www.bjorl.org
Brazilian
Journal
of
OTORHINOLARYNGOLOGY
SPECIAL
ARTICLE
Neurotology:
definitions
and
evidence-based
therapies
---
Results
of
the
I
Brazilian
Forum
of
Neurotology
夽
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
yaUniversidadeFederaldeSãoPaulo(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
夽 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/).
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/).
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
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
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).
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
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
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.
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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