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www.bjorl.org

Brazilian

Journal

of

OTORHINOLARYNGOLOGY

SPECIAL

ARTICLE

Neurotology:

definitions

and

evidence-based

therapies

---

Results

of

the

I

Brazilian

Forum

of

Neurotology

Márcio

Cavalcante

Salmito

a,

,

Francisco

Carlos

Zuma

e

Maia

b

,

Mário

Edvin

Gretes

c

,

Alessandra

Venosa

d

,

Fernando

Freitas

Gananc

¸a

a

,

Maurício

Malavasi

Gananc

¸a

a

,

Raquel

Mezzalira

e

,

Roseli

Saraiva

Moreira

Bittar

f

,

Alexandre

Camilotti

Gasperin

g

,

Anna

Paula

Batista

de

Ávila

Pires

h

,

Bernardo

Faria

Ramos

i

,

César

Bertoldo

f

,

Cícero

Ferreira

Jr.

a

,

Danilo

Real

f

,

Humberto

Afonso

Guimarães

j

,

Jeanne

Oiticica

f

,

Joel

Lavinsky

k,l

,

Karen

Carvalho

Lopes

m

,

Juliana

Antoniolli

Duarte

a

,

Lígia

Oliveira

Gonc

¸alves

Morganti

n

,

Lisandra

Megumi

Arima

dos

Santos

g

,

Lúcia

Joffily

o

,

Luíz

Lavinsky

p

,

Mônica

Alcantara

de

Oliveira

Santos

q,r

,

Patrícia

Mauro

Mano

s

,

Pedro

Ivo

Machado

Pires

de

Araújo

t

,

Pedro

Luís

Mangabeira

Albernaz

u

,

Renato

Cal

v

,

Ricardo

Schaffeln

Dorigueto

w

,

Rita

de

Cássia

Cassou

Guimarães

x

,

Rogério

Castro

Borges

de

Carvalho

y

aUniversidadeFederaldeSãoPaulo(Unifesp),EscolaPaulistadeMedicina,DepartamentodeOtorrinolaringologiaeCirurgia Cérvico-Facial,DisciplinadeOtologiaeOtoneurologia,SãoPaulo,SP,Brazil

bPontifíciaUniversidadeCatólicadoRioGrandedoSul(PUC-RS),PortoAlegre,RS,Brazil cPontifíciaUniversidadeCatólicadeCampinas(PUC-Campinas),FaculdadedeMedicina,SP,Brazil dUniversidadedeBrasília(UnB),Brasília,DF,Brazil

eUniversidadedeCampinas(Unicamp),DisciplinadeOtorrinolaringologiaCabec¸aePescoc¸o,Campinas,SP,Brazil

fHospitaldasClínicasdaFaculdadedeMedicinadaUniversidadedeSãoPaulo(HCFMUSP),SetordeOtoneurologia,SãoPaulo,SP, Brazil

gInstitutoParanaensedeOtorrinolaringologia(IPO-PR),Servic¸odeOtologiaClínica,Curitiba,PR,Brazil hUniversidadeFederaldeMinasGerais(UFMG),BeloHorizonte,MG,Brazil

iUniversidadeFederaldoEspíritoSanto(UFES),Vitória,ES,Brazil jHospitalMaterDei,BeloHorizonte,MG,Brazil

kUniversidadeFederaldoRioGrandedoSul(UFRGS),PortoAlegre,RS,Brazil

lUniversidadeFederaldeCiênciasdaSaúdedePortoAlegre(UFCSPA),SantaCasadePortoAlegre,PortoAlegre,RS,Brazil mUniversidadeFederaldeSãoPaulo(Unifesp),SãoPaulo,SP,Brazil

nUniversidadeFederaldeMinasGerais(UFMG),FaculdadedeMedicina,DepartamentodeOtorrinolaringologia,BeloHorizonte, MG,Brazil

Pleasecitethisarticleas:SalmitoMC,MaiaFC,GretesME,VenosaA,Gananc¸aFF,Gananc¸aMM,etal.Neurotology:definitionsand

evidence-basedtherapies---resultsoftheIBrazilianForumofNeurotology.BrazJOtorhinolaryngol.2020;86:139---48.

Correspondingauthor.

E-mail:marciosalmito@yahoo.com(M.C.Salmito).

PeerReviewundertheresponsibilityofAssociac¸ãoBrasileiradeOtorrinolaringologiaeCirurgiaCérvico-Facial. https://doi.org/10.1016/j.bjorl.2019.11.002

1808-8694/©2019Associac¸˜aoBrasileiradeOtorrinolaringologiaeCirurgiaC´ervico-Facial.PublishedbyElsevierEditoraLtda.Thisisanopen accessarticleundertheCCBYlicense(http://creativecommons.org/licenses/by/4.0/).

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HospitalUniversitárioGaffréeeGuinle,UniversidadeFederaldoEstadodoRiodeJaneiro(Unirio),RiodeJaneiro,RJ,Brazil pUniversidadeFederaldoRioGrandedoSul(UFRGS),FaculdadedeMedicina,DisciplinadeOtorrinolaringologia,PortoAlegre, RS,Brazil

qIrmandadedaSantaCasadeMisericórdiadeSãoPaulo,FaculdadedeCiênciasMédicas,DepartamentodeOtorrinolaringologia, SãoPaulo,SP,Brazil

rInstitutodeAssistênciaMédicaaoServidorPúblicoEstadualdeSãoPaulo,SãoPaulo,SP,Brazil

sHospitalFederaldosServidoresdoRiodeJaneiro,DepartamentodeOtorrinolaringologiaeCirurgiadeCabec¸aePescoc¸o,Riode Janeiro,RJ,Brazil

tUniversidadeFederaldeGoiás(UFG),Goiânia,GO,Brazil uHospitalIsraelitaAlbertEinstein,SãoPaulo,SP,Brazil

vUniversidadeFederaldoPará(UFPa),FaculdadedeMedicina,Belém,PA,Brazil wHospitalPaulista,SãoPaulo,SP,Brazil

xUniversidadeFederaldoParaná(UFPR),Curitiba,PR,Brazil yClínicaBorgesdeCarvalhoOtorrinos,RiodeJaneiro,RJ,Brazil

Received24October2019;accepted6November2019 Availableonline3December2019

KEYWORDS

Vertigo; Dizziness; Neurotology

Abstract

Introduction:Neurotologyisarapidlyexpandingfieldofknowledge.Thestudyofthevestibular systemhasadvancedsomuchthatevenbasicdefinitions,suchasthemeaningofvestibular symptoms,haveonlyrecentlybeenstandardized.

Objective:To present a review of the main subjects of neurotology, including concepts, diagnosis and treatment of Neurotology, defining current scientific evidence to facilitate decision-makingandtopoint outthemostevidence-lackingareas tostimulatefurther new research.

Methods:ThistextistheresultoftheIBrazilianForumofNeurotology,whichbroughttogether theforemostBrazilianresearchersinthisareaforaliteraturereview.Inall,therewillbethree reviewpaperstobepublished.Thisfirstreviewwilladdressdefinitionsandtherapies,the sec-ondonewilladdressdiagnostictools,andthethirdwilldefinethemaindiseasesdiagnoses.Each authorperformedabibliographicsearchintheLILACS,SciELO,PubMedandMEDLINEdatabases onagivensubject.ThetextwasthensubmittedtotheotherForumparticipantsforaperiodof 30daysforanalysis.Aspecialchapter,onthedefinitionofvestibularsymptoms,wastranslated byanofficial translationservice,andequallysubmitted totheotherstagesoftheprocess. Therewasthenain-personmeetinginwhichallthetextswereorallypresented,andtherewas adiscussionamongtheparticipantstodefineaconsensualtextforeachchapter.The consen-sualtextswerethensubmittedtoafinalreviewbyfourprofessorsofneurotologydisciplines fromthreeBrazilianuniversitiesandfinallyconcluded.Basedonthefulltext,availableonthe websiteoftheBrazilian AssociationofOtorhinolaryngologyandCervical-FacialSurgery,this summaryversionwaswrittenasareviewarticle.

Result: ThetextpresentstheofficialtranslationintoPortugueseofthedefinitionofvestibular symptomsproposedbytheBáránySocietyandbringstogetherthemainscientificevidencefor eachofthemainexistingtherapiesforneurotologicaldiseases.

Conclusion:Thistextrationallygroupedthemaintopicsofknowledgeregardingthedefinitions andtherapiesofNeurotology,allowingthereaderabroadviewoftheapproachofneurotological patients based onscientific evidenceand national experience,which shouldassistthem in clinicaldecision-making,andshowthemostevidence-lackingtopicstostimulatefurtherstudy. © 2019 Associac¸˜ao Brasileira de Otorrinolaringologia e Cirurgia C´ervico-Facial. Published by Elsevier Editora Ltda. This is an open access article under the CC BY license (http:// creativecommons.org/licenses/by/4.0/).

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PALAVRAS-CHAVE

Vertigem; Tontura; Otoneurologia

Otoneurologia:definic¸õeseterapiasbaseadasemevidências–ResultadosdoIFórum BrasileirodeOtoneurologia

Resumo

Introduc¸ão: Aotoneurologiaéumaáreadeconhecimentoquetemseexpandidomuito rapi-damente.Oestudodosistemavestibulartemavanc¸adotantoquemesmodefinic¸õesbásicas, comoosignificadodossintomasvestibulares,foramapenasrecentementepadronizadas.

Objetivo: Apresentarumarevisãodosprincipaisassuntosdaotoneurologia,inclusiveconceitos, diagnósticoetratamentodaotoneurologia,definiraevidênciacientíficaatualparafacilitara tomadadedecisõesedemonstrarasáreasmaiscarentesdeevidência paraestimularnovas pesquisas.

Método: Estetextoéfruto doIFórumBrasileirodeOtoneurologia,quereuniuosprincipais pesquisadoresbrasileirosdessaáreaparaumarevisãodaliteratura.Serãofeitostrêstrabalhos derevisãoaserempublicados.Esteprimeiroabordouasdefinic¸ões easterapias,osegundo abordaráasferramentasdiagnósticaseoterceirodefiniráosprincipaisdiagnósticos.Cadaautor fezumlevantamentobibliográficonabasededadosdaLilacs,SciELO,PubmedeMedlinedeum determinadoassunto.OseutextofoientãosubmetidoaosdemaisparticipantesdoFórumpor30 diasparaanálise.Umcapítuloespecial,dadefinic¸ãodossintomasvestibulares,foitraduzidopor servic¸odetraduc¸ãooficialeigualmentesubmetidoàsdemaisetapasdoprocesso.Houveentão umareuniãopresencialem quetodosostextosforamapresentadosoralmenteehouveuma discussãoentreosparticipantesparaadefinic¸ãodeumtextoconsensualparacadacapítulo. Ostextosconsensuaisforamentão submetidosaumarevisãofinalporquatroprofessoresde otoneurologiadetrêsuniversidadesbrasileirase,porfim,finalizado.Apartirdotextocompleto, publicadonositedaAssociac¸ãoBrasileiradeOtorrinolaringologiaeCirurgiaCérvico-Facial,foi escritaestaversão-resumocomoartigoderevisão.

Resultado: Otextoapresentaatraduc¸ãooficialparaoportuguêsdadefinic¸ãodossintomas vestibulares propostospelaBaranySocietyeagrupaasprincipaisevidênciascientíficaspara cadaumdasprincipaisterapiasexistentesparaasdoenc¸asotoneurológicas.

Conclusão:Este texto agrupou de forma racional os principais tópicos de conhecimento a respeitodasdefinic¸õeseterapiasdaotoneurologia,permiteaoleitorumavisãoamplada abor-dagemdospacientesotoneurológicosbaseadaemevidênciascientíficaseexperiêncianacional, quedeveráauxiliá-lonatomadadedecisõesclínicas,emostraosassuntosmaiscarentesde evidênciaparaestimularnovosestudos.

© 2019 Associac¸˜ao Brasileira de Otorrinolaringologia e Cirurgia C´ervico-Facial. Publicado por Elsevier Editora Ltda. Este ´e um artigo Open Access sob uma licenc¸a CC BY (http:// creativecommons.org/licenses/by/4.0/).

Introduction

Neurotology is a medical discipline that explores the interface between otorhinolaryngology and neurology, comprising the clinical evaluation and treatment of sen-sorineuralhearing andbalance disorders.Inrecent years, newknowledge andnewtest modalities havebeen incor-porated intoneurotology.In ordertogather thescientific evidenceofneurotologyintoaneasily accessibleand suc-cinct document, the ABORL-CCF, through its Department ofNeurotology,developedtheNeurotologyForumsproject topresent todoctorswhotreatneurotologicaldiseasesan organized meeting of scientific evidence, facilitating the managementoftheneurotologicalpatient.

Methods

This textwastheresult ofthe INeurotologyForum,held withtheleadingspecialistsinthefieldinBrazilon

Septem-ber2,2017.Priortothemeeting,thetextshadbeenwritten basedona literaturereview togather scientificevidence dividedbytopics.ForthisfirstForum,thetopicdefinitions andtherapieswaschosen.Inadditiontoaliteraturereview, contactwasmadewiththeBaranySociety,whichauthorized theofficialtranslationoftheconceptsdefinedintheworld consensus of the international classification of vestibular diseases,1 which is publishedin its entirety in the Forum

text,onthewebsiteoftheBrazilianAssociationof Otorhi-nolaryngologyandCervicofacialSurgery(ABORL-CCF).

Thetextswrittenbytheexpertswerethenscreenedby theotherForumparticipantsafewweeksinadvance.Each authorpresentedtheirtextonthedayofthemeetingand, afterdebatingthemamongthosepresent, thetextswere finalized by consensus.The texts were then grouped and standardized.OnOctober27,2018,atafinalmeetingwith representativesoftheNeurotologytrainingservicesinthe country,the text wasread again, and minor final adjust-mentsweremade.TherecommendationsofthisfirstForum, basedontheliterature,areobjectivelydescribedand

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sum-marizedinthisarticle.Forfurtherreading,thereadermay refertotheForumtextpublishedinfullontheABORL-CCF website.

Results

Medicalcompetenciesinneurotology

The physician must respect the legal determinations and guidelinesdictatedbytheFederalCouncilofMedicine(CFM) and ABORL-CCF. The teaching of private medical acts in anyformofknowledgetransmissiontonon-medical profes-sionals,includingthosepertainingtoadvancedlifesupport, exceptremoteemergencycare,isprohibiteduntiloptimal resourcesarereached(ResolutionoftheFederalCouncilof

Medicine1718/2004,ratifiedbyABORL-CCFatthe

ABORL-CCFOrdinaryGeneralMeeting).

Themedicalreportwithdiagnosisistheexclusive com-petencyandprerogativeofthephysician,whoisresponsible for diagnosing the diseases and prescribing treatments, whereas the other professionals will act only within the scope of their respective legislations, according to the jurisprudenceoftheSuperior Courts.People whoperform actsofdiagnosisofdiseasesandprescriptionoftreatments shouldbereportedtotheauthoritiesforillegalpracticeof Medicine,acrimeprovidedforinthePenalCodewith penal-tiesranging fromsix monthstotwoyears inprison (Note

fromtheFederalCouncilofMedicineon08/21/2013).

The professionals that comprise the multidisciplinary teamcanperformtheproceduresprescribedbythemedical professionalwithinthelimitsoftheircompetency.Medical consultations,whichincludeanamnesis,physical examina-tion,andcreation ofdiagnostichypothesesandindication oftreatmentthatencompassthemedicalactarethe exclu-siveattributionsofthemedicalprofessionaland,therefore, to establish the diagnosis of the diseases is an exclusive medicalprerogative.

Consideringtheincreasingcomplexity ofmedical prob-lems, it is essential the team of physicians and other health professionals, suchas nurses, pharmacists, physio-therapistsandspeechtherapists,respectthelimitsoftheir competencies, prerogatives and their strict professional scope (Technical Note ofthe Department ofNeurotology

atABORL-CCF08/01/2017).

Definitionsofvestibularsymptoms1

Vertigo(inPortuguese:vertigem)

(Internal) vertigo is thesensation of self-motion when no self-motionisoccurring orthe sensationof distorted self-motion during an otherwise normal head movement. The termencompassesfalsespinningsensations(spinning verti-go)andalsootherfalsesensationslikeswaying,tilting, bob-bing,bouncing,orsliding(non-spinningvertigo).

Dizziness(inPortuguese:tontura)

(Non-vertiginous)dizzinessisthesensationofdisturbedor impaired spatial orientation without a false or distorted senseofmotion.

Vestibulo-visualsymptoms(inPortuguese:sintomas vestíbulo-visuais)

Vestibulo-visualsymptomsarevisualsymptomsthatusually resultfromvestibularpathology or theinterplay between visualandvestibularsystems.Theseincludefalsesensations ofmotionortiltingofthevisualsurroundandvisual distor-tion(blur)linkedtovestibular(ratherthanoptical)failure. Therearefivevestibulo-visualsymptoms:externalvertigo,

oscillopsia, visual lag, visual tilt,and movement-induced

blur.

Posturalsymptoms(inPortuguese:sintomasposturais)

Posturalsymptomsarebalancesymptomsrelatedto main-tenanceofpostural stability,occur-ringonlywhileupright (seated,standing,orwalking).Therearefourpostural symp-toms: Unsteadiness, directional pulsion, balance-related

near-fall,andbalance-relatedfall.

Definitionofvestibularsyndromes

Clinical history is the main tool in the search for patient diagnosis. AccordingtotheBáránySociety,vestibular syn-dromeshave been classifiedinto3 distinctgroups: acute, episodic,andchronic.2

Acutevestibularsyndromes(AVS)

These arecharacterizedbythe abruptonsetofvestibular symptomsthatpersistfordaysorweeks,commonly associ-ated withnausea,vomiting, gaitunsteadiness,movement intolerance and presence of nystagmus. After the initial peak of symptoms, thereoccurs improvement during the first week and gradual recovery over weeks to months. AVS may occur spontaneously, following trauma or expo-sure totoxic agents, amongothers.3 The main diagnoses

areacuteunilateralvestibularhypofunction(vestibular neu-ritis), hemorrhagic or ischemic cerebrovascular accident (CVA)intheposteriorfossa,labyrinthitis,headtraumaand vestibulotoxicity.

Episodicvestibularsyndromes

These present as recurrent episodes of vestibular symp-tomslastingfromsecondstohours.Theinter-crisisperiod may beasymptomatic or maintain some degree of milder dizzinessinrelationtothecrises.4Theycanbesubdivided

into spontaneous (Meniere’s disease, vestibular migraine, transient ischemic attack (TIA) of the posterior circula-tion) and triggered (benign paroxysmal positional vertigo (BPPV), perilymphatic fistula, superior semicircular canal dehiscence (SSCD)syndrome, vertebrobasilar insufficiency syndrome, medication sideeffects, andcentral positional vertigo(CPV).2

Chronicvestibularsyndromes(CVS)

They are characterized by the persistence of vestibular symptomsover along periodof time.Themain diagnoses arepersistentpostural-perceptualdizziness(PPPD),chronic idiopathic unilateral vestibulopathy, persistent unilateral vestibulopathyaftervestibularneuritis,unilateral vestibu-lopathy secondary to vestibular schwannoma, unilateral vestibulopathy after medical procedure, chronic bilateral vestibulopathy. Other diagnoses: CANVAS, posterior fossa

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tumors.5TheBáránySocietydoesnotspecifytheminimum

durationfromsymptomonsettodefineCVS.

Medicationsinneurotology

Vestibularsuppressors

Theymaybeprescribedintheacutephaseofvestibular dis-easesforsymptomaticrelief.Theiruseshouldbeasshort as possible so as not to impair the vestibular compensa-tionandavoidunwantedsideeffects.The mainclassesof medicationsandtheirmechanismsofactionare:

Calcium channel antagonists: sedative action, as they antagonizeslowcalciumchannelsandalsobecausethey have antihistamine action on H1 receptors. In addi-tion to these effects, peripheral vasodilation action is described.6Themostcommonlyusedmedicationsare

flu-narizineandcinnarizine. LevelofEvidence:B.

Degreeofrecommendation:recommended.

Antihistamines: vestibular sedatives with anti-cholinergic and antihistamine effect on H1 receptor. They can be used in acute vertigo, for the relief of nausea and vomiting,7 after repositioning maneuvers

and in the treatment of kinetosis. The most commonly used medications are dimenhydrinate,8 meclizine9 and

promethazine.10

LevelofEvidence:B.

Degreeofrecommendation:recommended.

Benzodiazepines: vestibular sedatives because they potentiatetheinhibitoryactionofGABA.Theymayalso be used in acute vertigo11 and kinetosis prophylaxis.12

The most commonly used drugs are clonazepam and diazepam.

LevelofEvidence:B.

Degreeofrecommendation:recommended.

Non-supressorvestibularmedications

Theyareindicatedforsymptomcontrolandprophylaxisof newcrisesofvertigo.Themainmedicationsinthisclassand theirmechanismsofactionare:

Betahistine: itis ahistaminergic modulatoracting asa weakH1agonistandastrongH3heteroreceptor antago-nist.Itsactioninthelabyrinthinvolvesmechanismsthat facilitate fluidcirculation in thestria vascularisvia the precapillary sphincter, thereby reducing endolymphatic pressure.13 Itsaction on the CNS occurs by facilitating

vestibular compensation andreducing bioelectric activ-ity in the vestibular nuclei, with the latter being dose dependent.13,14

LevelofEvidence:A.

Degreeofrecommendation:recommended.

GinkgobilobaEGb761Extract:itisanherbalmedicine, consisting of two active fractions: flavonoids and

ter-penoids.Ithasantiplatelet,antioxidant,antihypoxemic, anti-freeradical,andantiedemaactionsinboththe cen-tralnervoussystemandtheinnerear.15,16

LevelofEvidence:B.

Degreeofrecommendation:recommended.

Antidepressants. They are used for the prophylaxis of

vestibularmigrainecrisesandMeniere’sdiseaseand treat-mentofPPPD.The startof thetherapyshouldbegradual toassess possible adversereactions and treatmentshould becontinuedforatleast6monthsaftersymptomcontrol. Withdrawal,whenindicated,shouldalsobegradualtoavoid withdrawalreactionsbythepatient.

There are at least seven types of neurotransmit-ters involved in the transmission of vestibular system impulses. The top seven are glutamate, acetylcholine, GABA,dopamine,norepinephrine,histamineandserotonin. Mostantidepressant medications work by modulating the action of serotonin. Serotonin receptors are found in the peripheral vestibular system and the vestibular nucleus inthe central nervoussystem.17 The antidepressantsthat

inhibittheselectiveserotonin reuptakemayreduce tinni-tussymptomsthroughdirectinhibitionofelectricalimpulse transmission.18

Listedbelow aretherecommendations ofeach antide-pressantaccordingtothediagnosis:

Prophylaxis of vestibular migraine crises:

Nortripty-line(C,recommended),Amitriptyline(C,recommended),

Venlafaxine(B,recommended).

ProphylaxisofMeniere’ssyndromecrises:Sertraline(D,

optional),Escitalopram(C,recommended).

Treatmentofpersistentpostural-perceptualdizziness:

Sertraline (C, recommended), Paroxetine (C,

recom-mended), Imipramine (C, recommended), Fluvoxamine

(C,recommended),Milnacipran (C,recommended),

Flu-oxetine(D,optional),Citalopram(D,optional).

Anticonvulsants

Theyareusedinthetreatmentofvestibularparoxysmsand prophylaxisofvestibularmigrainecrisesandinthecontrol oftinnitus.The start of the therapy shouldbegradual to assesspossible adverse reactions. Withdrawal,when indi-cated,shouldalsobegradualtoavoidwithdrawalreactions bythepatient.

Anticonvulsantsareclassified accordingtotheir siteof action and potency: enhancers of GABA-mediated synap-ticinhibition(inhibitGABA-degradingtransaminase,or are directGABAergicagonists),sodiumchannelblockers(reduce electrical excitability of cell membranes), calcium chan-nelblockers(actinmaintainingelectricalfiring),inhibitors of glutamate synaptic receptors (lower the excitability threshold).The most commonly usedare carbamazepine, oxcarbazepine,topiramate,valproate,gabapentinand lam-otrigine.

Anticonvulsants with their degree of recommendation, accordingtodiagnosis,arelistedbelow:19,20

Treatment of vestibular paroxysm: Carbamazepine

(C, recommended), Oxcarbazepine (C, recommended),

Gabapentin (D, optional), Phenytoin (D, optional), Val-proicAcid(C,recommended).

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Fig.1 EpleyManeuverSequencebeginningattherightear.26

Prophylaxis of vestibular migraine crises: Topiramate

(C, recommended), Valproate (D, optional),

carba-mazepine (D, optional), Gabapentin (D, optional),

Lamotrigine(D,optional).

Othermedicationsactinginvestibulardiseases

Theyhelpcontrolvestibularsymptoms,fluctuatinghearing, auralfullnessandtinnitusinpatientswithMeniere’sdisease. • Diuretics: theycanbe usedtotreatMeniere’s disease, with cases being assessed individually. Although sev-eral experimental and radiological studies demonstrate the reversal of hydrops with the use of diuretics, the mechanism of action of thesemedications is stillmuch discussed.21Themoststudiedmedicationsare

chlorthali-done, which is a thiazidediuretic, acting onthe distal tubule22,23andacetazolamide,whichisacarbonic

anhy-draseinhibitor.24

LevelofEvidence:C.

Degreeofrecommendation:optional.

Proceduresinneurotology

Otolithrepositioningmaneuvers

These are distinct therapeutic procedures for vestibu-larrehabilitationexercises.Whilerehabilitationexercises, suchasCawthorne-CookseyorBrandt-Daroffprotocols,aim at mechanisms of neuronal plasticitysuch as adaptation, habituationandsensorysubstitution,otolithsrepositioning maneuversaimtoeffectivelyreleaseandrepositionotoliths

outoftheendolymphaticductandampouleandbacktotheir physiologicalutricularposition.Theyshouldbecarriedout bythedoctortoresolvethepositionalvestibularsymptoms inBPPV.

LevelofEvidence:A.

Degreeofrecommendation:strong.

Maneuvers for the posterior canal:25 there are two

main therapeutic maneuvers for posterior canal BPPV: theEpleymaneuverandtheSemontmaneuver.InEpley maneuver, the patient is placed in the Dix Hallpike diagnostic positionand remains so until nystagmusand dizzinesssubside,for1−2min.Theheadisslowlyrotated 90◦ tothe opposite side,being heldin thispositionfor 1−2min more.The bodyis rotatedtothelateral decu-bitusposition,followedbya90-degreemovementofthe headuntilthenose pointstothegroundata45-degree angle from the ground plane. This position is held for 30---60 seconds, and then the patient is asked toplace their chin on their chest and to sit slowly. The head remainslow for amomentbeforereturning tothe nor-mal position.The sequence of movements can beseen in Fig. 1. The Semont maneuver is indicated for the treatmentofcupulolithiasis oftheverticalcanal.When theposterior canalsareinvolved, thepatientisinitially seatedwiththelegshangingandthenmovedtothe lat-eraldecubituspositionoftheaffectedside,withthehead forminga45◦anglewiththestretcher.Nystagmusand/or vertigooccur,andthepositionismaintainedfor1−3min. Theexaminerholdsthepatient’sheadandneck,moving itquicklytowardtheothersideofthestretcher.Fromthe

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Fig.2 Semontreleasing maneuvertowardstheleft for the treatmentofrightposteriorcanalcupulolithiasis.26

beginningtotheendofthetrajectory,theheadiskeptin thesamepositionuntilitreachestheoppositeside,when thepatienttouchesthestretcherwiththeirforeheadand thenose(Fig.2).Inthecaseofthesuperiorcanals,the movementisperformedintheoppositedirectiontothat usedforthemaneuveroftheposteriorcanals.

Maneuvers for the lateral canal - canalithiasis (geotropic variant):27 thereare3 main particle

reposi-tioning methods for treating the lateral canal BPPV in the geotropic variant: the Lempert maneuver, forced and prolonged positioning, and the Gufoni maneuver. The Lempertmaneuver isthemostusedatthemoment of thebibliographicsurveyforthis work.Thepatientis madetolieinthesupineposition,thenrotateswiththe head turned 45degreestowards theaffected side.The patientisthentakenthroughaseriesof90-degreesteps towards theunaffected side remainingin each position for 10---30 seconds, completing a 360◦ turn andreturns to the supine position in preparation for a rapid and simultaneous face-up movement tothe sitting position (Fig.3).

Maneuvers forthelateralcanal -cupulolithiasis (apo-geotropicvariant):28,29thereistheGufonimaneuverand,

published more recently, the Zuma maneuver, usedfor boththerepositioningoftheparticleslocatedinthedome nexttoutricleonthecanalsideorintheanteriorarmof thesemicircularcanal.

Maneuvers for theanterior canal: thesewere not dis-cussed intheforum sinceanteriorcanalBPPVisa very rare and uncertain diagnosis and itspathophysiology is notwellunderstood.30

Complications of repositioning maneuvers: severe complicationsarerarebutshouldnotbeunderestimated. Observationofthepatient’s riskfactors,andtheuseof appropriatetechniques helptopreventthem, andtheir performance undermedicalsupervision isnecessary for thetreatment.Themaincomplicationsareseverevertigo

crisis,withnausea,vomitingandsweating,withfrequent

needformedication,conversiontoanothersemicircular

canal(themostcommonbeingtheconversionofthe

pos-terior semicircularcanal tothelateralcanal),requiring anadequatediagnosisofthenewaffectedcanalwith indi-cationofanothertherapeuticmaneuver,intracanalicular

otolith obstruction (canalith jam) requiring reversal of

position,osteoarticularandvascularcervicallesions,and endolymphatichydrops.31,32

Intratympanicinjections

Used tocontrol vestibular symptoms in Meniere’s disease and to recover rapidly progressive sensorineural hearing loss.Gentamicinapplicationisreservedfor thetreatment of Meniere’s disease unresponsive to clinical treatment. Becauseofthepossibilityofhearingloss,cochlearfunction should be monitored. Intratympanic corticosteroid ther-apy can be used for sudden sensorineural hearing loss, immune-mediatedinnereardiseases, andrapidly progres-sive sensorineural hearing loss, as primary, combined, or rescuetherapytooralcorticosteroidtherapy.33,34

Level of evidence: B (vertigo control) and C (hearing loss).

Degreeofrecommendation:recommended.

Vestibularrehabilitation

Itisaphysiologicalformoftreatmentofperipheraland/or centralvestibularsymptoms,allowing patientstoperform their usual activities tothe best of their capacities. It is basedoneye,headandbodyexerciseprotocols.The exer-cisesusevestibularadaptationmechanisms,substitutionof sensoryor motorstrategies andhabituation toaccelerate vestibularcompensationandregainbodybalance.35,36

Traditional: vestibular adaptation exercises intensify vestibular-ocularreflexgainandtolerancetohead move-ments,improvegazestability,vestibulo-visualinteraction duringheadmovements,andposturalstabilityin conflict-ingsensoryenvironments.Sensorysubstitutionexercises seektointensifyresidualvestibularfunctionandreplace thereducedorabsentvestibularfunctionwithalternative strategies of gazestabilization and static and dynamic posturalcontrol.Ontheotherhand,habituationexercises aimtodesensitizethemovementsand/orpositionsthat triggervestibularsymptomsthroughrepetitivestimuli.

LevelofEvidence:A.

Degreeofrecommendation:strong.

With platforms: the use of platforms may be a ther-apeutic option included in the vestibular rehabilitation protocol. Platforms are equipment where the individ-ual remains in an upright position, being submitted to body stability challenges caused by movements in the mid-lateral or anteroposterior direction or with flexion and extension of the ankle. There are studies showing moresignificant improvementintheDizzinessHandicap Inventory(DHI)andbodyswayscoresinpatientstreated withtheplatform,comparedtoconventional vestibular rehabilitation.37 However,otherstudiesfoundno

signifi-cantdifferencebetweenthetwotreatments.38

LevelofEvidence:C.

Degreeofrecommendation:recommended.

Vibrotactilesubstitution:theuseofvibrotactile substi-tution equipmentmaybea therapeuticoption included inthevestibularrehabilitationprotocol.Itisatechnique that accelerates centralcompensation through vestibu-lar rehabilitation by substitution. It consists of placing a vibrating device on the patient, most commonly an

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Fig.3 Lempertmaneuverforrightlateralcanaltreatment.26

adjustable waist belt, which consists of a main unit, responsible for detecting movement sways, and four vibratory units, located in the anterior,posterior, right lateral and left lateral positions,39 which vibrate when

there is inadequate body deviation and provide better posturalcorrection.40,41

LevelofEvidence:B.

Degreeofrecommendation:recommended.

VirtualReality:aimstorecreateenvironmentalchanges by stimulating sensory systems to adjust the reflexes involved in posturalcontrol andbalance strategies.42 It

utilizes virtual reality devicesthatenableimmersionin an illusoryworld,where theperception ofthe environ-mentismodifiedbyanartificialsensorystimulus,which can causeavestibulo-visualconflict andchange inVOR gain.42Comparedtotheconventionalvestibular

rehabili-tation(CawthorneandCookseyexercises),virtualreality rehabilitationshowed earlierimprovementresults, con-sidering scores such as DHI, visual analog scale, and computerizedposturography,inadditiontothelower fre-quencyofsessions.43

LevelofEvidence:B.

Degreeofrecommendation:recommended.

Neuromodulation:vestibularrehabilitationthrough neu-romodulation may be performed in patients with peripheral or central chronic vestibulardysfunction for atleast1yearwhohaveperformedandcompleted previ-ousvestibularrehabilitationtherapyprotocolwithlimited results,andwhosesymptomsandlimitationstoperform

activities ofdaily living stillpersist.44 Neuromodulation

promotesneuralmodulationbydirectelectricalor chem-ical stimulation in neural circuits in the brain, spinal cord, and peripheral nerves, restoring neural balance. It uses a portable device that induces neuroplasticity through noninvasive electrical stimulation of 4 cranial nerves:trigeminal,facial,glossopharyngealand hypoglos-sal. It is capable of neuromodulating subcortical areas ofrestrictedaccessibility,includingbrainstemand cere-bellum. The device shouldbe placed andsupported on the tongue; it generates electrical stimulation on the dorsal surface of the tongue and reaches receptors at adepth of 200---400 micronsbelow theepithelium. Sig-nificant improvement before and after treatment has beendemonstratedinthefollowingmeasurement varia-bles:DynamicGaitIndex(DGI),Activities-specificBalance Confidence Scale (ABC), Dizziness Handicap Inventory (DHI),andSensoryOrganizationTest(SOT).45

LevelofEvidence:B.

Degreeofrecommendation:recommended.

Surgeriesforvestibulardiseases

Labyrinthectomyandvestibularneurectomy:46

Proceduresthatmaybeusedinselectedcasesofdisabling vertigo refractory to clinical treatment. Labyrinthectomy is limited topatients with socially useless hearing, while vestibularneurectomyisoneoftheoptionsforpatientswith usefulhearing.

LevelofEvidence:C.

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Endolymphaticsacdecompression:47,48

The purposeof the procedure is the symptomatic control ofpatientswhoarerefractorytotheclinicaltreatmentof Meniere’s disease. However, no class or scientific society was found that adopted a recommendation for this sub-ject.The procedurebecomesanindividualoption ofeach professional,consideringthecharacteristicsofthecase.

LevelofEvidence:C.

Degreeofrecommendation:optional.

Occlusionofanteriorsemicircularcanaldehiscence

Itisatreatmentoptionforthecontrolofvertigosymptoms andisonlyindicated forpatientswithdisablingsymptoms ofsuperiorsemicircularcanaldehiscence(SSCD)syndrome. Thereisstillnoevidencetodeterminethemosteffective surgicaltechnique.Asystematicreviewpublishedin201749

concludedthatthesurgicaltreatmentofSSCDSyndromehas moderateeffectivenessfordisablingvertigocrises.

LevelofEvidence:C.

Degreeofrecommendation:optional.

Fistulaocclusion:50

Itisasafesurgicaltreatmentindicatedforthemanagement ofpatientswithperilymphaticfistula,aslongasitsdiagnosis isestablished.

LevelofEvidence:C.

Degreeofrecommendation:recommended.

Conclusion

The large amount of informationpresented in thisarticle is a demonstration of the advancement of neurotologyin recentyears.The authorshope thatthis reviewmayhelp the physicianto understand, approach andtreat patients withvestibularsymptoms.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest

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