Neuro-Otology PDF
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HANDBOOK OF CLINICAL NEUROLOGY Series Editors MICHAEL J. AMINOFF, FRANÇOIS BOLLER, AND DICK F. SWAAB VOLUME 137 AMSTERDAM BOSTON HEIDELBERG LONDON NEW YORK OXFORD PARIS SAN DIEGO SAN FRANCISCO SINGAPORE SYDNEY TOKYO ELSEVIER Radarweg29,POBox211,1000AEAmsterdam,Netherlands TheBoulevard,LangfordLane,Kidlington,OxfordOX51GB,UnitedKingdom 50HampshireStreet,5thFloor,Cambridge,MA02139,UnitedStates ©2016ElsevierB.V.Allrightsreserved. Nopartofthispublicationmaybereproducedortransmittedinanyformorbyanymeans,electronicormechanical, includingphotocopying,recording,oranyinformationstorageandretrievalsystem,withoutpermissioninwriting fromthepublisher.Detailsonhowtoseekpermission,furtherinformationaboutthePublisher’spermissions policiesandourarrangementswithorganizationssuchastheCopyrightClearanceCenterandtheCopyright LicensingAgency,canbefoundatourwebsite:www.elsevier.com/permissions. 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BritishLibraryCataloguing-in-PublicationData AcataloguerecordforthisbookisavailablefromtheBritishLibrary LibraryofCongressCataloging-in-PublicationData AcatalogrecordforthisbookisavailablefromtheLibraryofCongress ISBN:978-0-444-63437-5 ForinformationonallElsevierpublications visitourwebsiteathttps://www.elsevier.com/ Publisher:ShirleyDecker-lucke AcquisitionEditor:MaraConner EditorialProjectManager:KristiAnderson ProductionProjectManager:SujathaThirugnanaSambandam CoverDesigner:AlanStudholme TypesetbySPiGlobal,India HandbookofClinicalNeurology3rdSeries Availabletitles Vol.79,Thehumanhypothalamus:basicandclinicalaspects,PartI,D.F.Swaab,ed.ISBN9780444513571 Vol.80,Thehumanhypothalamus:basicandclinicalaspects,PartII,D.F.Swaab,ed.ISBN9780444514905 Vol.81,Pain,F.CerveroandT.S.Jensen,eds.ISBN9780444519016 Vol.82,Motorneuronedisordersandrelateddiseases,A.A.EisenandP.J.Shaw,eds.ISBN9780444518941 Vol.83,Parkinson’sdiseaseandrelateddisorders,PartI,W.C.KollerandE.Melamed,eds.ISBN9780444519009 Vol.84,Parkinson’sdiseaseandrelateddisorders,PartII,W.C.KollerandE.Melamed,eds.ISBN9780444528933 Vol.85,HIV/AIDSandthenervoussystem,P.PortegiesandJ.Berger,eds.ISBN9780444520104 Vol.86,Myopathies,F.L.MastagliaandD.HiltonJones,eds.ISBN9780444518996 Vol.87,Malformationsofthenervoussystem,H.B.SarnatandP.Curatolo,eds.ISBN9780444518965 Vol.88,Neuropsychologyandbehaviouralneurology,G.GoldenbergandB.C.Miller,eds.ISBN9780444518972 Vol.89,Dementias,C.DuyckaertsandI.Litvan,eds.ISBN9780444518989 Vol.90,Disordersofconsciousness,G.B.YoungandE.F.M.Wijdicks,eds.ISBN9780444518958 Vol.91,Neuromuscularjunctiondisorders,A.G.Engel,ed.ISBN9780444520081 Vol.92,Stroke–PartI:Basicandepidemiologicalaspects,M.Fisher,ed.ISBN9780444520036 Vol.93,Stroke–PartII:Clinicalmanifestationsandpathogenesis,M.Fisher,ed.ISBN9780444520043 Vol.94,Stroke–PartIII:Investigationsandmanagement,M.Fisher,ed.ISBN9780444520050 Vol.95,Historyofneurology,S.Finger,F.BollerandK.L.Tyler,eds.ISBN9780444520081 Vol.96,Bacterialinfectionsofthecentralnervoussystem,K.L.RoosandA.R.Tunkel,eds.ISBN9780444520159 Vol.97,Headache,G.NappiandM.A.Moskowitz,eds.ISBN9780444521392 Vol.98,SleepdisordersPartI,P.MontagnaandS.Chokroverty,eds.ISBN9780444520067 Vol.99,SleepdisordersPartII,P.MontagnaandS.Chokroverty,eds.ISBN9780444520074 Vol.100,Hyperkineticmovementdisorders,W.J.WeinerandE.Tolosa,eds.ISBN9780444520142 Vol.101,Musculardystrophies,A.AmatoandR.C.Griggs,eds.ISBN9780080450315 Vol.102,Neuro-ophthalmology,C.KennardandR.J.Leigh,eds.ISBN9780444529039 Vol.103,Ataxicdisorders,S.H.SubramonyandA.Durr,eds.ISBN9780444518927 Vol.104,Neuro-oncologyPartI,W.GrisoldandR.Sofietti,eds.ISBN9780444521385 Vol.105,Neuro-oncologyPartII,W.GrisoldandR.Sofietti,eds.ISBN9780444535023 Vol.106,Neurobiologyofpsychiatricdisorders,T.SchlaepferandC.B.Nemeroff,eds.ISBN9780444520029 Vol.107,EpilepsyPartI,H.StefanandW.H.Theodore,eds.ISBN9780444528988 Vol.108,EpilepsyPartII,H.StefanandW.H.Theodore,eds.ISBN9780444528995 Vol.109,Spinalcordinjury,J.VerhaagenandJ.W.McDonaldIII,eds.ISBN9780444521378 Vol.110,Neurologicalrehabilitation,M.BarnesandD.C.Good,eds.ISBN9780444529015 Vol.111,PediatricneurologyPartI,O.Dulac,M.LassondeandH.B.Sarnat,eds.ISBN9780444528919 Vol.112,PediatricneurologyPartII,O.Dulac,M.LassondeandH.B.Sarnat,eds.ISBN9780444529107 Vol.113,PediatricneurologyPartIII,O.Dulac,M.LassondeandH.B.Sarnat,eds.ISBN9780444595652 Vol.114,Neuroparasitologyandtropicalneurology,H.H.Garcia,H.B.TanowitzandO.H.DelBrutto,eds. ISBN9780444534903 Vol.115,Peripheralnervedisorders,G.SaidandC.Krarup,eds.ISBN9780444529022 Vol.116,Brainstimulation,A.M.LozanoandM.Hallett,eds.ISBN9780444534972 Vol.117,Autonomicnervoussystem,R.M.BuijsandD.F.Swaab,eds.ISBN9780444534910 Vol.118,Ethicalandlegalissuesinneurology,J.L.BernatandH.R.Beresford,eds.ISBN9780444535016 Vol.119,NeurologicaspectsofsystemicdiseasePartI,J.BillerandJ.M.Ferro,eds.ISBN9780702040863 Vol.120,NeurologicaspectsofsystemicdiseasePartII,J.BillerandJ.M.Ferro,eds.ISBN9780702040870 Vol.121,NeurologicaspectsofsystemicdiseasePartIII,J.BillerandJ.M.Ferro,eds.ISBN9780702040887 Vol.122,Multiplesclerosisandrelateddisorders,D.S.Goodin,ed.ISBN9780444520012 Vol.123,Neurovirology,A.C.TselisandJ.Booss,eds.ISBN9780444534880 vi AVAILABLETITLES(Continued) Vol.124,Clinicalneuroendocrinology,E.Fliers,M.KorbonitsandJ.A.Romijn,eds.ISBN9780444596024 Vol.125,Alcoholandthenervoussystem,E.V.SullivanandA.Pfefferbaum,eds.ISBN9780444626196 Vol.126,Diabetesandthenervoussystem,D.W.ZochodneandR.A.Malik,eds.ISBN9780444534804 Vol.127,TraumaticbraininjuryPartI,J.H.GrafmanandA.M.Salazar,eds.ISBN9780444528926 Vol.128,TraumaticbraininjuryPartII,J.H.GrafmanandA.M.Salazar,eds.ISBN9780444635211 Vol.129,Thehumanauditorysystem:Fundamentalorganizationandclinicaldisorders,G.G.Celesia andG.Hickok,eds.ISBN9780444626301 Vol.130,Neurologyofsexualandbladderdisorders,D.B.VodusˇekandF.Boller,eds.ISBN9780444632470 Vol.131,Occupationalneurology,M.LottiandM.L.Bleecker,eds.ISBN9780444626271 Vol.132,Neurocutaneoussyndromes,M.P.IslamandE.S.Roach,eds.ISBN9780444627025 Vol.133,Autoimmuneneurology,S.J.PittockandA.Vincent,eds.ISBN9780444634320 Vol.134,Gliomas,M.S.BergerandM.Weller,eds.ISBN9780128029978 Vol.135,NeuroimagingPartI,J.C.MasdeuandR.G.Gonza´lez,eds.ISBN9780444534859 Vol.136,NeuroimagingPartII,J.C.MasdeuandR.G.Gonza´lez,eds.ISBN9780444534866 Foreword ThisisthefirstvolumeintheHandbookofClinicalNeurologyseriesdevotedentirelytoneuro-otology,arelatively youngbranchofclinicalmedicinethatstudiesandtreatsneurologicdisordersoftheear,eighthcranialnerve,andasso- ciatedcentralpathways,leadingtosymptomssuchasvertigo,dizziness,andhearingdisorders. Herman Kingma and Raymond van de Berg start their chapter on the anatomy, physiology, and physics of the peripheralvestibularsystembyquotingWilsonandMelvill-Jones’prefacetothefamousbook,MammalianVestibular Physiology(1979):“Itiseasytounderratetheimportanceofasensorysystemwhosereceptorisburieddeepwithinthe skullandofwhoseperformanceweareusuallynotaware”.Afterreadingthisneuro-otologyvolume,itisnolonger possibletodenytheimportanceofthiscomplexsystemforournormaldailyactivitiesandthegreatimpactofuni-or bilateralvestibularloss. We were very fortunate to have as volume editors two distinguished scholars, Joseph M. Furman from the Department of Otolaryngology, University of Pittsburgh, Pittsburgh, PA, USA and Thomas Lempert from the DepartmentofNeurology,Schlosspark-KlinikandVestibularResearchGroup,Berlin,Germany.Theyhaveassem- bled an excellent, international, and multidisciplinary group of experts and guided them firmly to create this com- prehensivebook.Wearegratefultothemandtoallthecontributors. Thisvolumewillnotonlybeofinteresttoclinicalneurologists.Partsofitwillappealalsotopsychiatrists(e.g.,the chapteronfunctionalandpsychiatricvestibulardisorders)andpediatricians(e.g.,thechapteronvertigoanddizziness inchildren).Moreover,thefirstpartofthisvolume,whichdiscussestheanatomy,physiology,neurotransmitters,phar- macology,integrationofinformation,andphysicsoftheperipheralandcentralvestibularsystems,willappealtoboth neuro-otologistsandbasicneuroscientistsworkinginthefield. Inadditiontotheprintedversion,thevolumewillbeavailableelectronicallyonElsevier’sScienceDirectwebsite, whichisbecomingincreasinglypopularwithreadersandwillfacilitatethebook’saccessibility.Indeed,allofthevol- umesinthepresentseriesoftheHandbookareavailableelectronicallyonthiswebsite. Asalways,itisapleasuretothankElsevier,ourpublisher–andinparticularMichaelParkinsoninScotlandand KristiAndersonandMaraE.ConnerinSanDiego–fortheirassistanceinthedevelopmentandproductionofthis volume. MichaelJ.Aminoff Franc¸oisBoller DickF.Swaab Preface Vertigoanddizzinessrankamongthemostcommonsymptomsinprimarycare,otolaryngology,andneurology.Causes varyfromharmlessbutbothersomeconditionssuchasbenignparoxysmalpositionalvertigotolife-threateningemer- genciessuchasposteriorfossastrokes.Ourunderstandingofdiagnosis,pathophysiologicmechanisms,andeffective treatmentshasincreasedconsiderablyinthelasttwodecades.Newdevelopmentsincludealgorithmsforbedsidedetec- tionofvestibularstrokes,thedelineationofvestibularmigraineasoneofthemostfrequentcausesofrecurrentvertigo, descriptionofvariantsofbenignparoxysmalpositionalvertigo,devicesfortestingindividualsemicircularcanalsand otolithorgans,andtheadvancementofvestibularrehabilitationasthemostimportanttherapeutictoolinneuro-otology. ThisvolumeoftheHandbookofClinicalNeurologyassemblescontributionsfromleadinginternationalauthorsto communicatethecurrentclinicalknowledgeofneuro-otologyandacomprehensivelistofreferences.Chapters1–14 deal with basic knowledge and general principles of neuro-otology, such as anatomy, physiology, epidemiology, history taking, examination, and vestibular rehabilitation. This is followed by the disease-specific Chapters 15–28, coveringall commoncauses ofvertigo and dizziness. The numerous tables andfiguresinthis book make thefield ofvestibularscienceandmedicineevenmoreaccessible. JosephM.Furman ThomasLempert Contributors A.Alghadir K.-D.Choi DepartmentofRehabilitationSciences,Collegeof DepartmentofNeurology,CollegeofMedicine,Pusan AppliedMedicalSciences,KingSaudUniversity, NationalUniversityHospital,Busan,Korea Riyadh,SaudiArabia J.G.Colebatch A.A.Alghwiri NeuroscienceResearchAustraliaandDepartment DepartmentofPhysicalTherapy,Facultyof ofNeurology,PrinceofWalesHospitalClinical RehabilitationSciences,UniversityofJordan,Amman, School,UniversityofNewSouthWales,Sydney, Jordan Australia C.D.Balaban A.I.Colpak DepartmentsofOtolaryngology,Neurobiology, HacettepeUniversitySchoolofMedicine,Ankara, CommunicationSciencesandDisorders,and Turkey Bioengineering,UniversityofPittsburgh,Pittsburgh, PA,USA K.E.Cullen DepartmentofPhysiology,McGillUniversity,Montreal, P.Bertholon Quebec,Canada DepartmentofOto-Rhino-Laryngology,Centre HospitalierUniversitairedeSaintEtienne,SaintEtienne, France R.A.Davies DepartmentofNeuro-otology,NationalHospitalfor NeurologyandNeurosurgery,London,UK A.Bisdorff DepartmentofNeurology,CentreHospitalierEmile Mayrisch,Esch-sur-Alzette,Luxembourg M.Dieterich GermanCenterforVertigoandBalanceDisordersand T.Brandt DepartmentofNeurology,UniversityHospitalMunich, GermanCenterforVertigoandBalanceDisordersand CampusGrosshadern;andMunichClusterforSystems InstituteforClinicalNeurosciences,UniversityHospital Neurology(SyNergy),Ludwig-MaximiliansUniversity, Munich,CampusGrosshadern,Munich,Germany Munich,Germany A.M.Bronstein J.M.Espinosa-Sanchez Neuro-otologyUnit,ImperialCollegeLondon,Charing OtologyandNeurotologyGroup,Departmentof CrossHospitalandNationalHospitalforNeurologyand GenomicMedicine,CentreforGenomicsand Neurosurgery,London,UK OncologicalResearch(GENYO),Pfizer-Universityof Granada-JuntadeAndalucia,GranadaandDepartment C.Chabbert ofOtolaryngology,HospitalSanAgustin,Linares,Jaen, IntegrativeandAdaptativeNeurosciences,Universityof Spain AixMarseille,Marseille,France K.Feil M.Cherchi GermanCenterforVertigoandBalanceDisordersand DepartmentofNeurology,NorthwesternUniversity DepartmentofNeurology,UniversityHospitalMunich, FeinbergSchoolofMedicine,Chicago,IL,USA CampusGrosshadern,Munich,Germany xii CONTRIBUTORS M.Fetter NationalResearchStateUniversityTomsk,Tomsk, DepartmentofNeurology,SRHKlinikumKarlsbad, RussianFederation Karlsbad,Germany H.Lee J.M.Furman DepartmentofNeurology,KeimyungUniversitySchool DepartmentofOtolaryngology,UniversityofPittsburgh, ofMedicine,Daegu,Korea Pittsburgh,PA,USA T.Lempert J.F.Golding DepartmentofNeurology,Schlosspark-Klinikand DepartmentofPsychology,FacultyofScienceand VestibularResearchGroup,Berlin,Germany Technology,UniversityofWestminster,London,UK J.A.Lopez-Escamez T.C.Hain OtologyandNeurotologyGroup,Departmentof ChicagoDizzinessandHearingandDepartmentof GenomicMedicine,CentreforGenomicsand PhysicaltherapyandHumanMovementSciences, OncologicalResearch(GENYO),Pfizer-Universityof NorthwesternUniversity,Chicago,IL,USA Granada-JuntadeAndaluciaandDepartmentof Otolaryngology,ComplejoHospitalarioUniversitariode M.E.Hoffer Granada,Granada,Spain DepartmentsofOtolaryngologyandNeurological Surgery,MillerSchoolofMedicine,UniversityofMiami andUniversityofMedicineSportsMedicineand M.Magnusson PerformanceInstitute,Miami,FL,USA DepartmentofOtorhinolaryngology,LundUniversity, SkaneUniversityHospital,Lund,Sweden G.P.Jacobson HearingandSpeechSciences,VanderbiltUniversity, E.-M.Malmstr€om Nashville,TN,USA DepartmentsofOtorhinolaryngologyandPain Rehabilitation,LundUniversity,SkaneUniversity K.Jahn Hospital,Lund,Sweden Sch€onKlinikBadAiblingandGermanCenterfor VertigoandBalanceDisorders,Ludwig-Maximilians M.Mandalà UniversityofMunich,Munich,Germany DepartmentofOtologyandSkullBaseSurgery, UniversityofSiena,Siena,Italy A.Karkas DepartmentofOto-Rhino-Laryngology,Centre HospitalierUniversitairedeSaintEtienne,SaintEtienne, M.Masini France DepartmentofSocial,PoliticalandCognitiveSciences, UniversityofSiena,Siena,Italy K.A.Kerber DepartmentofNeurology,UniversityofMichigan S.Murphy HealthSystem,AnnArbor,MI,USA DepartmentofOtolaryngology,MillerSchoolof Medicine,UniversityofMiami,Miami,FL,USA A.Kheradmand DepartmentsofNeurologyandOtolaryngology–Head H.K.Neuhauser andNeckSurgery,JohnsHopkinsHospital,Baltimore, DepartmentofEpidemiologyandHealthMonitoring, MD,USA RobertKochInstitute,Berlin,Germany J.-S.Kim DepartmentofNeurology,SeoulNationalUniversity D.Nuti CollegeofMedicine,SeoulNationalUniversity DepartmentofOtologyandSkullBaseSurgery, BundangHospital,Gyeonggi-do,Korea UniversityofSiena,Siena,Italy H.Kingma S.M.Rosengren DepartmentofOto-Rhino-LaryngologyandHeadand NeurologyDepartment,RoyalPrinceAlfredHospital NeckSurgery,MaastrichtUniversityMedicalCentre, andCentralClinicalSchool,UniversityofSydney, Maastricht,theNetherlandsandFacultyofPhysics, Sydney,Australia CONTRIBUTORS xiii N.T.Shepard R.vandeBerg DepartmentofOtolaryngology,MayoClinic,Rochester, DepartmentofOto-Rhino-LaryngologyandHeadand MN,USA NeckSurgery,MaastrichtUniversityMedicalCentre, Maastricht,theNetherlandsandFacultyofPhysics, NationalResearchStateUniversityTomsk,Tomsk, J.P.Staab RussianFederation DepartmentofPsychiatryandPsychology,and DepartmentofOtorhinolaryngology–HeadandNeck M.vonBrevern Surgery,MayoClinic,Rochester,MN,USA DepartmentofNeurology,Park-KlinikWeissenseeand VestibularResearchGroup,Berlin,Germany D.Straumann InterdisciplinaryCenterforVertigoandNeurological VisionDisorders,DepartmentofNeurology,University M.S.Welgampola HospitalZurich,Zurich,Switzerland InstituteofClinicalNeurosciences,RoyalPrinceAlfred HospitalandCentralClinicalSchool,Universityof M.Strupp Sydney,Sydney,Australia GermanCenterforVertigoandBalanceDisordersand DepartmentofNeurology,UniversityHospitalMunich, S.L.Whitney CampusGrosshadern,Munich,Germany DepartmentofPhysicalTherapy,Universityof Pittsburgh,Pittsburgh,PA,USA M.Szczupak DepartmentofOtolaryngology,MillerSchoolof D.S.Zee Medicine,UniversityofMiamiandUniversityof DepartmentsofNeurology,Otolaryngology–Headand MedicineSportsMedicineandPerformanceInstitute, NeckSurgery,OphthalmologyandNeuroscience,Johns Miami,FL,USA HopkinsHospital,Baltimore,MD,USA HandbookofClinicalNeurology,Vol.137(3rdseries) Neuro-Otology J.M.FurmanandT.Lempert,Editors http://dx.doi.org/10.1016/B978-0-444-63437-5.00001-7 ©2016ElsevierB.V.Allrightsreserved Chapter1 Anatomy, physiology, and physics of the peripheral vestibular system H.KINGMA*ANDR.VANDEBERG DepartmentofOto-Rhino-LaryngologyandHeadandNeckSurgery,MaastrichtUniversityMedicalCentre,Maastricht, theNetherlandsandFacultyofPhysics,NationalResearchStateUniversityTomsk,Tomsk,RussianFederation Abstract Manymedicaldoctorsconsidervertigoanddizzinessasthemajor,almostobligatorycomplaintsinpatients with vestibular disorders. In this chapter, we will explain that vestibular disorders result in much more diverseandcomplexcomplaints.Manyoftheseothercomplaintsareunfortunatelyoftenmisinterpreted andincorrectlyclassifiedaspsychogenic.Whenwereallyunderstandthefunctionofthevestibularsystem, itbecomesquiteobviouswhy patients with vestibulardisorders complainaboutaloss ofvisualacuity, imbalance,fearoffalling,cognitiveandattentionalproblems,fatiguethatpersistsevenwhenthevertigo attacksanddizzinessdecreasesorevendisappears.Anotherinterestingnewaspectinthischapteristhatwe explainwhythefunctionoftheotolithsystemissoimportant,andthatitisamistaketofocusonthefunc- tionofthesemicircularcanalsonly,especiallywhenwewanttounderstandwhysomepatientsseemto suffermorethanothersfromthelossofcanalfunctionasobjectifiedbyreducedcaloricresponses. INTRODUCTION areflexia (thereisnot evena commonwordfor itinlay language)isamajorhandicaplikedeafnessorblindness. Intheirprefacetothebook,MammalianVestibularPhys- Butapparently,symptomsassociatedwithbilateralvestib- iology, published in 1979, the famous vestibular scien- ularareflexiaareoftennotrecognized,leadingtoadelay tists Wilson and Melvill Jones made a perceptive ofmanyyearsbeforeacorrectdiagnosisismade(vande statement: “It is easy to underrate the importance of a Bergetal.,2011;Guinandetal.,2015a;Guyot,2015).The sensory system whose receptor is buried deep within majorreasonisthatthefunctionofthevestibularsystemis the skull and of whose performance we are usually not poorly understood by both doctors and patients. This aware”(WilsonandMelvillJones,1979).Thisstatement unawarenessalsoledtoproblemsinobtainingpermission isstilluptodate,asmanydoctorsareunawareoftherel- todevelopavestibularimplantforhumans,verydifferent evanceofthevestibularsystemindailylifeandalsothink from the development of cochlear implants several that central compensation and sensory substitution decadesago.Onlyafterpublicationofanumberofscien- almost completely deal with vestibular loss and reduce tificarticlesshowingtheimpactandincidenceofsevere complaints to a minimum. Also, in unilateral loss, it is bilateralvestibularlosswasaSwiss–Dutchresearchteam often stated that the healthy labyrinth will take over. allowedtoexecutethefirsthumanvestibularimplantation How absurd such a statement is, becomes clear if we inAugust2012(Pelizzoneetal.,2014;PerezFornosetal., claimthatlosingoneearoroneeyeisofnoimportance 2014;Guinandetal.,2015b). as we can still hear with one ear and see with one eye. Thisallillustrateshowpoorlythefunctionandrele- Losingonevestibularorgan,likelosingoneearoreye, vance of the vestibular system is understood in clinical results in a disturbing asymmetry. Bilateral vestibular practice, andthis iswhat hasmotivatedustowrite this *Correspondenceto:HermanKingma,DepartmentofORLandHeadandNeckSurgery,MaastrichtUniversityMedicalCentre, Maastricht,theNetherlands.E-mail:[email protected]
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