Table Of ContentHANDBOOK OF CLINICAL
NEUROLOGY
Series Editors
MICHAEL J. AMINOFF, FRANÇOIS BOLLER, AND DICK F. SWAAB
VOLUME 137
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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]
