Meniere’sDisease:APatient-CenteredGuidetoDecisionMaking

By:JeffreyD.Sharon,MDAndLauraKirk,PA

WearegladyouhavesoughtcarewithusatUCSF!WehavenoticedthatpatientvisitsarenotlongenoughtothoroughlycoveralloftheimportantinformationaboutacomplicatedtopiclikeMeniere’sdisease.Therefore,wewrotethispatientguide,whichwillhopefullyhelptofillintheblanks,andgiveyouadetailedinformationonourapproachtomanagementofMeniere’sdisease.Asyouhavealreadyrealized,therearenoquickandeasyanswerswithMeniere’sdisease.Generally,ourtreatmentapproachis“trialanderror”,wherewewillinitiateatreatment,andseewhetherornotitworksforyoubeforetryingothertreatmentoptions.Therefore,it’sveryimportantthatwepartnertogethertofigureoutagoodtreatmentplan.WearegenerallylessdirectivewithourpatientswithMeniere’sdiseasetowardaparticulartreatmentplan.Instead,weviewourjobaseducatingpatientsaboutthevarioustreatmentoptionsavailable.Patientswillreturnforafollowupvisit,andreportbacktheirexperience.Forexample,“Thelowsaltdietworkedforawhile,butoverthelastmonthI’vehadacoupleofbadattacks.Canwetryasteroidinjection?”Givenourintentionofpartnershipwithyou,it’simportantforyoutolearnaboutMeniere’sdisease,aboutthedifferenttreatmentoptionsavailable,andabouttherisksandbenefitsofeach.AlotoftheinformationprovidedhereisfromanarticleI,Dr.Sharon,wroteafewyearsago.1

WhatisMeniere’sdisease?Meniere’sdiseaseisaconditionthatcauseshearingloss,dizziness/vertigo,earpressure/fullness,andtinnitus(ringingintheears).Weknowthatthesesymptomsoccurtogether,oftentimeswiththisstory:someoneexperiencesasuddenattackofvertigo(asensationofspinning),accompaniedbyalossofhearing,anincreasedvolumeoftinnitus,andapressuresensationinanear.Almostalways,patients

withMeniere’sdiseasecanpointtooneear,andclearlyexperienceeithertherightearortheleftearastheproblem.WedonotfullyunderstandMeniere’sdisease.Infact,wedon’tknowwhatcausesit.Thismakestreatmentmoredifficult,becausewithourincompleteunderstanding,wehavetomakeeducatedguesses.It’simportanttoknowthatcurrently,thereisno“cure”forMeniere’sdisease.Instead,thebestthatwecanofferaretreatments.Thetreatmentsworkwellforamajorityofpatients,butitalwaystakestimetofigureoutwhichtreatmentswillworkforyou.That’sdisappointing,becausemostpatientswithMeniere’sdiseasearereallysuffering,andtheywantthediseasegoneasquicklyaspossible.It’sourhopethatinthefuture,wewillknowwhatcausesMeniere’sdiseaseandhowtocureit!Fornow,wehavetorelyontheavailabletreatments.Luckily,throughatrialanderrorapproach,wearealmostalwaysabletofindagoodtreatmentthatworksforeachpatient.Butweneedtoprepareourselvesfortherealitythatthisisalong-term(chronic)disease,anditusuallytakessometimetofigureoutatreatmentregimenthatworksforyou.Ofhistoricalinterest,Meniere’sdiseaseisnamedafteraFrenchneurologist-ProsperMeniere(whichcanbewrittenMénièreorMenière,thelastonelikelybeingthemosthistoricallyaccurate).Hepublishedapaperin1861showingthattheinnerearcouldcausehearinglossandvertigo.Untilthatpointintime,itwasthoughtthatonlythebrain(cerebellum)causedvertigo.WhatcausesMeniere’sdisease?Wedon’tknow.Theinnerear,locateddeepintheskull,isanintricateandsmallspace,filledwithfluid,membranes,andnerves,andincludessensingorgansofbothhearingandbalance.Varioustheorieshavebeenadvocatedovertheyears,arguingthatthecauseisgenetic,autoimmune(whenthebody’simmunesystemattacksitself,likeinrheumatoidarthritisorlupus),vascular(duetoabloodsupplyproblemtotheear),infectious(duetoavirusthatwehaven’tyetidentified),relatedtootoconia(littlecrystalsintheinnerearthathelp

theearsensegravitybybeingaminiatureweight,migraine,orinnerearfluidcirculationproblems.Whatwedoknowisthatacertainpartoftheinnerear,calledtheendolymphaticspace,isalmostalwaysabnormalinpatientswithMeniere’sdisease.Thisspaceisinthecentralpartoftheinnerear,andissurroundedbythemuchlargerperilymphaticspace.InMeniere’sdisease,theendolymphaticspaceisballoonedoutward,andisthereforemuchlargerthanitshouldbe.Thisiscalledendolymphatichydrops.Inhistologicstudies(wheretheinnerearisexaminedunderamicroscope),allpatientswithMeniere’sdiseasesymptomshaveendolymphatichydrops.Thismeansthatitisaveryconsistentfinding,andiscertainlyrelatedtoMeniere’sdisease.Wedon’tknowiftheenlargedspacecausesMeniere’sdisease,iscausedbyMeniere’sdisease,orisotherwisetangentiallyrelatedtoMeniere’sdisease.Also,therearesomepatientswithendolymphatichydrops,whodon’thavethesymptomsofMeniere’sdisease.Thismeansthatthereisnota1:1correlationbetweenthetwoconditions.Itmaybethatendolymphatichydropscanberelatedtoothercauses.OrendolymphatichydropsmaynotdevelopMeniere’sdiseaseunlessanadditionalproblemispresent.Wejustdon’tknow.Ofinterest,moreandmoreresearchisbeingdonewithspecialMRI(MagneticResonanceImaging)sequences,whichcanseetheendolymphatichydropsinalivingperson.Thisisexciting,becausewedon’tbiopsytheinnerear(whichissosmallanddelicatethatabiopsywoulddestroyit).Therefore,alloftheinformationwehaveonthemicroscopicfindingsinMeniere’sdiseaseisbasedonlongtermcadaverstudies,wheretherewerepost-mortemexaminationsoftemporalbones(thepartoftheskullthatcontainstheinnerear).So,theideathatwearenowabletoseesomeofthesefindingsinalivingperson,withouthurtingthem,isveryexciting!HowdoIknowifIhaveMeniere’sdisease?Atthecurrenttime,Meniere’sdiseaseisaclinicaldiagnosis.Thatmeansthatthereisn’talaboratorytest,orapathologyresult,oranimagingfindingthatcantellyouthatyouhaveMeniere’sdisease.Instead,thediagnosisismadebyaclinician,aftercarefulconsideration

ofyoursymptoms,andreviewingyourhearingtest(audiogram).Thereareguidelinesformakingthisdiagnosis.Generally,ifyoumeetthecriteriaintheguidelines(reproducedbelow),andifyoudon’thaveanothercausefordizzinessasdeterminedbyyourclinician,thenyouaredeterminedtohaveMeniere’sdisease.Oneimportantpoint:weroutinelyseepatientswhohavebeendiagnosedelsewherewithMeniere’sdisease,butwhodonothavehearingloss.WhiletheremayberarevariantsofMeniere’sdiseasethatonlyaffectthevestibularsystem,thisisveryuncommon.Instead,mostofthesepatients,withnormalhearing,haveanothercausefortheirdizziness.Thatcauseismostcommonlyvestibularmigraine,whichisthemostcommoncauseofdizzinessintheUnitedStates.

2015consensuscriteriaforthediagnosisofMeniere’sdisease2Diagnosis CriteriaDefiniteMeniere’sDisease • Twoormorespontaneous

episodesofvertigo,eachlasting20minto12h

• Audiometricallydocumentedlow-tomidfrequencysensorineuralhearinglossin1ear,definingtheaffectedearonatleast1occasionbefore,during,orafter1oftheepisodesofvertigo

• Fluctuatingauralsymptoms(hearing,tinnitus,orfullness)intheaffectedear

• Notbetteraccountedforbyanothervestibulardiagnosis

ProbableMeniere’sDisease • Twoormoreepisodesofvertigoordizziness,eachlasting20minto24hFluctuatingauralsymptoms(hearing,tinnitus,orfullness)intheaffectedear

• Notbetteraccountedforbyanothervestibulardiagnosis

DoIneedanyothertestingdone?MedicalcentersacrosstheUnitedStatesmayeachhaveaverydifferentworkupforMeniere’sdisease.Withoutanationalstandard,theworkupcanfeelveryconfusing.Manyoftheteststhatarecommonlydone,likeelectrocochleography,vestibulartesting,glyceroltests,VEMPtests,andbloodtestscansometimesbeabnormal.However,noneofthesearereallygoodtests,meaningthattheyaren’talwaysabnormalinMeniere’sdisease,whilealwaysnormalwithotherconditions.Therefore,wedon’troutinelyorderanyofthesetests.Thebottomlineisthatwedon’t’findtheseteststobeparticularlyhelpful,andtheresultsdon’tchangeourmanagementofyourcondition.

Therefore,it’simportanttoknowthatourphilosophyinworkingupMeniere’sdiseaseistoruleoutotherthingsthatcancausesimilarsymptoms,ratherthanprovingthatMeniere’sdiseaseistheculprit.Thisisdifferentthanmanyotherconditions,whereweusetestingtoprovethediagnosis.Herewearetryingtoprovethatyoudon’thaveotherconditions.Thestandardofcareforanyonewithaunilateral(one-sided)sensorineuralhearinglossistohaveanMRIscan(withcontrast).SinceMeniere’sdiseasealmostalwayscausesone-sidedhearingloss,patientswithMeniere’sdiseaseshouldalwayshaveanMRIdoneatsomepoint.Thisisimportanttomakesurethatyoudon’thaveabraintumorofyoursymptoms,suchasavestibularschwannomaoranendolymphaticsactumor.However,pleasebeaware-almostalwaystheMRIisNORMALinpatientswithMeniere’sdisease.Somepatientsgetfrustratedwiththenormalimagingresult,becausetheyhopethattheMRIwillprovidetheirdiagnosis,butthatwasnotthegoal(orthecapability)ofthestudy.Youdoneedanaudiogram(hearingtest)todiagnosisMeniere’sdisease.Thisisthebesttestforassessingseverityofhearingloss.Theaudiogramcanbeusedtotrackanddocumentanychangesinhearing.It’salsohelpfulforfiguringoutifyoucouldbehelpedbyanyhearingrehabilitationtechnologies,likeahearingaidoracochlearimplant.SometimeswewillorderbloodworkforpatientswithMeniere’sdisease.Thismaybedonetocheckforautoimmunediseasesorforsyphilis.Autoimmunediseases(likerheumatoidarthritis)arediseaseswherethebodyattacksitself.Weknowthatsometimestheinnerearcanbeatargetfortheseattacks.Thiscanoccurexclusivelyintheinnerear,ormayimpactmorethebodyaspartofanotherautoimmunecondition(suchasrheumatoidarthritis,lupus,Sjogren’sdisease,scleroderma,type1diabetes,Cogan’ssyndrome,Hashimoto’sthyroiditis,Gravesdisease,andothers).Typically,autoimmunediseasewillcausefluctuatinghearingloss(hearinglossthatgetsbetterandgetsworse),inbothears(unlikeMeniere’sdisease),andpatientsimprovewithoralsteroids.Ifyoursymptomssoundsimilartothat,wewillordersometeststoevaluateforthepresenceofautoimmunediseases.TheselabtestsmayincludeESR,CRP,ANA,ANCA,SSA/B,andRF.

Ifyouhaveanyriskfactorsforsyphilis(asexuallytransmitteddiseasewithriskfactorsofunprotectedsexwithmultiplepartners),thenit’sagoodideatodoabloodtesttocheckforsyphilis.Ifthistesthasneverbeendone,thenpleaseletusknow,andwecanorderitforyou.Syphilisisatreatabledisease,withproperantibiotics.Finally,inendemicareas,sometimeswewillcheckforLymedisease.LymediseaseisnotendemicinCalifornia.Itbeginswithatickbite,thatthencausesaredrashthatspreadsoutward,lookinglikeanexpandingcircle(ortarget).Initslaterstages,Lymediseasecancausejointpains,fatigue,heartproblems,facialpalsy,andnervepain.Ifthisissuspectedatall,thenwewillsendyoutoaninfectiousdiseasedoctorforfurtherworkup.ThisisbecausetherearealotofnuancesintestingforLymedisease,anditiscommontohave“falsepositive”results(testsuggestsadiagnosisofLymedisease,whenthatisnotthecase).

WhatotherdiseasescanbeconfusedwithMeniere’sdisease,andhowdoyoutellthemapart?Disease TypicalSymptoms Howdoyoutellit

apartfromMeniere’sdisease?

VestibularMigraine Dizziness,whichcanlastforsecondstoyears.Migrainecanalsocauselight/soundsensitivity,visualdisturbances,headaches,headpressure,earpressure,tinnitus,andfacialpressure.Onebigclueisapersonalorfamilyhistoryofmigraine,butthisisnotalwaysthecase.

VestibularmigraineisVERYcommon-MUCHmorecommonthanMeniere’s.Itcancausesimilarsymptoms,liketinnitus,earfullness,dizziness,andsoundsensitivity.However,hearingisusuallyunaffected.AndthepatientwillusuallyhaveahistoryofmigrainesAND/ORmigrainesymptoms(light/soundsensitivity,orheadaches)closelytimedwithattacksofdizziness.

AutoimmuneHearingLoss

Fluctuating(meaningfrequentlychangingforbetterorworse)sensorineural(innerear)hearingloss,usuallyinbothears,thatgetsbetterwithoralsteroids(likeprednisone)

Autoimmunehearinglossusuallyinvolvesbothears.Manypatientshavealreadybeendiagnosedwithanotherautoimmunedisease.Finally,thehearingusuallyimproveswithoralsteroids,likeprednisone.

BPPV(BenignParoxysmalPositionalVertigo)

Intensedizzinesslastingsecondsto1minute,thatistriggeredbypositionalchanges(usuallyrollingoverinbed)

BPPVdoesn’tcausehearingloss.Thistypeofdizzinessiscausedbyspecificmovements(lookinguporrollingoverinbed).

TemporalBoneTumors

Usuallyjustcauseone-sidedhearingloss,and/ortinnitus,butcancausedizziness

MRIcaneffectivelyruleoutthepossibilityofatemporalbonetumor.

Otosyphilis OtosyphiliscancausesimilarsymptomstoMeniere’sdisease,includinghearinglossanddizziness.

It’sasexuallytransmitteddisease,soit’sveryunlikelytooccurwithoutanybehaviorthatputssomeoneatriskofanSTD.Inthosepatients,abloodtestcaneffectivelyruleoutthepossibilityofotosyphilis.

LymeDisease Commonlycausesaspreadingtarget-shapedrash,followedyearslaterbyjointpains,heartproblems,andnervedamage.Thisnervedamagecanpresentinvariableways,includinghearingandbalancesymptoms.

Sinceit’stransmittedbyatickbite,it’sunlikelytohappenwithoutthathistory.It’salsounlikelytooccurinplaceswhereLymeisn’tendemic.Thiscanbeworkedupwithabloodtest.

MultipleSclerosis Causesnerveproblems,likenumbness,weakness,blurryvision/blindness,andtroublewithcoordination/walking.

Multiplesclerosisusuallyaffectsthewholebody,notjustthehearingandbalancemechanisms.Ifitissuspected,thenfurtherworkupisdonebyaneurologist.Also,certainfindingsonMRIofthebrain,whichisdoneaspartoftheworkupofMeneire’s,willusuallyshowabnormalitieswithmultiplesclerosis(whitematterplaques).

PerilymphaticFistula Aperilymphaticfistula(PLF)referstoadrainageofinnerearfluidintothemiddleear.Thiscanoccur(rarely)afterheadtrauma,suchasaskullfracture,orsurgery(typicallystapessurgery).Some

Perilymphfistulacanbesuspectedifthereisanincitingevent,suchasaskullfractureorastapessurgery,whichcauseshearinglossandvertigo.Thereisnotestthatcan

physiciansbelievethatthemembranesthatseparatetheinnerandmiddleear(theroundandovalwindows)canspontaneouslyrupture,causingsymptomsofvertigo,hearingloss,andearpressure.However,thisisverycontroversial(meaningmanyphysiciansdon’tbelievethatthiscanoccur).

diagnosisthiscondition.Generally,thevertigoshouldoccurwithmiddleearpressurechanges,suchasoccurwithcoughing,sneezing,orstraining.Ifsuspicionexists,thenwecanperformafistulatest,whereweputairpressureagainsttheeardrum,andlookforeyemovementthatindicatethatthemiddleearpressurechangesarebeingtransmittedtotheinnerear.

SuperiorSemicircularCanalDehiscence(SCD)

SCDisararediseasethatresultsfromabsentboneoverpartoftheinnerear.Thiscausesseveralsymptoms,includingautophony(hearingyourownvoiceabnormallyloudly),hearingotherbodilysoundsthatyoushouldn’thear(likehearingyoureyeballsmove),hearingyourheartbeatinyourears,anddizzinessthatisprovokedbyloudsounds,orcoughing/sneezing/straining.

SCDcausesaslightlydifferentsetofsymptomsthanMeniere’sdisease,sousuallytheycanbedistinguished.FurtherworkupofSCDinvolvesaVEMPtest(donebyabalanceaudiologist),andaCTscanofthetemporalbones.

VestibularParoxysmia

Thisisararedisease,thatcancausebriefintensefeelingsofvertigo,thatcanbetriggeredbyheadmovementsorexercise.

VestibularParoxysmiaisveryrare.Inaddition,itdoesnotusuallycausehearingloss,andthedizzinesslastsafewseconds,notminutestohours,whichistypicalforMeniere’sdisease.

VestibularNeuritis Thisreferstoaninflammationofthebalancenerve,whichcausesaveryintensefeelingofspinningthatusuallylastsatleasta

Vestibularneuritisistypicallyaone-timeevent,whereasMeniere’sdiseasecausesrecurrentsymptoms

day,ifnotlonger.Thereisnohearingloss.Mostpatientswilljusthavetheoneepisodeofvertigo,butwillthenhavesomedizzinesswithrapidheadmotionsthatoccuruntilthebraincompensatesforthedamagethatwasdone.

overmanyyears.Also,thereisnohearinglosswithvestibularneuritis.Finally,aphysicalexamusuallyshowsatypicalfinding(positiveheadimpulsetest)withvestibularneuritis.

Labyrinthitis Labyrinthitisreferstoaninfectionoftheinnerear.Thisstartsabruptlywithdizzinessthatlastsseveraldays,inadditiontoprofoundhearingloss.Thedizzinessthengetsbetterovertimeasthebraincompensatesforthehearingloss.

Labyrinthitisisaone-timeeventwheretypicallyallhearingandbalancefunctionislostatonce,whereasMeniere’sdiseasecausesrecurrentsymptomsforyears.

PatulousEustachianTube(PET)

PETistherareconditionofhavingaEustachiantubethatisabnormallyopen(usuallyit’sclosedallthetime,andjustopenswhenyouswallow).Thiscausesafeelingofearfullness,butyoualsohearyourownvoiceandbreathingabnormally,likeyouareinabarrelwheresoundsechoorreverberatearoundinyourhead.

PETdoesnotcausehearinglossorvertigo,justearpressure.

WhatshouldIbedoingtokeeptrackofsymptoms? IrecommendthatallpatientswithMeniere’sdiseasekeeptrackoftheirsymptomswithadiary.Sincealltreatmentsaretrialanderror,it’simportanttohaveyourcarefulobservationsasfeedbacktotellwhetherornotatreatmentishelpful.Inotherwords,sincewearen’tsureaheadoftimeifaparticulartreatmentisgoingtobebeneficial,weneedtohavearecordthatsummarizesyoursymptomsbothbeforeandaftertreatment. Thedetailsofthesymptomdiaryarereallyuptoyou.Thesimplestwaytokeeptrackofsymptomsisjusttosumupallyoursymptoms(earpressure,vertigo,dizziness,tinnitus,hearingloss)each

dayintoanumberfrom0to10,with0indicating“nosymptoms”,and10indicating“theworsepossiblesymptoms”.Then,recordyour“number”eachday.WerecommenddoingthisinGoogleSheets.It’sfree,secure,andeasy.Belowisascreenshotofasamplesymptomdiarythattook2minutestosetup.

Allyouneedtodoisopenanewspreadsheet.Thencreate3columns:onecolumnfordate,oneforthesymptomscore,andonefornotes.Ifyouinputthecurrentdateintoacell,youcanjustdragthatonecelldown,andGooglewillfillinnewdatesforyou,sequentiallyfromyourstartingdate.Ineffect,thismeansthateachrowwillrepresentoneday.Onceyouhavesomedata,youcanhighlightthatdata,andthenclick“insert”and“chart”,andyouwillbeabletoseeagraphdetailingyoursymptoms.Fornotes,wesuggestthatyoumarkdownwhenyoustartandstopanewtreatment,oranythingelsethatseemsrelevant. Ifyouprefertorecordmoredata,thatistotallyfine.CreateacategoryforeachsymptomthatyougetfromMeniere’sdisease.Inaddition,youcancreate“compositescore”,whichisacalculatedvaluethatistheaverageofalltheindividualsymptomscores.Youcanweighteachsymptomdifferentlyifyoufeelthatsomeinfluencetheimpactofyourdiseaseonthequalityofyourlifemorethanothers.

Ifyouaren’tcomputersavvy-don’tworry.Justusethesimplestdiarythatworksforyou,whichcanevenbejustmarkinganumberfrom1-10eachdaytorepresentyoursymptomsonacalendar.Whyaremytreatmentoptions? TherearequiteafewtreatmentoptionsinMeniere’sdisease.Infact,thenumberofdifferentoptionscanbeoverwhelming!Thiscanmakeitdifficulttofigureoutwhichtreatmentisrightforyou,whichtreatmentsyoushouldtry,andinwhatorder.Beforewestartgettingintodetails,it’simportanttorealizethesuccessofeachtreatmentismeasuredbythereductioninfrequencyofvertigoattacks.Thatmeansthatsomeonefeelinglessdizzyindicatesatreatmentsuccess.Manynewpatientsaskifanythingcanbedonetochangethecourseofthedisease,whichdamagesbalanceandhearingovertime.Thesadansweris“no”,thereisnotherapythatcurrentlyexiststhathasbeenshowninastudytoalterthe“naturalhistory”ofMeniere’sdisease.Thatmeansthatthemedicalprofessioncurrentlydoesn’thaveawaytopreventdamagetotheinnerearcausedbyMeniere’sdisease.However,thereareeffectivewaystotreatthehearinglossandbalanceproblemscausedbyMeniere’sdisease.Also,keepinmind,mostpeoplesaythattheworstpartofMeniere’sdiseaseisnotthehearingloss,butinsteadtheattacksofvertigo,andtherandomnessandseverityofthoseattacks,whichmostaffectqualityoflife.Forpreventingattacksofvertigo,thereareseveraleffectivetreatments.

Tosimplifythings,wecanconsider4categoriesoftreatment:TreatmentCategory DetailsTreatmentsthataren’tharmful(mostofthetime),andseemtobehelpfulforsomepeople

ThiscategorycoversthemajorityoftreatmentsforMeniere’sdisease.Theevidenceforeachisreviewedbelow.The“standard”treatments,atleastintheUnitedStates,fallintothiscategory

- Lowsaltdiet- Waterpill(diuretic)- Steroids(usuallyinjectedintothe

ear)Inaddition,therearenumerousothertherapiesthatdon’tappeartobeharmful,andsomepeopleswearbythem,butthereisreallynogoodevidencethattheyarehelpful

- Betahistine- Endolymphaticsacsurgery(there

issomenewdataregardingavariantofthisprocedurethatisdiscussedbelow)

Treatmentsthatwork,butdosobyablating(destroying)innerearfunction

Thiscategorycoversseveralveryeffectivetreatments.However,theyallworkbydamagingtheinnerear.Theconceptisthatbydamagingtheear,theearwillbecometoo“weak”togenerateattacksofvertigo.However,theycomeatacost,whichisthatanyremaininghearingfunctionoftheearcanbelost.

- Gentamicin- Labyrinthectomy- VestibularNerveSection

Rehabilitation Thiscategorycoverstreatmentsthat

helpcompensateforthedamagedonetotheinnerearbyMeniere’sdisease

- Hearingaids- CochlearImplants- VestibularRehabilitation

SymptomaticTreatments Thesetreatmentsaren’tthoughttochangethedisease.Instead,theyjusttreatsymptomscausedbytheattacksofvertigo,includingdizziness,andnausea

- Meclizine

- Phenergan- Benzodiazepines- Zofran

Ok,nowthatwehavegoneoverthebroaddetails,let’sgothrougheachtreatmentindetail.Category Treatment DetailsTreatmentsthataren’tharmful(mostofthetime),andseemtobehelpfulforsomepeople

LowSaltDiet LowsaltdietisverycommonlyusedasthefirstlinetreatmentforMeniere’sdisease.It’sthoughttolowerthepressureintheinnerear.Whilethereislittleevidencethatitishelpful,therearecertainlysomepatientswhonoticeadifference.Finally,alowsaltdiet(like1500mgofsodiumdaily)isrecommendedbytheAmericanHeartAssociationforoverallhealthanyway.Hereisalinktosomeinformationregardingalowsaltdiet:https://www.ucsfhealth.org/education/guidelines_for_a_low_sodium_diet/.

Diuretic/WaterPill Adiuretic(liketriamterene/hydrochlorothiazide,oracetazolamide)isalsoverycommonlyusedafirstlinetherapy.Theconceptissimilartothelowsaltdiet:attemptingtolowerthepressureintheear.Reviewsofthebestavailableevidenceshowinconclusivefindings,butlowqualityevidenceseemstoindicatethatdiureticsmaybehelpful,atleastforsomepatients34.Todate,therehavebeentwoclinicaltrialsperformedwithaplacebogroup,andpatientsrandomizedtotreatment.56Theybothconcludedthatdiureticsarehelpfulwithpreventingattacksofvertigo,butbothstudydesignshavebeencriticized.It’srecommendedthatyoukeepaneyeonyourbloodpressureandelectrolytes(suchaspotassium)whileonadiuretic,andthisisdonethroughyourprimarycaredoctor.

SteroidInjections Therehavebeenseveralstudiesthat

(IntratympanicSteroids)

showbenefitofintratympanicsteroidinjections.Thisisaprocedurethatinvolvednumbinganareaoftheeardrum,andtheninjectingasteroidsolutionthoughtheeardrumdirectlyintotheear.Theproceduretakesaboutaminute,andcancausebriefdiscomfort.Mostpatientstoleratetheprocedurefine.Afterwards,youliedownwiththeinjectedearfacingupwardsfor20-30minutes.Thenyoumayreturntoyournormaldailyactivities.Forafewweeksaftertheinjection,it’srecommendedtoavoidgettingwaterintheear.Therisksoftheprocedurearereallylow.Thereisabouta1%riskofaperforatedeardrum.Asmalltrialin2005inMexicoshowedbenefitsofsteroidoverplacebo.7Twootherlargertrialscomparedsteroidinjectionstogentamicininjections(discussedbelow),andfoundconflictingresults.89Casanietal.foundthatgentamicinprovidedmuchbettercontrolofvertigo,whereasPatelelal.foundthatgentamicinandsteroidinjectionsprovidedsimilarcontrolofvertigo.Givenallthis,wethinkthatthereisgoodevidencetosupporttheuseofsteroidinjectionsinMeniere’sdisease,giventhattherisksarelow,andthepotentialbenefitsarehigh.Theyclearlydonotworkineveryone,butitismostreasonabletotrythistreatmentpriortoanydestructivetreatments.

Betahistine BetahistineisamedicationthatistakenbymouthforMeniere’sdisease.ItisverycommonlyusedinEurope,Canada,andotherpartsoftheworld,andmuchlesscommonlyusedintheUnitedStates.It’snotclearhowitworks,somethinkthatitimprovesbloodflowtothecochlea.Inalargemulticenterrandomizedclinicaltrial(BEMEDtrial),patientsdidequallywellonplacebo,low-dosebetahistine,orhigh-dosebetahistine.10Thatmeansthat

wehavesomereasonableevidencethatbetahistinedoesn’treallywork.Therefore,wedonotusuallysuggestitasatreatmentoption.Ontheotherhand,itdoesnotseemtocausesignificantsideeffects,anditisverysafe.Therefore,ifapatientwantstogiveitatry,basedontheirownresearch,wedon’tseemuchharminthat,andwearehappytoprovideaprescription.IntheUS,thedrughastobecompounded(madeforyoubyaspecialpharmacy),ororderedfromCanada,andthereforeitcanbealittleexpensive.IttakesmoreefforttogetthismedicationthanothermedicationsthatareapprovedbytheFDA.

MeniettDevice TheMeniettdeviceusespulsesofpressuretotreatMeniere’sdisease.Sincetheeardrumpreventspressuretransmissiontotheear,aneartubemustbeplacedpriortousingthedevice.Eartubesareplacedintheoffice,andittakesabout2minutesforplacement.Thereisalwaysasmallrisk(1-2%)ofhavingapersistentholeintheeardrumafterplacinganeartube.TherehavebeenseveralplacebocontrolledrandomizedtrialslookingatwhetherornottheMeniettdeviceworks.Ultimately,theredoesnotseemtobeabeneficialeffectfromthedevice.11Arecent,large,multicentertrialdidnotfindanyadvantageofthedeviceoveraplacebo.12Giventheavailableevidencetodate,wedonotroutinelyrecommendtheMeniettdevice.However,wedobelieveittobelowrisk,soifsomeonereallywantstotryit,basedontheirownresearch,wecanhelpfacilitatethat.

EndolymphaticSacSurgery

Theendolymphaticsacisapartoftheinnerear.Whilenooneisreallysureofitsexactfunction,therearetheoriesthatit’sinvolvedwithabsorptionofendolymph,orthatithasanimmunerelatedfunction.In1927,Portman

describedshuntingoftheendolymphaticsacasatreatmentforMeniere’sdisease,andithasbeenhotlydebatedeversince.Thesurgeryinvolvesarelativelysafeprocedurethattakesabout3hours,withpatientsabletogohomethesameday.However,allsurgeriesdocarrysomeelementofrisk,relatedtogeneralanesthesia,theincision(infection,numbness,bleeding,scarring,pain)andthevulnerablestructuresneartheareaofsurgery(hearingandbalancemechanism,nervethatmovesthemusclesoftheface,eardrum,earcanal,andbrain).Todate,therehavebeen2clinicaltrialsthatexaminedthissurgery,13bothbythesamegroupofauthors.Theyfoundthatpatientsimprovedafterendolymphaticsacsurgery,butthatpatientsalsoimprovedaftera“placebo”surgery.Therefore,theyconcludedthatendolymphaticsacsurgeryisnoteffective.Otherauthorshavearguedthattheiranalysisofthedatawasincorrect.14Also,somewouldarguethatif~2/3rdofpatientsgetbetterwithsurgery,perhapsitisworthdoingevenifthereisa“placeboeffect”.Therefore,ourapproachistosaythatwearecertainlywilling(andable)toperformendolymphaticsacsurgery,butthatwecannotstronglyrecommenditbasedoncurrentevidence.Morerecently,agroupinMontrealledbyProfessorIssamSalibahavepublishedresultsonavariationoftheendolymphaticsacprocedure.15Insteadoftryingtodecompressor“shunt”thesac,theyseparatethesacfromtherestoftheinnerear.Thatisaccomplishedwithplacingtitaniumsurgicalclipsalongtheendolymphaticduct,whichconnectsthesactotheinnerear.Dr.Salibahasdonethisprocedureinover200patients,andreportsthattheresultsarequitegood,

with95%ofpatientsgettingpermanentrelieffromvertigo,and~20%gettingimprovementinhearing.However,theseresultsareearly,andhavenotbeencorroboratedwithothercenters.Furthermore,thereisalonghistoryofproposed“miraclecures”forMeniere’sdisease,andingeneralweadvisesuspicionuntiltherearewellconductedstudiesthatprovethatanewtreatmentisefficacious.Whilewedon’thavemuchpersonalexperiencewiththisprocedure,basedonthepublishedresultsandtherelativelylowriskofsideeffectswearehappytodiscusstheproceduretopatientswhoarenotgettinganybenefitwithothertreatmentsinperson.

Treatmentsthatwork,butdosobyablating(destroying)innerearfunction

GentamicinInjection

Gentamicinisanantibioticwithinaclassofantibioticscalledaminoglycosides.Longago,aminoglycosideswerenotedtohavethesideeffectofcausinghearingandbalanceimpairments.Thishasbeenstudiedfurther,anditturnsoutthatgentamicinistoxictoinnerearhaircells,whicharecriticaltothesensoryfunctionoftheear.Interestingly,gentamicinismuchmoretoxictovestibularhaircells(onesresponsibleforbalance,andalsoresponsibleforthesymptomsofMeniere’sdisease),thantocochlearhaircells(theonesresponsibleforhearing).Thebasicideabehindgentamicinistofurtherweaken/damagetheinnerear,sothatitcannolongercauseepisodesofvertigo.IncaseswhereMeniere’shasalreadydamagedtheear,itissometimesadvantageoustojust“finishthejob”,andfurtherpoisontheearsothattheMeniere’s“burnsout”,orisnolongercapableofcausingsymptoms.Thisprocedureisdoneintheoffice,andissimilartoasteroidinjectionintheear.Theriskofcreatingaholeintheeardrumislow,about1%.Thereisariskhearingloss(about17%riskofasmallchangein

hearing),anddeafness(~3%risk).16Manypatientsweseeareundertheimpressionthatgentamicinalwayscausesdeafness,butthisiscertainlynotthecase,it’sjustaknownpotentialsideeffect.Theothersideeffectisthatgentamicincanreducethenormalfunctioningofthebalancesystembeyondtheinjectedear.Thisismostnotablewithareflexcalledthevestibulo-ocularreflex,whichkeepsthingsthatwearelookingat(visualtargets)steadyaswewalkormoveourheads.Withdamagetothatreflex,onecanexperiencedizzinessorblurredvisionwithrapidheadturns.Whatisreallyoccurringisthattheeyesnowneedafractionofasecondtocatchupwiththehead.Luckily,vestibularphysicaltherapyisprettyeffectiveinrehabilitatingthatreflex(likeallrehab,itcanneverrestoreperfectfunction,butitdoesrestoresgoodenoughfunction).About50%ofpatientsgetreliefwithoneinjectionofgentamicin,andtheother50%needmorethanoneinjection.Weprefertoslowlytitratetheinjections,meaningthatwegiveone,andthenseehowyoudoandrepeatasneeded.Thereasonswespaceoutinjectionsaretominimizerisktohearing,andalsotomaintainanyresidualbalancefunction.Thedecisiontoproceedwithgentamicinisatoughonetomake,givenalltheinformationabove.However,thereisreallygoodevidencefrommultipletrialsthatit’shighlyeffective.17Itistheonetreatmentthatalmostalwaysworks,whichisprettyremarkableconsideringit’saproceduredoneintheoffice.Forpatientswhohavetriedthenon-destructivetreatments,havenotgottenbetter,andfeelthattheirdiseaseissignificantlychangingtheirlives,wedorecommendgentamicin.ThedecisionismucheasiertomakeiftheMeniere’s

diseasehasalreadydamagedthehearing,asyouhavelesstopotentiallylose.Finally,therearesomecases(about5%)ofbilateralMeniere’s,wherethediseaseaffectsbothears.Inthosecases,wedoemployextracautionwhenconsideringgentamicin,becausewedon’twantyoutolosebalancefunctioninbothears.Thatcanresultinsevereimbalanceandunsteadiness.Luckily,bilateralMeniere’sdiseaseisrelativelyrare.

Labyrinthectomy Labyrinthectomyreferstosurgicaldestructionoftheinnerear(“thelabyrinth”).Theprocedureisdoneundergeneralanesthesia,andusuallytakesabout3hours.Allresidualhearingandbalancefunctionislostwiththeprocedure.Unlikegentamicin,whichcanproduceapartialablation,labyrinthectomyalwaysproducesacompletelossoffunction.Therationalforthisprocedureissimilartogentamicin:sincetheearhasbeendamagedbyMeniere’sdisease,by“finishingthejob”,orcompletingthedamage,theearwillnolongerbeabletogenerateattacksofvertigo.Thesuccessrateisveryhigh,providedthatthediagnosisofMeniere’sdiseasewascorrect.However,likewithgentamicin,therearecertain“costs”associatedwithdestroyingtheear.Thisincludeslossofallresidualhearing(“deafear”),andalsolossofanyremainingbalancefunction.Thiscancausedizzinesswithheadmovements,andalsounsteadiness.Vestibularphysicaltherapyisusedtohelpwithcompensationafterwards.Youusuallystayinthehospitalfor1-3daysafterthesurgery.Additionally,therearerisksrelatedtoanyearsurgery,includinginfection,numbness,bleeding,scarring,andpain,damagetothenervethatmovesthemusclesoftheface,tothetastenerve,totheearcanaloreardrum,andleakage

ofspinalfluid.Sincetheadventofgentamicin,wearedoinglabyrinthectomiesmuchlessfrequently.Thisisbecausewecanusuallyachievethesameresult,butwithanoutpatientprocedure,ratherthananinvasivesurgery.Furthermore,sincegentamicinproducesapartiallossoffunction,andlabyrinthectomyproducesacompletelossoffunction,rehabilitationisusuallybetterandeasieraftergentamicin.Therefore,weareusuallydoinglabyrinthectomiesinonlyafewsituations:ifsomeonehadgentamicin,butdidn’tgetanybenefit,orifwewanttodoanothersurgicalprocedureatthesametime(likeacochlearimplanttorestorehearing).

Vestibularnervesection

Avestibularnervesectionisasurgicalprocedurewherethevestibular(balance)nerveisdivided.Thisprocedureisdoneunderanesthesia,andinvolvesa“craniotomy”,whichmeanscreatinganopeningintheskulltoaccessthebraincavity.Thenerveisthencutneartheopeningoftheinternalauditorycanal,whichisabonytunnelthroughwhichthehearingandbalanceandfacialnervestravelontheirwayfromthebraintotheear.Becauseofthis,theprocedurecarriesalltherisksofalabyrinthectomy,butalsoleakageofspinalfluid,anddamagetothebrain(seizure/stroke/death).Withthatsaid,risksareactuallyprettylow,andit’sconsideredasafeprocedure.Theusualhospitalstayis2-4days.Theprocedureisoftendonewithbothanearsurgeonandaneurosurgeon.Thedistinctadvantageofthisprocedureoveralabyrinthectomyisthathearingispreserved,becauseonlythebalancenervesarecut,notthehearingnerve.Hearinglossisalwaysariskoftheprocedure,butthatriskislow(~5%).

However,similartoalabyrinthectomy,thisproducesacompletelossofbalancefunction.Sincegentamicinismuchlowerrisk,anditonlyproducesapartiallossofbalancefunction,andtherisktohearingisrelativelylow,wegenerallythinkthatgentamicininjectionsarepreferabletoavestibularnervesection.

Rehabilitation RegularHearingAid

HearingaidsareveryusefulforrestoringhearingthathasbeenlostduetoMeniere’sdisease.Everyyearhearingaidoptionsandfunctionimprove,astechnologyadvances.Today’scurrentmodelsemployavarietyoftechnologiesdesignedtoimprovesoundclarity,includingdirectionalmicrophones,backgroundnoisereduction,anddigitalprocessingofthesoundinformation.Additionally,itisincreasinglyeasytoconnectahearingaidtootherdevices,suchasacellphone,oraTV.ForMeniere’sdisease,werecommendseeinganaudiologisttoproperlyfitthehearingaidratherthanorderingalisteningdeviceonline.ThatisbecausetherearesomenuanceswithhearingaidsandMeniere’sdisease.Forexample,thehearingcandeteriorateovertime,andthereforeyouusuallywanttogetamorepowerfulhearingaidthanyouneed,justincasethatoccurs.Hearingaidsaretailored(programmed)toyourspecificpatternofhearinglossbasedonyourhearingtest.It’sworthpayingparticularattentiontoonevariableonthehearingtest:thewordrecognitionscore(WRS).Thisscoreisameasureofhowclearlywordsareperceived,providedthattheyareloudenough.Thisisimportant,becausebeyondacertainlevelofhearingloss,itdoesn’treallymatterhowloudyoucanmakethesoundwithahearingaid,speechwilljustsoundlikenoisewithoutclarity.Asaruleofthumb,iftheWRSisabove50%correct,thenahearingaidis

helpfulinrestoringsoundforcommunication.Ifthevalueisbelow50%,thenahearingaidismorelikelytojustamplifynoise,andnotprovideclearsound,andisthereforenotashelpful.Inthosecases,aCROShearingaid,aboneanchoredhearingaid,oracochlearimplantcanbehelpful(seebelow).Hearingaidsarealsothesinglebesttreatmentfortinnitus,orringingintheears.

CROSHearingAid ACROS(contralateralroutingofsound)hearingaidisaspecializedtypeofhearingaidthattakessoundsfromthesideofthebadear,andsendsthemovertothegoodear.Thisrequireswearingtwodevices,amicrophoneonthebadear,andareceiver(akaspeaker)inthegoodear.Patientswithsinglesideddeafnessusuallyexperience4problems:

- Troublehearingsoundsthataredirectedtowardsthebadear.Forexample,ifIhaverightsidedhearingloss,andIamdrivingacar,Iwillhavetroublehearingthepassenger.

- Difficultytellingwheresoundsarecomingfrom.

- Increaseddifficultywithlisteninginnoisyenvironments.

- Listeningtakesmoreenergy,soyouaremoretiredattheendoftheday.

CROShearingaidsaregoodforthefirstproblem(hearingsoundsonbadside),theydon’thelpwiththesecondproblem(soundlocalization),andtherearemildbenefitswiththethirdandfourthproblems(backgroundnoiseandlisteningfatigue).However,theyarenon-invasive,andthereisatrialperiodforallhearingaidsallowingyoutoreturnthemwithin45daysifyoudonotfindthemhelpful.Therearenodownsides,otherthanlosttime/money.Therefore,it’s

alwaysreasonabletotryaCROShearingaid.

BoneAnchoredHearingAid(BAHA)

SimilartoaCROShearingaid,aBAHAisusedtotreatsinglesideddeafness.However,thisisasurgicalprocedure.Theconceptisthatbyvibratingtheskullonthebadside,thesoundscanbesentthroughtheskulltothegoodside(meaningwedon’t“feel”thosevibrations,we“hear”them,andtheysoundjustlikeregularsounds).TherearetwoBAHAvariants:onewhereascrewisplacedintotheskull,andcomesoutthroughtheskin,andasoundprocessorissnappedontothat.Inthesecondvariation,amagnetisscrewedtotheskull,thenitiscoupledtoanexternalmagnet,whichisattachedtothesoundprocessor.Inthissecondtype,thereisnoscrewcomingoutthroughtheskinasamagnetholdstheprocessorinplace.Thesoundqualityisslightlybetterwiththescrewcomingthroughtheskin(becausenosoundenergyislostintransmissionofvibrationsacrossskin),butontheotherhand,somepeopledon’twanttohaveascrewcomingoutthroughtheirskin.Interestedpatientsareencouragedtovisitthecompanywebsites(www.cochlear.com,www.oticonmedical.comandwww.sophono.com).Ingeneral,thesoundqualityisverygoodwithaBAHA,andyouonlyhavetowearonedevice(asopposedtotwowithaCROSaid).However,itdoesinvolveasurgicalprocedure,withrisksofinfection,woundproblems,healingproblems,bleeding,scarring,pain,andfailureofthedevicetoproperlyhealintothebone.

CochlearImplant

Cochlearimplantsareusedtorestorehearingforsinglesideddeafness.UnliketheCROSandtheBAHA,theyactuallyrestorehearingtothedamagedear,ratherthansendingsoundsovertothegoodear.Asurgicalprocedureisneeded

------------------------Vestibularrehabilitationtherapy(VRT)

toplacetheimplant,whichtakes2-3hours.Theprocedureisdoneasanoutpatient,andyougohomethesameday.It’simportanttoknowthatthisafundamentallynewanddifferentwayofhearing,andthereforeittakesquiteabitoftimeforthebraintoadaptand“relearn”howtohear.Italsotakeseffort,andwearingthedeviceasoftenaspossibleisnecessarytomaximizebenefit.It’salsoimportanttoknowthatatthetimeofwriting,theFDAhasnotyetapprovedthedevicesforusewithsinglesideddeafness,insteadtheyrequirebothearsbedeafpriortoimplantation.Thismeansweareusingthedevicesinan“offlabel”fashion,andalsothatwemayhaveafightwithyourinsurancecompanytogetthedeviceapproved.Interestedpatientsareencouragetolearnmorethroughthecompanywebsites:www.cochlear.com,www.advancedbionics.com,andwww.medel.com.Interestingly,cochlearimplantsseemtobereallyhelpfulfortinnitusassociatedwithsinglesidedhearingloss.----------------------------------------------------Somephysicaltherapists(PTs)arespecificallytrainedinevaluationandrehabilitationofvestibularweaknesses.ThesePTscanmeetwithyoutoassessyourbalanceabilitiesandthenteachyouaseriesofexercisestohelpyourbalancesystemcompensateforthedamagedonebyMeniere’sdiseaseorduetotreatmentsforthedisease.VRTtypicallyrequiresaseriesof3-5visitswithaPT,withexercisesdonebypatientsathomeinbetween.

SymptomaticTreatments

Meclizine Meclizineisanantihistaminethatisusedtotreatdizziness.Themostcommonmisconceptionisthatitcanprevent

dizziness.Thisisnotthecase,insteadmeclizineisusedtomakeyoufeelbetterifyouhavedizziness(meaningtoreducetheintensityofthedizzinessthatyouarefeeling).Sedationiscommon,anddrymouthcanoccur.Itisusuallytakenbymouth,asneededfordizziness,and25mgisthetypicaldose.Itshouldbeusedverycautiouslyintheelderly,ifatall.

Benzodiazepines(likeClonazepam/KlonipinorValium/Diazepam)

Benzodiazepinesarealsousedtosymptomaticallytreatdizziness.Thatmeansthattheywilllessentheintensityofthedizzinessthatyouexperience,buttheydonotpreventfutureattacksofdizziness.Theyarestrongerthanmeclizine,andthereforeshouldbetriedifmeclizineisnotfoundtobeeffective.Acommondosageis0.25or0.5mgofclonazepam,takenasneededfordizziness.Oneseriousconcernwithbenzodiazepinesistheiraddictivepotential.Therefore,greatcautionisneeded,andtheyshouldonlybeusedacoupletimespermonth.Webelievethatmorefrequentusagecanbedangeroustoyou,addicting,andcounterproductivetoyourbalancesystem.Sideeffectsincludesedation,moodchanges,anddizziness(ironicallyallmedicationsthattreatdizzinesscarrythispossiblesideeffect).

Zofran Zofranisastronganti-nauseamedication.Itisfastacting,andeffective.Theusualdoseis4mg,andthereisaformulationthatdissolvesunderthetongue.Possiblesideeffectsincludeheadache,heartrhythmchanges,andliverandGIproblems.However,mostofthetimeitiswelltolerated,anditseemstobeveryeffective.

Phenergan Phenerganisanotheranti-nauseamedication.Theusualdosageis25mg;itcanbegivenorallyorasasuppository.Itisusuallywelltolerated,butpossiblesideeffectscanincludeheartproblems,skinproblems,allergicreactions,drymouth,

dizziness,sedation,andotherrarebutserioussideeffects.WeusuallyfindthatZofranismoreeffective,butPhenerganisagoodoptiontotryinpatientswhodon’tgetgoodnauseareliefwithZofran.

WhichtreatmentshouldIchoose? Whilethereisn’tarightorwronganswertothisquestion,hereismyadvice:- Everyoneshouldeatalowsaltdiet.It’snotharmful,andtheAmericanHeartAssociationrecommendskeepingsaltunder1500mganywayforcardiovascularhealth.Plus,itmaybebeneficialforMeniere’s.

- Everyoneshouldtryadiureticatsomepoint.Whiletheevidenceisn’tgreat,thistreatmentisconsidered“standardofcare”,andthebenefitsseemtooutweightherisks.Wedon’tadvocateforbeingonadiureticforeverifyoudon’tfeellikeitishelping.Wedofeelthata3-monthtrialisveryreasonableineveryonewithMeniere’sdisease,toseeifit’shelpfultoyou.

- Ifyouarestillgettingvertigoattacks,despiteeatingalowsaltdiet,andtakingadiuretic,thenyoushouldtrysteroidinjections.Thereisgoodevidencethattheyarehelpfulformanypatients.Infact,Irecommendtryingasteroidshotatleast3times.Theharm(riskofasmallholeintheeardrum)isrelativelylow(1%),andtheprocedureisusuallywelltolerated(meaningwhileitcertainlyisn’tfun,mostpatientsdon’tthinkit’sparticularlypainful).

- Ifyouarestillgettingvertigoattacks,andlowsaltdiet,diuretic,andsteroidinjectionsaren’thelping,youshouldthinkaboutendolymphaticsacsurgery.Seeaboveforanin-depthdiscussion.

- Ifyouarestillgettingvertigoattacks,andlowsaltdiet,diuretic,andsteroidinjectionsaren’thelping,youshouldthinkaboutagentamicininjection.WeseemanypatientswhomIthinkwouldbenefitfromgentamicin,butwhochoosenottotryitforfearofsideeffects.Whiletherearecertainlysideeffectstogentamicininjection,it’sveryeffectiveatpreventingattacksofvertigo.Therefore,youhavetoweighwhichisworse-thesideeffects,ortheattacksofvertigo.Thesideeffectsincludeapossibility(17%)ofworsenedhearing,3%riskofdeafness,andlikelihoodofoscillopsia(inabilityoftheeyestokeepupwiththeheadwith

quickheadmovement,resultinginblurringofthevisualworldwithquickheadmovements)16.However,hearinglosscanbetreatedwithahearingaidorcochlearimplant,andoscillopsiacanbetreatedwithvestibularphysicaltherapy.

- Youshoulddefinitelydoyourownresearch,andtalktootherMeniere’spatientsthroughonlinechatroomsandsupportgroups.

- Ifhearinglossbothersyou,youshouldconsiderahearingaid,oracochlearimplant.Thedevicecanbeatraditionalhearingaidifthereisstillsomehearingintheear,oraCROSorboneanchoredhearingaidifthereisn’tadequatehearingintheaffectedear.

- Ifyouhaveimbalance,oroscillopsia(blurringofthevisualworldwithquickheadmovements),thenyoushoulddovestibularphysicaltherapy.

- Youshouldhaveaplanforanyattacksofvertigo,withmedicationstohelpyoufeelbetterduringattacks.Thisusuallyincludesamedicationtotreatnausea(likeZofran,whichcomesinaformthatdissolvesunderthetongue),andalsoamedicationfordizziness(likeClonazepamorMeclizine).

- AnyonewhodevelopsanxietyordepressionbecauseofMeniere’sdiseaseshouldseeamentalhealthprofessional.

- Ifyouhaveahistoryofmigraine,thenyoushouldtrymigrainetreatmentsbeforedoinganyablativetreatmentsforMeniere’sdisease.Thetwodisordersarecloselyrelated,andwefindthatpatientswilloftengetbetterwithmigrainetreatments.

Migraine MorethanhalfofpatientswithacleardiagnosisofMeniere’sdiseasehaveahistoryofmigraine,whichiswayhigherthanwouldbeexpectedbychance.18Thereisastrongconnectionbetweenthesetwodiseases,whichwedon’treallyunderstand.It’spossiblethatmigrainespredisposepeopletothedevelopmentofMeniere’sdisease.Furthermore,migraineisthemostcommoncauseofdizzinessintheUnitedStates,farmorecommonthanMeniere’sdisease.SometimespatientswithMeniere’sdisease,andahistoryofmigraine,have“migrainous”featuresduringattacksofvertigo,includinglightsensitivity,soundsensitivity,orheadache/headpressure.Therefore,ifthereisahistoryofmigraine,ormigrainousfeaturesduringattacksofvertigo,themigraineaspectofthediseaseshouldbetreated.

It’sbeyondthescopeofthisreviewtodiscussmigrainetreatments,butpleaseconsiderreadingthisexcellentreviewofmigraineassociateddizziness:http://www.hopkinsmedicine.org/otolaryngology/_docs/Migraine%20patient%20handout.pdf. Inshort,migrainetreatmentinvolvesavoidanceofmigrainetriggers(suchasstress,poorsleep,pain,certainfoods,hormonalchanges,andsensorytriggerlikebrightlightsorstrongsmells),useofvitamins/dietarysupplements(magnesium,riboflavin,andothers),andprescriptionmedications. Manypeopleunderestimatemigraine,andhowmuchitcanbecontributingtobalanceandearsymptoms.

WhenshouldIgetvestibulartestingdone? Vestibulartesting,atthispointintime,isnotneededtodiagnoseMeniere’sdisease.Inthelatestagesofthedisease,manypatientsdoendupwithabnormalitiesonvestibulartesting.ThesearenotspecifictoMeniere’sdisease,butareamarkerofthedamageitdoes.Thisincludesunilateralweaknessoncalorictesting,andchangestotheVEMP(vestibularevokedmyogenicpotentials)responses.Interestingly,vHIT(videoheadimpulsetest)testingisusuallynormal(exceptduringattacks).Itseemsclearthattestingshouldbedonebeforeanyablativetreatment,likegentamicinorsurgery.WhataboutothertreatmentsthatIheardabout,orfoundthroughresearchontheInternet? Meniere’sdiseaseislifechanging,andwhiletherearenumeroustreatmentsavailable,thereisn’tcurrentlyanideal“cure”.Inthatvacuum,thereisanever-endingsupplyofunproventreatments.Allofthemaresupportedbyanecdotalevidence(meaningthatsomepeopletriedthem,andfoundthemtobeeffective).Wecannotendorseanytreatmentthathasnotbeenevaluatedinascientificstudy.Thisisbecauseweknowthatnomatterwhatwedo,somepatientswillgetbetter(justbecausethediseasetendstogetbetterovertime).Unlessanewtreatmentisevaluatedbyascientificstudy,whereitiscomparedtoaplacebo,wereallydon’tknowifitiseffective.Furthermore,aswithallmedicaltreatments,thereisalwaysapotentialforharm.So,feelfreetodoyourownresearch,butpleasemaintainagooddegreeofsuspicion.

Ifthesetherapiesreallyworked,thenwhywouldn’ttheirinventorswanttostudythemscientifically,andproveit?ArethereanygoodresourcesontheInternetforMeniere’sdisease?

Yes-thereare.Seebelow.Inparticular,IthinkthereisalotofreallygoodinformationonDr.TimothyHain’swebsite,whichisthefirstlink.Dr.TimHainWebsite,sectiononMeniere’sdisease(highlyrecommended)http://www.dizziness-and-balance.com/disorders/menieres/menieres.htmlNationalInstituteofHealth(NIH)websitehttps://www.nidcd.nih.gov/health/menieres-diseaseVestibularDisordersAssociationhttp://vestibular.org/menieres-diseaseWikipediahttps://en.wikipedia.org/wiki/M%C3%A9ni%C3%A8re%27s_diseaseFinalthoughts:Meniere’sdiseasecanbehorribletoexperience.Itisalifechangingillnessthatcausessuddenandviolentepisodesofdizziness,tinnitus,andearpressure,allthewhileslowlydestroyingtheinnerear,causingpermanenthearingdamage.Theepisodesoccursuddenlyandwithoutwarning,andareveryintense,soitiscommontoexperienceanxiety,stress,sadness,andasenseofhopelessness.Theseareallimportantthingsthathavetobeaddressedaswell.Sometimespatientsdowellwiththeirowncopingstrategies,likefamily/friendsupport,meditation,mindfulness,exercise,yoga,taichi,andthingslikethat.Othertimes,theyrequirehelp,likefromapsychiatrist,tohelptreatanyanxietyanddepressionthatcanoccur.

WithtreatmentofMeniere’s,thegoalistoreduceandpreventtheepisodesofdizziness.Weareusuallyabletoachievethatgoal,becausewehavetheoptionofchemicallyorsurgicallydamagingtheinnerearintentionallysothatitcannolongercauseepisodesofvertigoordizziness.Currently,wedon’tseemtobeabletopreventthehearinglossthatoccursovertime,butweareabletooffertreatmentsforthathearingloss(likehearingaids,orcochlearimplantswhenhearingaidsarenolongersuccessful).ManyscientistsaroundtheworldstudyingMeniere’sdisease,andwecontinuetohopethatwecanfigureoutthecausesofMeniere’sdisease,andhowtocureit.

Meniere’sdiseaseworksheet

(Pleaseindicatewhatpriortreatmentsyouhavehad.)Thepurposeoftheworksheetistohelpyoufigureoutwhatyouhavealreadytried,

whatworksforyou,andwhatyouhavenotyettriedthatperhapswouldbereasonable.

Treatment

TriedIt? DiditHelp? Details

LowSaltDiet

Diuretic(waterpills-likeHCTZ/Triamterene,orDiamox)

OralSteroids(Steroids,likeprednisone,takenbymouth)

IntratympanicSteroids(steroidinjectionsintotheear)

Betahistine

MeniettDevice

EndolymphaticSacSurgery

IntratympanicGentamicin(Injectionofantibioticintotheear,withthegoalofpoisoningtheinnerearbalancesystem)

Vestibularnerve

section(surgicalcuttingofthebalancenerve)Labyrinthectomy(Surgicaldestructionoftheinnerear)

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