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JOURNAL READING
Adhika Manggala
UNISSULA
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CLINICAL REVIEWDiagnosis and treatment
o M!ni"re#s disease
Shakeel R Saeed
Uni$ersit% De&artment o Otolar%ngolog%'ead
and Ne(k S)rger%* Man(hester Ro%al In+rmar%*Man(hester
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In 1861 Prosper Mnire described a syndromecharacterised by deafness, tinnitus, and episodicvertigo. Contrary to the thining at that time, hecorrect!y thought that the condition "as adisorder of the inner ear.
In 1#$8 the principa! under!ying patho!ogy,endo!ymphatic hydrops, "as described by%a!!pie and Cairn
but the precise etio!ogy of Mnire&s disease sti!!remains e!usive
Introd)(tion
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Methods and So)r(e
hand search of themain
ear, nose, and throat'ourna!s in the (ng!ish
!anguage
of a Med!ine searchand current issues of
agiven 'ourna!.
Med!ine search references usingthe te)t "ord *Mnire&s+ and -6
citations using *endo!ymphatichydrops.+ Combining the t"o sets
gave - references, andcombining this set "ith the term*surgery+ yie!ded 8 references. Ie)amined the abstracts of these
-6$ citations. /ecent artic!eson surgery for Mnire&s disease
predominant!y report
resu!ts and various minormodi0cations to estab!ishedsurgica! techniues. 2rtic!es !ooing
at the etio!ogyand pathogenesis of Mnire&s
disease and ne" forms
of treatment
references to te)tboos and a revie"
artic!e
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E&idemiolog%
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Anatomi(al3Mnire&s disease is associated "ith severa!abnorma!ities of the tempora! bone, inc!uding reducedpneumatisation of the mastoid and hypo p!asia of thevestibu!ar aueduct. 4he endo!ymphatic sac is sma!! and !iesin an abnorma! position be!o" the !abyrinth.
Geneti(,
Imm)nologi(al3 4he endo!ymphatic sac is osmotica!!y andimmuno!ogica!!y active. (vidence of immune comp!e)deposition in the endo!ymphatic 5ac in patients "ithMnire&s disease has reinforced the be!ief that the disease isan immune disorder.
Viral3 Ca!eno et a! sho"ed speci0c Ig( to herpes simp!e)virus types I and II, (pstein7arr virus, and cytomega!ovirus inthe serum of patients "ith Mnire&s disease.
Etiolog% and -athogenesis
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Vas()lar34he association bet"een migraine and thesymptoms of Mnire&s disease. Migraine may antedate the
Mnire&s symptoms by many years and occurs in as many asone in three patients.
Meta.oli(3(ndo!ymph is a potassium rich hyperosmo!ar uidthat is positive!y charged "ith respect to peri!ymph. InMnire&s disease distension of the endo!ymphatic space
either a!ters membrane permeabi!ity or causes rupture of/eissner&s membrane, !eading to potassium into)ication of thehair ce!!s and vestibu!ar neuroepithe!ium, "ith resu!tantdeafness and vertigo. /epeated or protracted e)posure of thehair ce!!s to potassium resu!ts in chronic !oss of hair ce!!moti!ity, and the initia! characteristic uctuating deafness isrep!aced by progressive permanent deafness.
-s%(hologi(al39bsessiona! traits, psychosomaticpersona!ities, and neuroses has been noted in patients "ithMnire&s disease.
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9bstruction of endo!ymphatic duct:sac
2!teration in production and absorption of
endo!ymph
;istension of endo!ymphatic sac
Increased in pressure and rupture of innermembranes
s?
-atho&h%siolog%
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Clini(al /eat)res
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5tage I
In the ear!y phase of the disease, the predominant symptom isvertigo. 4his is characteristica!!y rotatory or rocing and isassociated "ith nausea or vomiting. 5igns of vaga! disturbance,such as pa!!or and s"eating, may occur, but !oss ofconsciousness is not a feature. 4he episode is often preceded byan aura of fu!!ness or pressure in the ear or side of the head andusua!!y !asts from @ minutes to severa! hours.
5tage II
4he hearing !oss becomes estab!ished but continues touctuate. 4he deafness is sensori neura! and initia!!y aects the!o"er pitches =0g a?. 4he paro)ysms of vertigo reach theirma)imum severity and then tend to become !ess severe.
5tage III
4he hearing !oss stops uctuating and progressive!y "orsens,both ears tending to be aected so that the prime disabi!ity isdeafness =0g =b? and = c??. 4he episodes of vertigo diminish andthen disappear, a!though the patient may be unsteady,especia!!y in the dar.
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Mased bone conduction thresho!ds in right ear sho"ingprogressive sensorineura! hearing !oss "ith progression of
Mnire&s disease
=a? !o" freuency !oss =b? high freuency !oss =c? severe!oss across "ho!e freuency range
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In$estigation
0he gl%(erol deh%dration testmeasures the audiometric response to an ora! dose ofg!ycero!. Improvement in scores for hearing !o"freuency sounds and discriminating speech isdiagnostic as there is no other condition apart from
endo!ymphatic hydrops in "hich this change isobserved.
Ele(tro(o(hleogra&h%
gives a high!y characteristic "aveform in hydrops,though this test may give negative resu!ts in the ear!y
and !ate stages of the disease =0g $?.
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DD
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Conser$ati$e and S)rgi(al meas)res
that are aimed principa!!y at abo!ishingthe frightening and disab!ing vertigo.
0reatment
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A()te Atta(ks PhenothiaAines B proch!orperaAine
vestibu!ar suppressants 2ntihistamines B CinnariAine
7enAodiaAepines sedative and an)io!yticeects
Conser$ati$e meas)res
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Maintenan(e treatment Dietar% salt restri(tion Di)reti(s B frusemide, ami!oride, and
hydroch!orothiaAide attempt to modify theendo!ymphatic hydrops
Vasodilatorsprophy!a)is on the basis thathydrops is due to ischaemia of the striavascu!aris.
1etahistinereduce the reuency andseverity of attacs at a starting dose of 16mg$):day
;e)amethasone =Corti(osteroids? contro!!ing the symptoms of vertigo
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A.lati$e treatmentIntrat%m&ani( Gentami(in=2minog!ycosides?
de!ivering inner ear treatment by diusion
from the midd!e ear, presumab!y through theround "indo" membrane, thus avoiding damageto the contra!atera! ear.
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S)rgi(al treatment
Destr)(ti$e to
hearingDEabyrinthectomyDCoch!eosaccu!otomyD
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S)mmar% -oints
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