Jurnal Sudden Deafness 1

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J m

AcadAudi ol 4 370-375 ( 1993)

Sudden

Heari ngLoss i n

Mu ti pl e Scl er osi s

Case

Report

Brad

St ach*

G ori a

De gado-Vi l ches*f

Abstract

Thi s

case

l l u s t r a t e s t he

occur r ence of

a

sudden

heari ng s e n s i t i v i t y

l oss

t h a t , i n a l l l i k e l i h o o d ,

was t he

r e s ul t o f

brai nst em

di sorder

resul ti ng f rom

mul ti pl e scl erosi s MS)

Subj ect LD

s a

youngwomnwho

deve opedasuddenhear i ng

l oss

whi l e

hospi t al i zed

f o r exacer bat i on

of

symtom

rel at ed t o mul ti pl e scl erosi s

By

her own

r e p o r t , she hadnormal heari ng i n her e f t

ear at t he

t i me of hospi tal i zati on

Four days

a f t e r admssi on,

shedeve opeda

heari ng

l o s s i n

her l e f t e a r ,

accomanedby roari ng t i n n i t u s and

ul l ness

An

audi ol ogi c

eval uati on r eveal ed

asubstant i al

hi gh- f r equency

s e n s i t i v i t y l oss i n

t he

e f t

ear The

combi nat i on of absent acousti c

refl exes,

depressed

speech

underst andi ng, abnormal

Bekesy audi ometr y, andan abnormal

audi t ory

brai nstem

response

ABR)

was consi stent w th

br ai nstem

s i t e

of

di sorder

Over

the

next 2 weeks, hear i ng

sensi ti vit y recovered t o w thi n normal

l i m t s

Thi s

change

i n

hear i ng

sensi ti vit y coi nci ded w th

t he

recovery

of acoust i c

refl exes,

imrovemnt i n speech under -

standi ng, and

p a r t i a l

r ecovery of the BR

KeyWrds

:

Audi t ory

br ai nstem

response ( ABR) ,

mul ti pl e

scl erosi s

( MS) ,

suddenhear i ng

l oss

ul ti pl e scl erosi s

MS) s a

neurol ogi c

di sease characteri zed by m ti pl e

f ocal

deme i nati ng pl aques

These

pl aques can

occur throughout the

brai n,

but

have

a redi l ect i on

for

the

per i ventr i cul ar

whi te

mtter of

the

brai n

stem Becauseof the nature

of thedi sease, a l l

sensory

and

mtor system,

i ncl udi ngtheaudi tory

syst emcanbeaff ected

t o

a

greater

or l esser

extent

Becausethenumer

and oci ofthe

esi ons

wthi nthe

brai n

stem

vary

consi derabl yamngpati ents,

seque aevary

con-

si derabl y as wel l

Audi tory

di sorders

rel atedt o m ti pl escl e-

*Departmnt

of

Ot orhi nol aryngol ogy and

Commn -

cati ve Sci ences,

Bayl or

Col l ege of

Medi ci ne,

Houst on,

Texas ; t curr entl y Di vi si on

of

Audi ol ogy and Hear i ng

Re-

sear ch,

Georgetown

Uni versi ty

Medi cal

Cent er, Wshing-

t o n, DC

and

curr entl y

Texas

Chi l dren s

Hospi tal ,

Hou-

ston, Texas

Repri nt

requests : Brad A Stach, Di visi on of Audi ol -

ogy and

Heari ng

Resear ch,

GeorgetownUni versi ty Medi -

cal Cent er, 3800

Reser voi r

Road

NW

Washi ngton, DC

20007

rosi s are numrous

and

are best

ref l ected

i n

audi omtri c masures that assess

brai nstem

i ntegr i ty J erger et a l , 1986a)

D mn shed

speech

understanding Osenet a l ,

1975

; Hann ey

et

a l ,

1983),

abnorml

acousti c

refl ex

threshol ds

(Col ett i ,

1975

Hess, 1979), abnorml supra-

threshol d refl ex l atency

and

aml i tude Stach

and

erger, 1984 Jerger et a l , 1986b), abnorml

mski ng evel

di f f erence

Nof fsi nger et

a l , 1972

Hann ey et a l , 1983), and

abnorml

audi tory

brai nstemresponse Robinson

and

Rudge,1977

Stockhardet a l , 1977

Jacobson

and

Jacobson

1990

StachandHudson, 1990) are

not uncom

mn n pati ents whohave mul ti pl e

scl erosi s

Less

wel l

understood, however,

are theef-

fects

of m ti pl escl erosi s on

heari ng

sens i t i vi ty

( e . g . Stachet a1,1990)

There

appears

t o be gen-

eral agreemnt

that

chroni c

heari ngsens i t i vi ty

l ossmy

occur

i n pati ents

wthm ti pl escl ero-

s is

Von

Leden

and

Hort on,

1948 Noffsi nger et

a l , 1972

Cohen

and

Rudge, 1984 Musi eket a l ,

1989)

Theaudi omtri c

patternappears t o beas

vari abl e

as the l esi on site,

and

no si ngl e

confi g-

urati onemrges

as

bei ng

characteri sti c of m -

t i pl e

scl erosi s

I ndeed, the

audi omtri c

confi gu-

rati on

of chroni c heari ng

sensi t i v i ty

l oss

has

beenvari ousl y descri bed as

uni l ateral

andbi -

370



Heari ngLoss i n

Mul ti pl e

Scl erosi s/ St ach andDel gado- Vi l ches

l ateral ;

ml d

and severe

andhigh f requency,

dome

shaped,

and

l owfrequency

There have al so been reports of sudden

heari ng

l oss associ ated

wth mul ti pl e

scl erosi s

(VonLeden andHorton 1948

Ph i l l i p s ,

1952

Hal l berg, 1956 Ci tronet a1,1963

Daugherty

et

al 1983

;

Fischer

et

al

1985

Sheaand

Brack-

mann, 1987

;

Barrett et

al

1988

Frankl in

et al

1989) I n most cases, i t i s uncl ear

as to

whether

the transi ent

hearing

l oss occurs as

a resul t of

scl eroti c l esi ons of

the brai nstem

or

as a esul t

of some di opat hi c suddenhearing oss

of cochl ear

ori gi n

Since

thepresence of mul ti pl e

scl erosi s

i n a

pati ent does not precl udeasudden

hearing

l oss of some

other

eti ol ogy, when

ahearing

l oss

does

occur i n

such

apati ent, i t s cause i s of ten

obscured

Recent

studi es of

acutehearing

oss i nmul -

t i pl e scl erosi s

have used

measures

of cent ral

audi tory

nervous

system

f uncti on, such

as the

audi torybrainstem

response (ABR),

i n

an

effort

t o determne the

s i t e

of

the

l esi on responsi bl e

for the hearing l oss

However,

si nce pat i ent s

wth

MScan

have

abnormal

BRn the

pres-

enceof

normal

peri phera

sensit i v ity, an

abnor-

mal

BRi n the

presence of a

sensorineura

heari ng

l oss

may

be

unre ated

For example,

Frankl i n

et al

1989)

reported

two

cases of

acute

heari ng oss i n

pati ents

wth mul ti pl e

scl erosi s

Al thoughthe BRwas

abnormal

i n both, there

wasno

evidence t o suggest

that

such an

abnor-

mal i ty

di d

not predate thehearing

oss

I n

other

studi es, hearingsensit i vit y

returned

to normal

l evel s,

whil e

the BRremained

abnormal

Fischer

et

a l ,

1985

Shea and

Brackmann,

1987)

I n such cases, the noti on

that the BR

was abnormal bef ore the sensit i vit y

l oss oc-

curredmust

be

entertained

Perhaps

themost

convinci ng evidence of acute hearing

impai r-

ment

emerges f rompati ents who

have

exper i -

encedcoi nci dent al

f l uctuati on of

heari ng

sensi -

t i v i t y

and r et r ocochl ear

si gns Fi scher

et al

1985)

Thepresent

paper

provi des

an

i l l ustrati ve

exampl e

of

a

hearing

sensit i vit y

l oss

that,

i n

a l l

l i kel i hood,

occurredas a esul t of mul ti pl e

scl e-

rosi s

The

pat i ent , a 22-year-old

woman

wth

mul ti pl e scl erosi s,

deve oped

asudden

heari ng

l oss

whil e

hospi t al i zed for

exacerbation

of the

di sease

Resul ts of audi ol ogi c eval uat i ons,

both

at thetime of her

heari ng

l oss

and af ter

her

hearing

returned

to normal , suggestedthat the

heari ng

sensit i vit y l oss

was retr ocochl ear

i n

nature

Thi s

case supports thenoti on

that

sud-

den hearing

l oss can resul t f rombrainstem

di sorder associ ated

wth mul ti pl escl erosi s

C SEREPORT

Descri pti on

of Subj ect

andCl ini cal

Course

Subject LD

a 22-year- old female, was

di ag-

nosed

wth

mul ti pl e

scl erosi s

6

months pri or

t o

thetimeof her

sudden

hearing oss

The

n i t ia l

di agnosi s was

made

onthebasi s of abnormal

MR scansandabnormal

vi sual evoked poten-

t i a l s

At that ti me, her

BR

wasnoted

to

be

normal

OnJ une 2 1990,

fol l ow ng

2 weeks of ver-

ti go and ef t- si ded

numbness,

LD

was

admtted

to the hospi tal

wth

severe vert i go,

nausea,

vomti ng,

and

di pl opi a

She

was

placedi mmedi -

at el y on

i ntravenous

therapy

for

dehydrati on

For the

next

4days, she

underwent

a seri es of

tests

to

complete

adi agnost i c battery or

mul t i -

pl e

scl erosi s

horough

neurophysiol og c

assessment

was carr i ed out on J une 4 as part of the

test

battery

Resul ts f romthe audi tory evoked

po-

tenti al

assessment

showedanormal BRn

the

ri ght

ear, wth an absol ute waveVatency of

5.44

msec

and I - V

i nterwave i nterval of 3

. 73

msec

The

BRn the l e f t ear

was

abnormal ,

wth

an absol ute waveVatency

of

6

. 21

msec

and

I - V

nt erval of4. 64msec

The

major

port i on

of

the

de ay

was nthe

I I I -V nterval

Al though

an

audi ol ogi c

eval uat i onwas

not

carri ed

out

at

that

ti me,

LD

was

noted to have

normal

behaviora

threshol ds to cli ck st i mul i

Duri ng

the4

days

of testi ng, the di pl opi a,

vert i go, and

nausea began to

subside

On

the

morningof J une

6

however,

LDdeveloped a

sudden

hearing

oss i n the e f t ear, accompanied

by si mul taneous

numbness

of

her j awand

ear,

roaring t i nni tus,

anda sense of

f ul l ness

She

al so

stated

that

her

di pl opi a

had

worsened

DataandObservati ons

Initi a

Audi ol ogi c

Eval uati on

naudi ol ogi c

eva uationwascarri edouton

J une 7 at the Methodi st

Hospi ta Audi ol ogy

Servi ce i n

Houston,

Texas

The

eval uati on

con-

si sted of pure-t one, immttance,

and speech

audi ometry Immttance

audiometry i ncl uded

measures of tympanometry,

stati c immttance,

andacousti c refl ex thresholds

Speech

audi om

etry consi sted of

si ngl e-syl l abl e,

phonet i cal l y

bal anced (PB) words,

presented i n

qui et,

and

the

Syntheti c Sentence I denti f i cati on

SSI )

test

J erger et al

1968 , presentedwth

i ps i l ateral

371



J ournal of

the

Amri canAcademof

Audi ology/Volum

4 Number 6

November 1993

cometi tionat

a mssage-to-cometi ti on

rat i o

of 0

dB

I mmttance

audiomtry

was

consi st ent wth

norml

mddl e ear f uncti on

bi l ateral l y,

charac-

t er i zed

by

norml

tymanogram,

norml

stat i c

immttance,

and

norml ri ght uncrossed

and

r i ght

crossed

acoust i c ref l exes

However, acous-

t i c

r ef l exes

wth

sound

t o

the

l e f t

ear l e f t

uncrossedand

eft

cr ossed) were absent at a l l

f requenci es

Resul ts of

pure-tone andspeech audiom

etry

are

shown

i n

Figure 1 Ri ght ear resul t s

were consi st ent

w th norml

heari ng

sensi t i v-

i t y through4000

Hz

and

aml dsensi t i vi ty l oss

at 6000

and

8000 Hz

Speech understanding

wasnorml

Left ear

resul t s showed adramtic,

st eepl y

sl opi ng,

high- frequency sensori neural

heari ng

oss I n

addi t i on,

speechunderstanding

was s i gni f i cant l y

depressedf o r both

PBwords

and

theSSI est

When

scor es on

both

masures

were comared t o

expected

performnce f o r

cochl ear

hearing

oss of

si m l ar degree Yel l i n et

al

1989),

they

f e l l below

the

l ower

l i m t s of

Right

Ear

Left Ear

Pure Tone

Audiomtry

250 1000

4000

L S

0

U

e

a

0

dB

0

20

40

60

80

100

250 1000

4000

l

Speech

Audiomtry

0

0

0 40 80

0 40 S

HL n

dB HL n dB

Audiomtry

~ Refl ex Thresholds

t]

Unmsked

AC

ncrossed

ashed

AC

Mrossed

Soeerh~AAndiom

trv

re

BWrds

i n

qui et)

SSI

Sentences 0 dB

MCR)

Figure 1 Ai r - conduct i on AQ

pure-tone audiometry,

acousti c refl ex t hr eshol ds, and

speech audiometri c

re-

sul ts

i n a

22- year- ol d feml e

w th

adi agnosi s of

mul t i pl e

scl erosi s

Testi ng

was comletedon

J une 7 1990, 1

day

after she

r eport ed

a

sudden

heari ng

l oss in her l e f t ear

Phonet i cal l y

balanced PB) wordtesti ng

was

carr i ed

out

i n qui et,

and

the

Synt heti c

Sentence

I denti f i cati on

SSI )

test

was

carr i ed out at a

message- t o- compet i t i on rat i o

(MCR) of 0

dB

100

80

60

40

20

0

Ri ght

Ear

Left Ear

I psi

Contra

I ps i

Contra

Fi gure 2 Auditory

brai nstemresponses, w th como-

nent

peaks

I I I ,

and

V,

f romtheri ght

and eft

ears of

a

22-year-ol dfeml e w th

a

diagnosi s

of ml ti pl escl erosi s

Testi ng

was

comletedon

J une

8, 1990, 2

days

after

she

reporteda

sudden

heari ng

l oss

i n her

eft

ear

Sti ml i

were

al ternating

c l i cks

presented

at ani ntensi tyof 90dB

nHL

and arate of 11. 1/ sec

norml

That

s theexpectedscores f oracochl ear

heari ng l oss

wth

a

pure-tone

average of

thi s

degree are better

than those att ai ned by thi s

subj ect

Because

we

were unsure

about

whether

or

not we woul dbe abl e

t o record

an

ABR n

anear

wth such

asubstanti al

high-frequencyheari ng

l oss, we reverted

t o a

t r adi t i onal

si te-of- l esi on

test andcarri edout

di agnost i c

Bekesy

audiom

etry Resul tswereconsi st ent

wthretrocochl ear

di sorder

LD s

thresholds

by Bekesy audiom

etry t o a pul sed tone were

siml ar t o conven-

t i onal behavi oral thresholds

However,

s ign i f i -

cant audi tory

adaptation

was present i n r e-

sponse

t o acontinuous

t one,

r esul t i ng i naType

I I I

Bekesyaudi ogram

Resul tsof

ABR

est i ng are

shown n Figure

2 ABRs

were

recorded

i n

response

t o cli cks of

al ternat i ng

pol ar i ty presented

at

90

dB

nHLat

a

rate

of

11. 1/ sec Theri ght ear ABR

was nor-

ml

Wve atency

was

5

. 4

mec,

and

the

I - V

i nterwave

i nterval

was 3

. 9 mec

The

l ef t

ear

ABR

was

st r i ki ngl y

abnorml , characteri zed

by

the

presence of

waves and I onl y I n

addi t i on,

wave wasnoted

t o be of excessi ve

aml i tude

n

comari son t o the

eft

ear

Because

of

thepara-

dox of

being

abl e t o

record a wave

I

i n the

presence

of

sucha

substanti al

hearing

sensi t i v-

i t y l oss, thewave was

trackedt o i t s threshol d

Resul ts are

shown

i n

Figure 3 Wve

I

was

fol l owed

down

t o 50dB

nHL

Because awave I

372



Heari ng

Loss

i n

Mul ti pl e

Scl erosi s/ St ach andDel gado- Vi l ches

80dBnHL

60

dB

nHL

Right Ear

250

1000

4000

Left Ear

l Pure

ToneAudiometry

dB

0

20

40

60

80

100

250 1000 4000

SpeechAudiomtry

50dBnHL

Fi gure

3

Audi tory

brai nstemresponse

from

thele f t

ear of a

22-year-old

female

wth

a

diagnosis

of ml ti pl e

sclerosi s

Test i ng

wascoml eted

onJ une

8,

1990,

2 days

af ter she

reported

a

sudden heari ng oss

i n

her

le f t ear

Sti ml i were

al ternati ng cl i cks

presented

at

three

i ntensi ti es at a

rate

of 11. 1/sec

V

0 40

80

0

40 80

HL n dB

HL n

dB

could

be measured

n the

presence

of thi s

degree

of sensi t i vi ty

l oss,

we

concl udedthat the

cause

of the heari ng l oss

occurred

at

a evel

beyond

that of the cochl ea and

eighth nerve

Thus, the overal l

pattern of resul ts from

thi s i n i t i a l eva uation

was

consi stent

wth

retro-

cochl ear di sorder

Evi dence incl uded the

ab-

sence

of

acoust i c

refl exes,

depressed speech

understanding,

the

presence

of audi tory

adap-

tat i on,

andthepreservation

of

awave

I i n

the

presence

of a severe

high-frequency heari ng

l oss

Audi ol ogi c Re-eval uati on

LD

was discharged

fromthe hospi t al sev-

eral days ater OnJ une11,

she

noti ced that her

hearingbegan to return

and

that theroaring

t i nni tus

decreased

By

J une

16, thet i nni tus

and

vert i go had subsi ded Her

on y

resi dual com

pl ai nt at t hi s

time

was

that of

an

echo i n her

l e f t ear

On une

29, LD

was re-eval uat ed

Audi ol ogi c

resul ts

showed

that heari ng

sensit i vi ty , acous-

t ic

ref l ex t hreshol ds,

and

speech

understanding

had a l l

returned

to normal i n

the l e f t ear

I mmttance audi ometrywas

consi stent

wth

normal

mddl eear functi onbi l ateral l y,

charac-

t eri zed

bynormal

tymanogram,

normal stati c

immttance,

and normal

crossedand

uncrossed

acoust i c

ref l exes Acousti c

ref l exes

wth

sound

t o the

l e f t

ear

had

returned

to normal l evel s

Resul ts

of

pure-tone and

speech

audi om

etry areshown n

Figure

4

Lef t ear resul ts had

imroved

dramatica l y Both ri ght and e f t

ear

Audi omtry

Ref l ex

7hreshol ds

Rceech Audi omtry

nmasked

AC

ncrossed

BWrds i n

qui et)

]

Masked

AC

®rossed N

SI

Sentences

(0 dB

MCR)

Fi gure

4 A r- conducti on

AC)

pure-tone

audiometry,

acousti c

refl ex

threshol ds,

andspeech audi ometri c

re-

sul ts i na

22-year-oldfemale

wtha

diagnosis

of ml ti pl e

scl erosi s

Test i ngwas

coml eted

on J une

29,

1990,

23

days after she

reported

a

sudden heari ng oss

i n

her

eft

ear

Phonetica l ybal anced PB)

wordtestingwascarried

out i n

qui et ,

andthe

Syntheti c Sentence I denti f i cati on

SSI )

test was carriedout

at amessage-to-cometi ti on

rati o MCR) of 0 dB

pure-tone

thresholds

wereconsi stent

wth nor-

mal heari ng sensi t i vi ty

through

6000

Hz I n

addi ti on,

speech

understanding

i n

the e f t

ear

was

nowat normal

mximumevel s

ABR esul t s are

shown

i n Figure5

ABRs

were

recorded

i n

response

to cl i cks of

al ternat-

i ngpol ari ty presented

at 80dB

nHL

at a

rate of

11 . 1/sec

The

ri ght

ear

ABR

remainednormal ,

wth awave atency

of 5 4 mec

and

a

I -V

i nterwave

nterval

of

3

8

mec

The

e f t

ear

ABR

remained abnormal,

al though wave eap-

peared

at a l atency of 7

8

mec Resul ts of the

three

ABReva uationsof

the l e f t

ear

are

shown

i n

Figure 6 The

i n i t i a l

ABR

test, car ri ed out

pri or

to the

onset

of the

sudden

hearing

l oss,

wasthe

mst wel l formed The

secondABR

est,

carri ed

out i n the

presence

of

the

sudden

l oss,

showed

an

absenceof the l ater waves

The

i nal

measurement,

carri ed

out af ter

heari ng

sensi -

t i v i ty

had

returned,

showed

a re-emergence

of

wave

100

80

60

40

20

0



J ournal of

the

Amri canAcademof

Audiology/Volum

4

Number 6

November

1993

Date

Ri ght

Ear

Left Ear

Fi gure

5

Auditory brai nstem

responses,

wth como-

nent peaks I I I ,

and

V, from

the

ri ght

and

eft

earsof

a

22-year-ol d

feml e

wt hadiagnosisof ml ti pl e

scl eros is

Testing

was

comleted

on

J une

29,

1990,

23 days

af ter

she

reporteda

sudden

heari ng oss i n her eft

ear Sti ml i

wereal ternatingc l i cks

presentedat

an

intensity

of 80dB

nHL

and a

rate

of

11

. 1/ sec

COMMENT

L

D s

hearing

sens i t i vi ty l oss appeared

t o

be duet o

retrocochl ear di sorder

Audio-

l ogi c

evidence included

absent acoust i c

ref l exes

wth

soundpresentedt o the

l e f t ear, depressed

speech

understanding, and

abnorml audi tory

adaptation Fol l owng

resol ut i on

of

the

sensi -

t i v i t y

l oss, acoust i c ref l exes

and speech under-

standing returned

t o

norml l evel s

Audi toryevoked pot ent i al

resul ts were i n

agreemnt

wth behavioral

masures, i n that

ABR

changes

corresponded

wth heari ngsensi -

t i v i t y

changes

TheABR

was reported t o be

norml

at the ti mof di agnosi s,

6mnths

pri or

t o the

hearing

oss i nci dent

Athough

the

ABR

that was

recorded 2

days

bef ore

the onset of

heari ng l oss

was considered

t o

be abnorml ,

i t

was

f ai r l y

wel l formd i n comarison

t o subse-

quent

recordings

The s l i ght

l atency

abnorml -

i t y coul dhave

been

re f l ect i ng the

beginningof

thedisease

proeess that was ater

t o resul t i n a

substanti al

sens i t i vi ty

oss Regardl ess,

the

ABR

obtainedat theti m

of the

heari ng

oss

hadonl y

waves I

and

I I as

masurab e comonents,

re-

f l ect i ng asubstanti al

changei n eighthnerve

or

brainstemntegr i ty

That the

wave was

present

at i nt ens i ty l evel s as

l owas 50

dB

i n

anear

wth

a

PTA average

ofthreshol ds

at 1 2 and4

kHz)

of

85

dB

i ndi cated

that

the oss

was probab y

not

I psi

Contra

I psi

Contra

6/ 4

6 / 8

6/ 29

Fi gure

6

Audi tory brainstemresponse

from

the l e f t

ear of a

22-year-ol d feml ewth a

diagnosis

of ml ti pl e

scl erosi s

Testingwascoml etedon three

dates

1)

J une

4,

1990,

2

daysbefore shereporteda

sudden

heari ng

oss

i n

her

eft

ear

2)

J une

8,

1990,

2 days

aft er she

reported

thel oss

;

and

3)

J une

29, 1990, 23 daysaf ter

she

reported

the

l oss

cochl ear

Fol l owng

resol ut i on of the

sens i t i vi ty

l oss,

wave

eturned,

al though

i t s

l atency

remined abnorml

Thus, the

ABR

l uctuat ed

along wth

the heari ng

sensi t i vi ty l oss

O

course,

the

fact that a pati ent has

ml -

t i pl e

scl erosi s

that i s

at an exacerbated

stage

does not preclude that

person fromhaving

a

sudden

i di opat hi c

heari ng

l oss

of cochl ear

ori -

gin I f the l oss were of

cochl ear or i gi n,

however,

the

pattern of audi ol ogi c

resul ts

would

mst

l i kel y have been one of

better speech under-

standi ng, a

masurab e

acoust i c ref l ex at 500

Hz,

noaudi toryadaptati on, and

anabsent

ABR

Onepossi bl e mchani smfo r

sudden hear-

ing

l oss i n

a

patient wth ml ti pl e

scl erosi s

i s

rel ated

t o the course of the di sease

process

Probab y

as a resul t of al t ered

immuneregul a-

t i on,

l ymhocytesescapefromthe

b oodstream

penetrate

brain

ti ssues,

and

destroy

mel in

Duringthe

destructi on

pr ocess,

transi ent

edem

develops n

thet i ssue aroundthe

demelinating

l esi ons This

can affect

transmssion

through

axons,

whi ch

resul t s i n exacerbation of

sym-

tom Once

the i nfl ammtory acti on

subsi des,

swell ing i s

reduced, and

remssion

begins

374



Heari ngLoss i n

Mul ti pl e

Scl erosi s/Stach

andDel gado- Vi l ches

Ri vera,

1990)

Thus,

f the demyel i nati on

pro-

cess

occurs

i n

theaudi tory brain stem t

coul d

resul t i n

an

audi tory

di sorder

that

hasanacute

onset

duri ngthe

i nf l ammati onpr ocess,

f ol l owed

by a reducti on i n

symptoms

as

the

swel l i ng

subsides

LD sheari ng

oss

r ef l ected

thedi sease

course

that

i s

t ypi cal

of mul ti pl e

scl erosi s

She

entered thehospi t al as a resul t of a rel apse of

thedi sease Duri ngtheheight of theexacerba-

t i on

of her symptoms, she

deve oped

a

hearing

sensit i vi ty l ossthat

appeared

o be

r et r ocochl ear

i nnature Fol l owngdi schar ge, andas herother

symptoms

beganto remt her

heari ngreturned

t o normal

A though sudden

hearing

l oss i s

sel dom

reported i n pati ents wth mul t i pl e scl erosi s,

thi s

case

demonstrates

that the

di sease,

prob-

abl y as aresul t of demyel inationat

some

pr eci se

point n theaudi torynervous systemcancause

a

hearing

sensit i vi ty l oss That the hearing

returned

duri ng

remssi on further

i mpl i cates

mul ti pl e

scl erosi s

as the

cause

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Jurnal Sudden Deafness 1