o s i s of t h i s stu d y . Those who d ie b e fo re b i r t h u s u a lly lo o k v e ry plump due to an a c cu m u latio n o f w a te r in th e t i s s u e s ( f e t a l h y d ro p s ). Those who a re b o rn a l i v e seem q u i t e norm al a t f i r s t , but by th e tim e th e y a re a few h o u rs o ld , th e s k in i s o f te n a g o ld en yellow c o lo r which may p e r s i s t f o r e ig h t t c t e n weeks. These b a b ie s a r e anem ic and th e r e d b lo o d c e l l s , e r y th r o c y te s , a re o f te n red u ced to o n e -h a lf o r o n e - t h i r d of th e norm al number. S ince th e s e i n f a n t s a r e so b a d ly in need o f more b lo o d c e l l s , th e organs where b lo o d c e l l s a re form ed, th e l i v e r , s p le e n , and bone marrow, p e rm it c e l l s to 278 Iv an W. Brown, " P re s e n t S ta tu s o f r h F a c t o r ," Am erican Jo u rn a l o f Uursinp:. 4 8 :1 4 -1 7 , Ja n u a ry , 1948. 189 escap e i n t o th e c i r c u l a t i o n b e fo re th e y a r e r i p e . These u n rip e c e l l s a re known a s e r y t h r o b l a s t s and as th e y a re p r e s e n t in th e b lo o d i n la r g e numbers in t h i s d is e a s e , i t i s c a lle d " e r y t h r o b l a s t o s i s . " The n e u r o p a th o lo g ic a l p i c t u r e o f k e m i c t e r u s w a s f i r s t d e s c rib e d b y O rth in 1 8 7 5 . Schmorl^®^ p u b lis h e d th e f i r s t a u to p sy f in d in g s o f t y p i c a l changes i n th e b r a i n . The s t r u c t u r e s m ost commonly im pregnated w ith pigm ent w ere: th e c a u d a te , l e n t i c u l a t e , su b th a la m ic , and d e n ta te n u c l e i , th e th a la m i, th e mammilary b o d ie s , th e crnua ammonis, th e n u c l e i o f th e c r a n i a l n e rv e s , th e o l i v e s , and even p a r t s of th e c e r e b e l l a r c o r te x , as w e ll a s th e a n t e r i o r and p o s t e r i o r h o rn s o f th e s p i n a l c o rd . L a te r in v e stig a to rs^ ® ^ * ^ ® ^ con firm ed Schm orl’ s f in d in g s adding m id b ra in a r e a s and o f f e r ing f u r t h e r d e t a i l s c o n c e rn in g th e c h a r a c te r o f th e de g e n e ra te p e rio d t h a t ta k e s p la c e i n p a t i e n t s who su rv iv e J . O rth , "TJeber das Vorkommen von B ilir u b in k r y - s t a l l en b e i neugeborenen K in d e rn ,” V irchow ’ s A rch iv f u r P a th o lo g is c h e Anatomie und P h y sio lo g ie und f u r t e l i n i s c h e MedizinT 6J]%7?=— ------- -------------------------------------------------- 2B0p^ Schm orl, "Zur K en n tn iss des I k te r u s Neonatorum in sb e so n d e re d e r d a b e i a u f tr e te n d e n G eh irn v eran d e ru n g e n ," V erhandlungen d e r d e u tsc h e n p a th o lo g is c h e n G e s e l l s c h a f f t , 1 5 :1 0 9 -1 1 0 , 1904.---------------------------------------------------------- ------------ 281 Aimmerman and Y annet, l o c . c i t . l o c . o i t . 190 th e n e o n a ta l p e rio d * I n a June 1948 r e p o r t of c l i n i c a l sig n s in h e r s u r v iv o r s o f k e r n l c t e r u s , Lande^®^ o b se rv e d th a t " fo u r p a t i e n t s a r e d e a f* ” P e r tin e n t L a b o ra to ry E v id e n c e * A des, M e ttie r and C u l l e r p r o d u c e d a sm a ll l o c a l i z e d l e s i o n in th e m e d ia l g e n ic u la te o f th e c a t r e s u l t i n g in a tw enty d e c ib e l im p a irm e n t o f h e a rin g a c u i t y in th e c o n d itio n e d re sp o n se ex p e rim en t. K ry te r and Ades^®^ found t h a t a f t e r rem oval of th e a u d ito r y c o r te x and o f th e i n f e r i o r c o l l i c u l i in t h e i r c a ts th e a b s o lu te i n t e n s i t y lim ens ro s e by an av erag e o f f o r t y d e c i b e l s . K ry te r and Ades a ls o r e p o r te d norm al a b s o lu te th r e s h o ld s in the ab sen ce of th e a u d i to r y c o r te x alo n e in th e c a t . Ades^^^ o b serv ed t h a t p o t e n t i a l s co u ld be evoked i n th e s u p e r io r c o l l i c u l i by a c o u s tic s tim u la tio n and t h a t th e s e p o t e n t i a l s c o u ld be g r e a t l y d im inshed by e lim in a tio n o f th e i n f e r i o r c o l l i c u l i o r by s e c tio n in g ^^^L ande, o p .c i t . , p . 698. W . Ades, P. A. M e ttle r , and E. A. C u lle r , " E f f e c t of L esio n s in M edial G e n ic u la te B odies Upon H earing i n C a t ,” A m erican J o u rn a l of P sychology, 1 2 5 :1 5 -2 3 . Ja n u a ry , 1939: '— --------- 285j^^ D. Dr y t e r and H. W . Ades, " S tu d ie s on th e Func t i o n o f th e H igher A c o u stic Nervous C enters in th e G a t,” American J o u rn a l o f P h y s io lo g y , 5 6 :5 0 1 -5 3 6 , O cto b e r, 1943. 286 H. W . A des, "M idbrain A u d ito ry Mechanisms in C a t s ,” J o u rn a l of H eu ro p h y sio lo g y . 7 :4 1 5 -4 2 4 , November, 1944. 191 betw een th e I n f e r i o r and s u p e r io r c o l l i c u l i . T hat th e d e g e n ra tiv e p ro c e s s o f k e r n lc te r u s may dam age p o r tio n s o f th e e ig h th n erv e system in i t s g ro s s d e s t r u c t i o n of b a s a l g a n g lia a r e a i s n o t an u n re a s o n a b le h y p o th e s is . F u r th e r r e s e a r c h in v e s t i g a t i o n s in t h i s a r e a sh o u ld b e de sig n e d to in c lu d e j o i n t a n a to m ic a l s tu d i e s o f d e c re a se d é f y t h r o b l a s t o t i c s by th e n e u ro -a n a to m is t and th e tem p o ral bone a n a to m is t. None o f th e d e t a i l e d d e s c r ip tio n s in th e l i t e r a t u r e o f b r a i n t i s s u e damage in th e s tu d ie s o f e r y th r o - b l a s t o t i c s a t au to p sy have been done by th e e ig h th n erv e e x p e r t. The p o s s i b i l i t y t h a t th e a t h e t o s i s and th e im pairm ent o f h e a rin g a c u i t y r e s u l t from th e same p a th o lo g ic a l p r o c e s s — e r y t h r o b l a s t o s i s — does n o t b e a r a p e s s im is tic n o te f o r th e c l i n i c i a n . R ecent c l i n i c a l and e x p e rim e n ta l reports^®*^ d e s c r ib e th e u se of th e s u b s t i t u t i o n t r a n s f u s i o n . Rh n e g a tiv e b lo o d i s in fu s e d d i r e c t l y th ro u g h th e .u m b ilic a l co rd a t b i r t h , w ith sim u lta n e o u s e x fu s io n o f th e c h i l d ’ s r h p o s i t i v e a n tib o d y -la d e n plasm a. The r e s u l t s have been p ro m isin g . I n t e r e s t i n h e m o ly tic d is e a s e i s h ig h among m e d ic a l r e s e a r c h e r s and th e checking o f e r y t h r o b l a s t o s i s i s ^^"^W allerstein , op. c i t . , pp. 2 5 -2 8 . 192 th e fo c u s of much o f t h e e f f o r t . I t rem ains to be seen w hether th e c o n tr o l o f e r y t h r o b l a s t o s i s w i l l .r e s u l t in th e e lim in a tio n of th e predom inance of. b i l a t e r a l n erv e ty p e a u d ito r y im pairm ent in th e a t h e to i d and in th e e l im in a tio n of th e a t h e t o s i s t h a t now r e s u l t s from k e r n l c t e r u s . UmPTER VI SUM M ARY The p u rp o se s o f t h i s i n v e s t i g a t i o n were (1) t o d e s c r ib e th e n a tu re of h e a rin g e f f i c i e n c y i n r e p r e s e n t a t i v e groups of d i f f e r e n t i a l l y d ia g n o sed c e r e b r a l p a l s i e d c h i ld r e n , d e te rm in in g group te n d e n c ie s and in te r - g r o u p d i f f e r e n c e s ; and (2) to c l a r i f y the n a tu r e of o t i c p a th o lo g y r e s p o n s ib le f o r th e h e a rin g d e f i c i e n c i e s in t h i s p o p u la tio n . The r e se a rc h was conducted w ith th e a c t i v e c o o p e ra tio n and p a r t i c i p a t i o n o f th e O u tp a tie n t O lin ic and th e D epartm ents of O to lary n g o lo g y and C e re b ra l P a ls y a t th e C h i ld r e n ’s H o s p ita l, Los A n g eles, C a l i f o r n i a . The fo llo w in g e x a m in a tio n s and t e s t s were a d m in is te re d : 1. A g e n e ra l p h y s ic a l ex am in atio n and n eu ro m u sc u lar e v a lu a tio n d u rin g w h ic h .a d i f f e r e n t i a l d ia g n o s is of c e r e b r a l p a l s y was made. 2 . A p e d i a t r i c stu d y in v o lv in g p r e - n a t a l d a ta , b i r t h h i s t o r y , and th e d is e a s e and d ev elo p m en tal h i s t o r y o f each s u b j e c t . 3. An e a r , n o se , and t h r o a t ex am in atio n perform ed by a h o s p i t a l r e s i d e n t o to la r y n g o lo g is t and augmented by o to lo g l c a l h i s t o r y and g e n e ra l d is e a s e d a ta o b ta in e d from th e p a r e n t and a v a ila b le m e d ical r e c o r d s . 194 4* A pure to n e a i r co n d u c tio n t e s t of th r e s h o ld a c u ity . 5. A pu re to n e bone c o n d u c tio n t h r e s h o l d t e s t when a m a rg in a l l o s s was o b ta in e d by a i r c o n d u c tio n p ro c e d u re s . 6 . A speech r e c e p tio n th r e s h o ld t e s t u sin g A u d ito ry T e st No. 14, a c o n s ta n t le v e l re c o r d in g o f spondee words develo p ed by th e H arvard P sycho-A coustic L a b o ra to ry . The a u d ito r y t e s t d a t a , o to r h in o la r y n g o lo g ic a l ex am in atio n f i n d i n g s , and m e d ic a l and h e a rin g h i s t o r y were surveyed by two o t o l o g i s t s and judgm ents r e g a rd in g th e p ro b a b le r e s t r i c t e d fo c u s o f p a th o lo g y were o ffe h e d . A ll of th e g a th e re d d a ta were s u b je c te d to s t a t i s t i c a l a n a ly s is and th e e x p e rim e n ta l sub-groups were com pared. A ir co n d u ctio n th r e s h o ld s were compared (1) to t o t a l p o p u la tio n norms r e p r e s e n te d by au d io m eter c a l i b r a t e d z e ro s , and (2) to a c o n tr o l group of f o r t y "n o rm al” h e a rin g non h andicapped c h i ld r e n . The sam pling f in d in g s in c lu d e d th e fo llo v /in g : 1. Of an o r i g i n a l group of 174 s u b je c ts I n v ite d to p a r t i c i p a t e , o n e - th ir d d id not re sp o n d , 2, One s u b je c t in ev e ry seven t e s t e d could n o t meet th e re q u ire m e n ts o f r e l i a b i l i t y and v a l i d i t y a r b i t r a r i l y imposed i n th e t e s t i n g s i t u a t i o n . 195 3. The sample c o n s is te d of t h i r t y - e i g h t s p a s t i c s , f o r ty - o n e a t h e t o i d s , n in e r i g i d i t i e s , two tre m o rs , and e le v e n of a m ixed c l a s s i f i c a t i o n . F iv e a t a x i a s u b je c ts were se e n b u t could not be t e s t e d . 4. The mean av e ra g e age was te n y e a rs and one m onth, ra n g in g from f i v e y e a rs and te n months t o n in e te e n y e a rs and te n m onths. The s ta n d a r d d e v ia tio n was th r e e y e a rs and one month. 5. T w o -th ird s o f th e t o t a l sample were m a le s , one- t h i r d fe m a le s. This r e l a t i o n s h i p was c o n s i s t e n t in th e groups e x c e p t f o r th e r i g i d i t i e s where th e p r o p o r tio n s were r e v e r s e d . 6 . The m a jo r ity o f the s u b je c ts were q u a d r ip le g ic s w ith th e h é m ip lé g ie s co m p risin g o n ly o n e -f o u rth o f th e 8p a s t i c sam ple. 7. The s e v e r i t y p r o p o rtio n s ran k ed in d escen d in g o rd e r : m o d e ra te, m ild , s e v e re , and m ixed. The a t h e to i d s r e c e iv e d a l a r g e r p r o p o r tio n o f " s e v e r e ” d ia g n o se s th a n d id th e o th e r g ro u p s. 8 . The p e rc e n ta g e o f e t i o l o g i e s in th e n a t a l p e r io d p red o m in ated f o r th e t o t a l sample and f o r th e a t h e t o i d , t r e mor, and combined g ro u p s. The " u n d i f f e r e n t i a t e d ” group was alm o st a s la r g e . P o s tn a t a l i l l n e s s seemed to r e s u l t in s p a s t i c ty p e p a l s i e s . A ll o f th e e r y t h r o b l a s t o s i s s u b je c ts 196 were a t h e t o i d s ; o n e -fo u rth o f the a t h e t o i d s were e r y th r o - b l a s t o t i c s . 9 . Almost tw o - th ir d s o f th e t o t a l sample were com p l e t e l y devoid of drug tr e a tm e n t. More th a n f i f t y p e r ce n t of th e a t h e to i d s r e c e iv e d dru g s in te n d e d t o q u ie t in v o lu n t a r y movement. The t e s t s of a u d ito r y th e sh o ld a c u i t y gave th e f o l low ing r e s u l t s : 1 . A s i g n i f i c a n t l y l a r g e r mean and median av e ra g e h e a rin g l o s s a t a l l f r e q u e n c ie s f o r b o th e a r s was found f o r th e a t h e to i d sample when compared to th e norm al r e f e r e n c e z e ro o f th e c a l i b r a t e d a u d io m e te r, and when compared to th e mean th r e s h o ld s of a c o n t r o l group o f f o r t y in d iv id u a ls o f s i m i l a r age. 2. The mean a v e ra g e s o f th e s p a s t i c , r i g i d i t y , and combined groups d id n o t d e v ia te s i g n i f i c a n t l y from th e c a l i b r a t e d mean z e ro r e f e r e n c e . 3 . The r i g i d i t y group showed o n ly sm all and s t a t i s t i c a l l y i n s i g n i f i c a n t d e v ia tio n s from th e mean th r e s h o ld s o f th e c o n tr o l g ro u p . 4. E ig h t o f th e te n d if f e r e n c e s betw een s p a s t i c and c o n tr o l group mean th r e s h o ld s were o n ly chance v a r i a t i o n s , w hile s i g n i f i c a n t b u t sm all d i f f e r e n c e s were found f o r 256 c y c le s , l e f t e a r , and 4096 c y c le s , r i g h t e a r . 197 5 * F or th r e e f re q u e n c ie s of th e l e f t e a r , the d if f e r e n c e s between th e th r e s h o ld s of th e com bined group and th e c o n t r o l group w ere s t a t i s t i c a l l y s i g n i f i c a n t , b u t th e o th e r seven m easures w ere w ith in chance p o s s i b i l i t i e s . 6 . The a t h e t o i d s showed c o n s id e ra b ly more v a r i a b i l i t y and skew th a n d id th e o th e r groups r e f l e c t e d in com pari sons o f sta n d a rd d e v ia tio n s and fre q u e n c y p o ly g o n s. The polygons o f th e o th e r g roups ro u g h ly ap p ro x im ated norm al d i s t r i b u t i o n s . 7. When th e th r e e speech fre q u e n c ie s w ere av e ra g ed , o n e - th ir d o f th e a t h e to i d s showed av erag e lo s s e s o f more th a n te n d e c ib e ls compared to only fo u r p e r c e n t of th e s p a s t i c s . 8 . More th a n o n e -fo u rth of th e a t h e t o i d s rem ained below a tw en ty d e c ib e l a v e ra g e ( th r e e m id d le fre q u e n c ie s ) a r b i t r a r y l i n e , w h ile only one s p a s tic e a r and one combined ty p e s u b je c t had l o s s e s of tw en ty d e c ib e ls or more. 9 . One s u b je c t in e v e ry s ix t h a t w ere a b le to meet th e demands o f th e p u re to n e t e s t were u n a b le t o r e p e a t th e spondee words. 10. The mean a v e ra g e l o s s e s f o r spondee words were l a r g e r th a n th e pure to n e m easu res f o r a l l g ro u p s. A part from th e a t h e t o i d s , some o f th e lo s s e s f o r o th e r groups approached th e s t a t i s t i c a l re q u ire m e n ts of th e t r a t i o 198 t e s t of s i g n i f i c a n c e . The s p a s t i c s c o n s t i t u t e d a la r g e enough p o p u la tio n t o su rp a ss th e one p e r c e n t l e v e l of c o n fid e n c e . 1 1. The a t h e t o i d mean v a lu e s f o r th e spondee m easur es w ere w e ll beyond th e one p e r c e n t l e v e l o f s ig n i f ic a n c e . When th e a t h e t o i d s were com pared to th e s p a s t i c s , th e m agnitude of th e d if f e r e n c e was la r g e enough to g iv e one p e r c e n t c o n fid e n c e d e s p ite th e f a c t t h a t th e s p a s t i e s were a b le to d e v ia te s i g n i f i c a n t l y from th e c a l i b r a t e d z e ro . 12. D iffe re n c e s betw een th e means o f th e two e a rs a t each fre q u e n c y were l e s s th a n one d e c ib e l a t fo u r of th e f i v e f r e q u e n c ie s . At 256 c . p . s . , 1 .5 9 d e c ib e ls s e p a ra te d th e two e a r s . Froduct-m om ent c o r r e l a t i o n s betw een th e e a rs ran g ed from .79 to .8 7 f o r pu re to n e s , r i s i n g to .9 0 f o r th e spondee m e asu res. 13. B i s e r i a l c o e f f i c i e n t s o f c o r r e l a t i o n betw een te n s io n d e s c r ip tio n in a t h e t o i d s and th e continuum o f h e a r ing a c u i t y were sm all and d id n o t su g g e st any r e l a t i o n s h i p betw een th e te n s io n a t h e t o i d or n o n -te n s io n a t h e t o i d and h e a rin g lo s s # 14. Nine s u b je c ts s u s ta in e d u n i l a t e r a l m a rg in a l l o s s es o f f i f t e e n d e c ib e ls a t two f re q u e n c ie s o r tw enty d e c ib e ls a t one fre q u e n c y t o w a rra n t bone c o n d u c tio n t e s t i n g . Four s p a s t i c s , fo u r a t h e t o i d s , and one r i g i d i t y t o t a l l e d th e g roup. 199 . 15. N in e te e n s u b je c ts showed b i l a t e r a l m a rg in a l l o s s e s , w ith a th e to i d s co m p risin g f o u r te e n o f t h i s number. Only t h r e e s p a s t i c s were judged to have b i l a t e r a l im p a ir m en ts. 16. S u b je c ts w ith marked b i l a t e r a l l o s s e s f o r a i r co n d u c tio n s ti m u li te n d e d to have s i m i l a r l y d e v ia n t th r e s h o ld s f o r bone co n d u cted s t i m u l i . No d e f i n i t e te n d e n c ie s were observed f o r th e u n i l a t e r a l im pairm ents. The r e l i a b i l i t y o f th e method of a i r co n d u ctio n th r e s h o ld d e r iv a tio n was s tu d ie d by com paring th e r e s u l t s of th e f i r s t t e s t w ith th e f in d in g s o f a second. A r e p r e s e n t a t i v e sam pling of f o u r te e n s u b je c ts were a v a ila b le f o r com parison, w ith th e fo llo w in g f in d in g s : 1. The av erag e d if f e r e n c e betw een T e s t #1 and T est # 2 , w ith o u t r e g a r d to s ig n , ran g ed from 1 .7 9 d e c ib e ls t o 5*36 d e c ib e ls . The l a r g e s t d if f e r e n c e betw een any p a i r of th r e s h o ld s was te n d e c ib e l s . 2. The d if f e r e n c e s between th e means o f th e two t e s t s ran g ed from .3 5 to 3 .2 2 d e c ib e ls . No p a r t i c u l a r p a t t e r n of d i r e c t i o n of d if f e r e n c e s was o b se rv e d in th e means where f i v e f r e q u e n c ie s showed in c r e a s e s and as many d e c re a se d in mean th r e s h o ld . 3. T r a t i o com parisons o f th e means o f T e st #1 and T est #2 f a i l e d to in d ic a t e any s i g n i f i c a n t d i f f e r e n c e s . 2 0 0 ex cep t f o r an i s o l a t e d in s ta n c e a t 1024 c . p . s * , r i g h t e a r . 4. A s c a t t e r diagram o f a l l f i r s t t e s t v a lu e s on two e a r s p l o t t e d a g a in s t a l l second t e s t v a lu e s d em o n strate d a h ig h , p o s i t i v e , r e c t i l i n e a r r e l a t i o n s h i p . 5. When c o n s id e re d as m easu res of r e l i a b i l i t y , th e a b s o lu te d i f f e r e n c e s , th e t r a t i o s , and s c a t t e r diagram would seem t o i n d ic a t e r e l i a b l e r a t h e r th an u n r e l i a b l e m ethods. The o to r h in o la r y n g o lo g ic a l e x a m in a tio n s y ie ld e d th e fo llo w in g o b s e rv a tio n s : 1 . The two m e d ic a l c o n s u lta n ts c o n s id e re d th e p h y s i c a l c o n d itio n s of t h i s c e r e b r a l p a ls y group to be n o t o u t s ta n d in g ly v a r i a n t from th o s e o f th e g e n e ra l p o p u la tio n , in c lu d in g th e u s u a l amount and k in d o f p a th o lo g ic a l symptom. 2 . The d if f e r e n c e s betw een r i g h t and l e f t e a rs in th e t o t a l group were judged to b e c l i n i c a l l y i n s i g n i f i c a n t . 3. The c e r e b r a l p a ls y s u b je c t showed n o t one in s ta n c e o f c o n g e n ita l m a lfo rm a tio n o f o b se rv a b le s t r u c t u r e s o f th e e a r . 4. Drum p e r f o r a t i o n s were n o t found in any s in g le e a r . 5. A pproxim ately h a l f o f th e t o t a l g ro u p , h a l f o f th e a t h e t o i d s , and s i x t y p e r c e n t of th e s p a s tic s showed r e t r a c t e d ty a p a n ic membranes. These p r o p o r tio n s were con- 20 1 s id e r e d norm al f o r th e a re a . 6 . An o p a le s c e n t drum was n o te d i n th e l a r g e s t num b e r o f th e s u b j e c t s . Toynbee movement was d i s c e r n i b l e in a p p ro x im a te ly f i f t y p e r cen t of th e t o t a l g ro u p . 7* No o u ts ta n d in g v a r i a t i o n s were found in th e ex t e r n a l n o se . 8 . Where d e v ia te d s e p ta were se e n , a n t e r i o r d i s l o c a tio n s were more common th a n p o s t e r i o r d e v i a ti o n s . 9 . No p o ly p i were observ ed and th e anatomy o f th e t u r b i n a t e s was g e n e r a lly norm al. 10. The b u c c a l c a v ity was a v e ra g e "n o rm al” in h y g i en e, d e n t i t i o n , c o lo r and ap p earan ce o f b u c c a l m ucosa, and p a te n c y o f the s a l i v a r y d u c ts . A few h ig h p a l a t e s were o b se rv e d . 11. F a u c ia l t o n s i l s were p r e s e n t in o n e - h a lf to two- t h r i d s o f th e fo u r l a r g e r groups. Most of th e t o n s i l s ap p e a re d p a l e , f ilm , w ith empty c r y p ts , i n d i c a t i v e o f h e a lth y anatomy. 1 2. Edematous t o n s i l s ap p eared in o n e - fo u rth o f th e s p a s t i c s and i n f o u r te e n p e r c e n t of th e a t h e t o i d sam pling. 13. The pharynx ap p e ared norm al in the m a jo r ity . 14. Adenoids w ere p r e s e n t i n f i f t e e n p e r cen t or more of each group ex c ep t th e combined ty p e . The s iz e of th e adenoid was sm a ll or medium in m ost o f th e s u b je c ts . 20 2 b u t tw en ty p e r cent of th e a t h e to i d s showed la r g e ad en o id m asses. On th e b a s i s o f th e i n t e r r e l a t i o n s h i p s of a u d itr o y t e s t d a ta w ith m e d ic a l ex am in atio n and case h i s t o r y d a ta , each o t o l o g i s t judged th e l o c a t i o n of th e p a th o lo g y re s p o n s i b l e f o r th e h e a rin g im pairm ent. The judgment r e s u l t s were : 1 . Agreement was found betw een two judges in th e in dependent judgm ents of f o r t y - f o u r out o f f o r ty - s e v e n e a r s . 2* Seven o f th e n in e u n i l a t e r a l lo s s e s were judged to be m iddle e a r p a th o lo g y m a n if e s ta tio n s . Only one e a r was of p e r c e p tiv e type w ith disagreem ent r e g a rd in g p a th o lo g y fo cu s f o r th e l a s t s u b je c t. 3 . A pproxim ately tw o - th ir d s o f th e b i l a t e r a l im p a ir ment group were judged to have some involvem ent of th e c o c h le a - e ig h th n e r v e - c o r te x system . 4. F if t e e n of th e e a rs i n , t h e b i l a t e r a l group were judged to have some r e s p o n s ib le m iddle e a r p a th o lo g y . 5. F iv e of th e b i l a t e r a l group e a rs w ere judged to be o f m ixed ty p e - b o th co n d u c tiv e and p e r c e p tiv e . 6 . A ll of th e u n i l a t e r a l and b i l a t e r a l lo s s e a rs of th e s p a s t i c group r e c e iv e d co n d u c tiv e ty p e c l a s s i f i c a t i o n s . Not one p e r c e p tiv e judgment was g iv e n to th e s p a s t i c g roup. 203 7 . Only one of th e f o u rte e n b i l a t e r a l l y im paired s u b je c ts was judged t o be f r e e from in n e r e a r in v o lv em en t. When th e r e l a t i o n s h i p s betw een judged e t io lo g y o f c e r e b r a l p a ls y and h e a rin g im pairm ent was s tu d ie d by u se of th e p o i n t - b i s e r i a l c o r r e l a t i o n te c h n iq u e , th e fo llo w in g r e s u l t s were o b ta in e d : 1 . The c o r r e l a t i o n s betw een h e a rin g lo s s and p r e n a t a l i l l n e s s , i n ju r y a t b i r t h , c o n g e n ita l anom aly, p o s t n a t a l i l l n e s s , and th e u n d i f f e r e n t i a t e d c l a s s i f i c a t i o n g ro u p s were n e g a tiv e , r e l a t i v e l y sm a ll, and n o t s i g n i f i c a n t 2 . A ll of th e r v a lu e s f o r th e e r y t h r o b l a s t o s i s s u b je c ts were v e ry h ig h , p o s i t i v e , and v ery s i g n i f i c a n t . The c o r r e l a t i o n s te n d e d to in c re a s e w ith fre q u e n c y . 3. A ll e le v e n o f th e e r y th r o b 1a s t o t i c s were a t h e t o i d s . 4. A ll elev en of th e e r y th r o b la s t o t ic s had a b i l a t e r a l h e a rin g im pairm ent. 5. A ll e le v e n r e c e iv e d a p e r c e p tiv e o r mixed judgment f o r each e a r . 6 . The b u lk o f th e b i l a t e r a l p e r c e p tiv e o r m ixed judgment e a rs were shown to be e r y t h r o b l a s t o s i s and a t h e t o i d s u b j e c t s . 7. When th e e r y t h r o b l a s t o s i s s u b je c ts were ex clu d ed from th e a t h e t o i d g ro u p , th e rem aining t h i r t y a t h e t o i d 204 s u b je c ts showed th r e s h o ld m easures c o n s id e ra b ly c l o s e r to norm al. The p o i n t - b i s e r i a l r e l a t i o n s h i p s betw een drug dosage and h e a rin g l o s s were found to be sm a ll and i n s i g n i f i c a n t . The p o s s i b i l i t y was c o n s id e re d t h a t e r y t h r o b l a s t o s i s i s a r e s p o n s ib le e t i o l o g i c a l f a c t o r b o th f o r a t h e t o s i s and f o r th e b i l a t e r a l p e r c e p tiv e ty p e h e a rin g im pairm ent of t h a t a t h e t o i d . 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Putnam, "A Group Study o f th e E f f e c t o f G lutam ic Acid Upon M ental F u n c tio n in g in C h ild re n and A d o le s c e n ts ," Psychosom atic M edicine. 9 :1 7 5 -1 8 3 , M ay-June, 1947. Zimmerman, F. T ., and Herman Y annet, "C e re b ra l S equelae of I c te r u s G ravis Neonatorum and t h e i r R e la tio n to K e rn ic t e r u s ," American J o u rn a l o f D ise a se s of C h ild re n . 49: 419-430, F e b ru a ry , 1935. G. PUBLICATIONS OF LEARNED ORGANIZATIONS Bucy, Paul G ., " C o r tic a l E x t i r p a t i o n in T reatm ent of In v o lu n ta ry Movements," P ro c eed in g s o f th e A s so c ia tio n f o r R esearch in Nervous and M ental D is e a s e s . XXI. B a ltim o re : W illiam s and W ilkins Company, 1942. pp. 551-595. C h ild re n With C e re b ra l. P a lsy - A R eport on th e Problem in C a lT fo rh fa and a Suggested Program f o r T h e ir C are. Sacram ento: S ta te D epartm ent of P u b lic H e a lth and E d u c a tio n . 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W iener, " A g g lu tin a b le F a c to r in Human Blood R ecognized by Immune S era f o r Rhesus B lood," P ro ceed in g s o f th e S o c ie ty f o r E x p erim e n tal B iology and M ed icin e. 43:223, J a n u a ry , 1940. . 231 L id d e l l, E* G* T ., and G. S. S h e rrin g to n , "R eflex e s in Response t o S tr e tc h (M yotonic R e f le x e s ) ," P ro ceed in g s o f th e Royal S o c ie ty o f London. B 96;212-242, May, 1924. L i t t l e , W. J . , "On th e In flu e n c e of Abnormal P a r t u r i t i o n , D i f f i c u l t L ab o rs, P rem ature B ir th and A sphyxia Neona torum on th e M ental and P h y s ic a l C o n d itio n o f th e C h ild , E s p e c ia lly in R e la tio n to D e f o r m itie s ," T rans a c tio n s of th e O b s t e t r i c a l S o c ie ty o f London. I I I . I s é ï . London: Longman, G reen, Longman, and R o b e rts, 1862. 480 p p . Newhart, H orace, and S c o tt N. R eger, " S y lla b u s of Audio m e tric P ro c ed u res in A d m in is tra tio n o f a Program f o r th e C o n se rv a tio n o f H earing of S c h o o l. C h ild r e n ," Supplement t o th e T ra n s a c tio n s of th e American Academy o f Bphthalm ology and O to la ry n g o lo g y , Omaha: Douglas m E tiV g " ■€ompa-gy,~l94ÏÏ; ' 28 pp."... N ile s , May W atrous, "Use o f G eneral Sem antic T echniques in P h y s io th e ra p y ," P apers From th e Second American Congress on G eneral S em a n tic s. e d lte d b y M. K endig. C hicago: I n s t i t u t e o f G eneral S em antics, 1941. pp. 183-186. P h e lp s, W inthrop M., " P re s e n t Methods Aimed a t T o ta l C o n tro l of th e C e re b ra l P a lsy Program ," P ra c e e d in g s o f th e C onference on th e C e re b ra l P a ls ie d t h i l d . Los A ngeles: C a lif o r n ia S ocleby f o r C rip p le d C h ild re n , 1946. P p. 28-37. "R eport o f th e Committee on th e C o n se rv a tio n o f H earing o f th e Am erican Academy o f Ophthalmology arid O to la ry n g o lo g y ," T ra n s a c tio n s o f th e American Academy o f Ophthalmology and O to la ry n g o lo g y . 4 5 :2 5 -2 8 , 1940. "R eport o f th e Committee on th e Cons e r v a tio n o f H earing of th e American Academy o f Ophthalmology and O to la ry n g o l ogy, ^ransahtj^pns o f th e American Academy o f O phthal- ’ m o lo g y a n d O to la ry n g o lo g y . 4 6 :4 8 . 19%I D. BULLETINS B u l l e t i n No. 38. M in n e a p o lis: Audio Development Company, 1946. 2 pg. 232 G e n e ra liz e d Age and Sex T rends i n H earing L o ss. N a tio n a l H e a lth Survey, H earing Study S e r ie s , B u l l e t i n # 7 . W ashington D. C .: tJ. S. P u b lic H e a lth S e rv ic e , 1938. 35 p p . , v i . Normal H earing By A ir and Bone C onduction. N a tio n a l H ealth Survey, H earing Study S e r ie s , B u l le t in # 4 . W ashington D. C .: U. S. P u b lic H e a lth S e rv ic e , 1938. 18 pp . v i . Normal H earing f o r Speech a t each Decade of L if e . N a tio n a l H e a lth S urvey, H earing Study S e r ie s , B u l l e t i n # 5 . W ashington, D. C .: U. S. P u b lic H e a lth S e rv ic e , 1938. 20 pp. v i . P re lim in a ry A n a ly sis o f A udiom etric D ata in R e la tio n to C l in ic a l H is to ry o f Im p aired H earin g . N a tio n a l H e a lth Survey, H earing Study S e r ie s , feu lle b in # 2 . W ashington, D. G .: Ü. S. P u b lic H e a lth S e rv ic e , 1938. 27 pp. P re v a le n c e o f A u ral Fat hology and C l i n i c a l H is to ry o f & p a ire 3 ^ H e a rin g Among Males and Fem ales o f V arious A ges. N a tio n a l H e a lth Survey, H earing Study S e r ie s , B u l l e t i n # 3 . W ashington, D. C. ; U. S. P u b lic H e a lth S e rv ic e , 1938. 6 pp. Sex D iffe re n c e s and Age V a r ia tio n s in H earing Loss in R e la tio n to S tage o f iO eafness. N a tio n a l H e a lth Survey, H earing Study S e r ie s , Ë u l ï e t in # 6 . W ashington, D. C .: TJ. S. P u b lic H e a lth S e r v i c e / -1938. 31 p p ., v i i i . S ig n if ic a n c e . Scope, and M ethod, of a C lin ic a l I n v e s t i g a t i o n of H earin g in th e G en eral P o p u la tio n . N a tio n a l H e a lth Survey, H earing Study S e r ie s , B u l le t in # 1 . W ashington, D. C. : II. S. P u b lic H e a lth S e rv ic e , 1938. 30 pp. Speech T ra in in g f o r S p a s ti c a . New York: D iv is io n f o r P h y s ic a lly H andicapped o f th e Board .of E d u c a tio n , 1945- 1946. 40 pp. E. ENCYCLOPEDIA ARTICLES P h e lp s , W inthrop M., " S p a s tic P a r a l y s i s ." The C yclopaedia of M ed icin e. S urgery ahd S p e c i a l t i e s . XI, 203-215, 1945. 233 P. UNPUBLISHED MATERIALS Hansen, R uth, "M uscle R e ed u c atio n i n C e re b ra l P a lsy With T ra in in g o f P a re n ts in Home A d m in is tra tio n o f T r e a t m e n t." U npublished M a s te r’ s t h e s i s . The U n iv e rs ity o f S ou th ern C a l i f o r n i a , Los A n g eles, 1937. 207 pp. W olfe, W illiam , "A C l i n i c a l E v a lu a tio n o f F i f t y C e re b ra l P a ls ie d I n d iv id u a l s ." U npublished D o c to r’s d i s s e r t a t i o n . S ta te U n iv e r s ity o f Iowa, Iowa C ity , 1947. 92 p p . APPENDIX 255 (Name) (A ddress) Dear Mrs. We e n c lo se an appointm ent f o r a s p e c ia l h e a rin g e x a m in a tio n , s e p a r a te and d i s t i n c t from any o th e r h e a rin g ex am in atio n your c h i l d may have h a d . P le a s e come f o r t h i s ap p o in tm e n t, even i f you b e lie v e your c h i l d ’ s h e a rin g norm al, as we a r e making a s p e c i a l stu d y o f th e h e a r in g o f c e r e b r a l p a l s i e d p a t i e n t s . I f you f i n d i t im p o ssib le to be h e r e , p le a s e te le p h o n e th e H o s p ita l, OLympia 1181, E x te n sio n 44, to change th e a p p o in t ment to a n o th e r d a t e . Very t r u l y y o u rs, CHILDREN’S HOSPITAL SOCIETY (M iss)Rose M. W ell D ir e c to r Out P a t i e n t D epartm ent RMW :B FIGURE 9 COPY OF LETTER TO PARENTS INVITING RESEARCH PARTICIPATION 236 0 ) -p C E J P § II H M E 4 O K C O m p C O < m P m o p O si d g E-i O O § P (D P 4 i •H P 4 I — 1 rH 2 C O îJ rH n 3 *>0 < D rH 0 ) c s 3 rH g O a rH I G P «H o C O _T ^ X S O U A rH O c d O T Ï ! x 3 o o o p •H P ^ a < D I — I O rH C O • * < D T j • X G O rH cd rH cd W Î H O (p s P-i t s 0 •xP -p g o cd cd S H 6 P p 3 C 0 Î H M D - p O 0 > O B A X O G • X A cd TJ U 0 U o •H 0 0 A o 0 J h A P 0 0 a O • x • x * r H P P P B B O 0 © G g g p 0 G < H ArH 0 0 rH G G 0 0 0 rG G G G B E -i B rH rH G 44 rH 0 X S 0 rH 0 rH rH 0 S 0 g O G p A O 0 'G O G G rH O 0 O îxî O O P 0 G O P G 0 A nj 0 "X p p Ü o 0 p G 0 G p 0 O 0 G P •H ï* 0 rH Ü rH W G 0 Xi rH 0 « X & •§ •X A 0” n d G 0 M p d p e G o 0 X G. A a ' o 0 G A P O 0 G o • x « X * t H P p p G G o 0 © G G o G 0 G « M ArH W T O rH G G 0 0 0 P G G G E H 54 o rH m C i3 M A â G 0 rH G T O G 0 G f c iÛ ü rH 0 G •H G 1 A G P 0 P X i G 0 p G 0 G <tî rH 0 0 l> > 0 N A 0 a G 0 P P 0 A A rH p G 0 © P «H T O 0 G G 0 T O A N A G rH G •H p 0 0 A G G 0 G 0 3 •H «H 0 T O G N P 0 G •H A A |xî G rH G >^»P 0 •H 0 X 0 A 0 0 0 0 A X ü P C O CJ H ^ 0 0 5 4 A P 0 H 54 0 0 A M A 0 (O 237 I 0 T O o 0 1 A O G O A O O o o A A P P G G 0 0 A A A A 0 0 Ü O X X 0 0 © < D # 50 tiO P 0 0 J G G G 50 0 0 A > > © < < M 50 G • H P P O 0 G 50 G •X 0 T O A G A 0 g X P G G A A 'd 0 a o A 0 G -G A & T O 1 T O 0 A P A A 0 A i o G 0 G 0 I — I G § • . A 0 •X N 0 * H A CO 0 A 0 G A P G P 0 O P a «xp . 0 1 3 G 0 G 0 0 A G T O 0 «X 0 0 « X G A 1 3 A 0 0 A T O O •X A 0 o • 5 0 P 0 *G G 0 0 T O A i3 0 G t G 0 > A P O < O < 4 O • X T O T O 5 0 5 0 0 G P A •X A 0 T O G G - x G |> 5 P T O 0 _ 0 0 rG i3 o 0 I •x S T O G P G Ë o Fs A î>» A Ü O « X 0 T O A P 0 G A © P G o G O T O 0 l3 O G A I i3 © O o A 0 G 0 A G 5 0 O !>s A P P A 0 G A P § T O P 0 ■g k ) G T O 0 . 5 O A G 0 O p A A C O E H T O •xG 0 0 0 A T O A T O A O A A S A A A T O G o 5 4 P 0 G N 0 A P T O A 1 0 G A 0 0 P G 0 0 A P 0 # x P A 0 G X P G 0 g O 0 N # % A •H 0 O T O A C O P T O <c 1 A © 0 0 G 1 — 1 0 A G G A p 0 p •H G A G G P © ^ P 0 G A: G o O î> a « X 0 o P 0 O ^ 50 A Fa P 0 0 A o A G p G A O 0 P G O P > G P M A 0 0 A T O A T O G 0 P • X 0 0 T O C 3 A P G A 0 H 0 A '~ - O A • > P 0 Ü * x A A 50 0 P 0 0 0 G G G *H G P 0 0 ü . 0 G TOP 0 > o p 0 A < O <d P T O • • • • 1 o •H A 0 o G p T O T O T O O •H 5 0 P G î» P G X p A 0 G A O p O P A G © G A O 0 N 0 g T O P •H P Fj 0 A CO < P ^ I •X^ K O M o G ; Iz ; M A S P O M o M E H « g § S A 5 4 O C0 A Î o 0 - 90- 256 512 1024 2048 256 512 1024 FIGURE 11 2048 M EAN AUDIOGRAM FOR FORTY NORM AL CHILDREN USED AS CALIBRATION GROUP 238 4096 R ight Mean - .8 8 -1 .6 3 .50 -1 .2 5 S.D . 5 .8 0 4 .2 4 4 .1 7 5.56 L e f t Mean -2 .7 5 -2 .2 5 - .6 3 - .8 8 S.D . 4 .0 2 4 .3 2 3.9 0 3 .8 5 4 P Le gene Rigl Lef- . t e a r ; e a r ___ — — ■ — '3,25 4 .6 8 ■3,75 4 .7 1 4096 239 CEREBRAL PALSY CHILDREN AUDIOMETRIC RESULTS Case Number D ate t e s t e d Name Mean L o ss, A ir: (012*2048) Speebb. R ecep tio n (Spondees) I n t e n s i t i e s o f Masking U sed: 128 256 Rt -Lt . • • # . . . . ft # ft ft ft f t ft f t . . ft. ft ft ft ft f t f t f t f t Notes r e R e l i a b i l i t y and V a lid ity # and O th e r................................................ .... F requency ( c . p . c . ) 512 1024 2048 4096 8192 H E A R I N a L 0 8 8 (db) 0 1 0 20 50 40 60 60 70 80 90 Ï 1 -------- ---- -------- \-------------------------- 1 . . I : -........... _________I i ... ...... .— 1...... ...... . . i 128 256 512 1024 2048 4096 8192 : A ir Red: AD 2 Bone B lue: AS 0 : AD: X: AS: M; O pposite e a r masked : T h re sh o ld b e fo r e m asking FIGURE 12 AUDIOGRAM FORM USED IN RESEARCH 240 L i s t 5-A L i s t 5-B doorway h o rse sh o e ea rth q u ak e ic e b e r g a i r p la n e b eeh iv e w hitew ash doorm at p lay g ro u n d scarecro w b ir th d a y workshop o h u rc h b e ll f o o t s t o o l n o rth w e st p la tfo rm hardw are lig h t b u lb s ta irw a y w ayside eyebrow ja c k k n if e d o o rs te p h o rse sh o e e a rth q u a k e ic e b e rg o atm eal p la y n a te r a i l r o a d schoolboy b la c k b o a rd eardrum arm c h air b la c k o u t cowboy greyhound shipw reck to o th b ru s h , sid e w alk w ashboard b la c k b o a rd eardrum a i r p la n e beeh iv e b ir th d a y doorm at ic eb o x o u ts id e backbone cookbook schoolhouse sundown bloodhound f a r e w e ll shipw reck to o th b ru s h sch o o lh o u se sundown m ousetrap pancake cowboy greyhound p lay g ro u n d scarecrow w ild c a t y a r d s ti c k s u n s e t woodchuck lo o k o u t p ad lo ck arm c h a ir b la c k o u t coughdrop grandson d a y lig h t highway n o rth w e st p la tfo rm sh o tg u n toyshop d a y lig h t highway c h u rc h b e ll f o o t s t o o l b a s e b a ll daybreak f i r e f l y l i f e b o a t rainbow s t a r l i g h t r a i l r o a d schoolboy w hitew ash workshop coughdrop grandson oatm eal playm ate h e a d lig h t l i g h t b u l b ic e b o x o u ts id e hardw are mushroom s u n s e t woodchuck backbone cookbook f i r e f l y l i f e b o a t lo o k o u t p ad lo ck d o o rs te p hotdog rainbow 8t a r l i g h t s ta irw a y w ayside dugout hous ework sid e w a lk w ashboard eyebrow ja c k k n ife m ousetrap pancake bloodhound f a r e w e ll sh o tg u n toyshop w ild c a t y ard s t i c k h e a d lig h t mushroom b a s e b a ll daybreak dugout housework doorway hotdog FIGURE 13 LISTS OP SPONDEE W ORDS (RECORDED) USED IN SPEECH RECEPTION THRESHOLD TEST TABLE XXXI ANALYSIS OP SUBJECTS GIVEN "MIXED" RATING OP SEVERITY .841 Type M ild- M oderate M ild- Severe M oderate- S evere M ild-M oderate- S evere S p a s t i c i t y 6 1 1 A th e to s is 2 1 R ig id ity 2 Tremor ”Combined" 1 ] L 1 T o ta l Sample 11 2 1 242 TABLE XXXII AGE, EXTENT OP INVOLVEMENT, AND SEVERITY OP THE HANDICAP IN THE TEST-RETEST RELIABILITY GROUP S u b je c t Age* E x te n t Severity- S p a s tic #11 6-8 H em iplegia M ild #29 12-0. T r ip le g ia M ild #31 6— 6 Q u a d rip le g ia Legs s e v e re , arms m ild #34 10-1 Q u a d rip le g ia S evere A th e to ld #s 10-11 Q u a d rip le g ia S evere #5 7-10 ‘ Q u a d rip le g ia M oderate #6 8-3 ^ a d r i p l e g i a M ild #13 10-11 Q u a d rip le g ia M ild #19 9-10 , Q u a d rip le g ia M oderate #20 9-4 Q u a d rip le g ia S evere #29 . 16-11 Q u a d rip le g ia M oderate #33 8-1 Q u a d rip le g ia M oderate #41 12-0 Q u a d rip le g ia S evere R i g id ity •- #5 6-8 H em iplegia M oderate ■ » Age a t f i r s t t e s t TABLE XXXIII SPECIFIC JUDGEMENTS BY TW O JUDGES OP HEARING LOSS TYPE R ig h t Ear Judge #1 Judge #2 L e ft Ear Judge #1 Judge #2 S p a s tic s #1 G C #9 G G #14 C G G G #16 C G G G #22 C G #36 G G #42 C G G G A th e to id s #1 G G #5 P P P P #6 M M P P #8 P P #10 P P P P #11 P P P P #12 P P P P #13 P P P P #18 P P P P #19 P P P P #20 C G #21 P P P P #25 C P #26 G G G G #29 M M M M #30 P P P P #52 P P P P #56 M M M M R i g id ity • #4 G G Tremor #2 C M G G Combined #1 G G G P

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Asset Metadata

Creator Lassman, Frank M. (author)

Core Title A clinical investigation of hearing deficiencies and some possible etiological factors in a group of cerebral palsied individuals

Contributor Digitized by ProQuest (provenance)

Degree Doctor of Philosophy

Degree Program Speech

Degree Conferral Date 1950-08

Publication Date 07/05/1950

Defense Date 07/05/1950

Publisher Los Angeles, California (original), University of Southern California (original), University of Southern California. Libraries (digital)

Tag Health and Environmental Sciences,OAI-PMH Harvest

Format theses (aat)

Language English

Permanent Link (DOI) https://doi.org/10.25549/usctheses-oUC11255982

Unique identifier UC11255982

Identifier DP31983.pdf (filename)

Legacy Identifier DP31983

Dmrecord 827809

Document Type Dissertation

Format theses (aat)

Rights Lassman, Frank M.

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Type texts

Source University of Southern California (contributing entity), University of Southern California Dissertations and Theses (collection)

Access Conditions The author retains rights to his/her dissertation, thesis or other graduate work according to U.S. copyright law. Electronic access is being provided by the USC Libraries in agreement with the author, as the original true and official version of the work, but does not grant the reader permission to use the work if the desired use is covered by copyright. It is the author, as rights holder, who must provide use permission if such use is covered by copyright.

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