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
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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
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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
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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
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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
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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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