impact of congenital bilateral vestibular loss (cbvl) on
TRANSCRIPT
Impactofcongenitalbilateralvestibularloss(CBVL)
oncognitivedevelopmentinchildrenwithprofoundsensorineuralhearingloss
Laboratoired’Explora0onfonc0onnelledel'équilibrechezl'enfant(EFEE)Départementd’ORLetChirurgieCervico-facialeduPrThierryVanDenAbbeeleHôpitalRobertDebré,48BoulevardSérurier,75019Paris,France
CharbelNassif*,DerekHamilton,Sylve9eWienerVacher,BenoitVirole,SolineLecervoisier,SimonaCaldani,RimaObeid
Financialdisclosure
• ResearchGrants:GrandAudiFon/AdvancedBionics
ImpactofCBVL
Gazeandhead-trunkstabilizaFon:*BraswellJ,RineRM.Evidencethatves0bularhypofunc0onaffectsreadingacuityinchildren.IntJPediatrOtorhinolaryngol.2006;70(11):1957‑65.
Posturomotorcontrol:**Wiener-VacherSR,ObeidR,Abou-ElewM.Ves0bularimpairmentaXerbacterialmeningi0sdelaysinfantposturomotordevelopment.JPediatr.2012;161(2):246‑51.
Hypothesis:
The absence of vesHbular informaHon early in life can lead to reduced cogniHveperformancesandspaFalcogniFverepresentaFons.Central compensaHon may not be able to palliate completely the lack of vesHbularinformaHonforcogniHveperformancesonalongterm.
MaterialsandMethods• ProspecFvestudy(childrenre-examined12yearsaXer1stevalua0on)• Comparisonbetween3groups(N=67,childrenbetween9-13yearsold)Inclusioncriteria:• Group1(n=12):CBVL+SNHLdiagnosedbeforetheageof2years• Group2(n=11):SNHL+NormalVesFbularfuncFondiagnosedattheageof2yearsandconfirmedattheageof12years• Group3(n=44):Normalhearingandves0bularfunc0on
Exclusioncriteria:• Associateddisabili0es• Neuropsychiatricdisorders• Congenitalophthalmologicorcraniofacialmalforma0ons• Exclusivesignlanguagecommunica0on
Ba9eryoftestsVisualacuitysta0canddynamic(ac0veandpassiverota0on)(near&far)CogniFvetasks:• WISC-IV(nonverbalsubtests:perceptualreasoning,workingmemory,processingspeed)
• Nepsy-II(visuospa0alprocessing:arrows,blockconst,routefinding…)• VirtualmazeMWT(Morriswatertask)(D.Hamilton)
Results
VisualAcuity
NEPSYTEST&WISCIV
Group 1 :Profound SNHL + Vestibular areflexia Group 2 : Profound SNHL + Normal Vestibular Function Control : Normal hearing + Normal Vestibular Function
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Arrows(10+/-2)
OrientaFon/cartes(rang%)
Copiefigures(10+/-2)
DomainevisuospaFal
(%)/10)
Cubes Mémoirechiffresle9res
Codes Séquencesle9reschiffres
Matrices Symboles TestdeBarrage
Wisc IV NEPSY Test
Pa0entsscore50-75%ofareflexicpaFentswereweaktodeficient
NosignificantstaFsFcaldifferencebetweengroups
VirtualMorriswatertest
Conclusion• CBVLaXer12yearssFllshowseveredropofvisualacuityindynamiccondiFonandlower cogniFve scores compared to children with normal ves0bular func0on (withSNHLornormalhearing).
• These results suggest that CBVL remains a handicap that may never compensatecompletely.
IMPACTONCOCHLEARIMPLANTSTRATEGY:
• 10% risk for complete ves0bular loss aXer unilateral implanta0on, 1 to 2 % risk forbilateralves0bularlossifsimultaneousbilateralimplanta0on*• CompletevesFbularlossshouldbeavoidedaspossibleasaconsequenceofasurgicalprocedure• Bilateral simultaneous implantaFon should be reserved to complete bilateralves0bularlossbeforeCI(meningi0s,Usher..)• Inothercases,weprefersequenFalbilateralimplantaFon(minimum3monthsapart)withvesFbulartesFngbeforeandahereachCI.• Incaseofsymmetricalhearingloss,thefirstCIisdoneonthelessfunc0onalves0bule
*Ves0bularimpairmentspre-andpost-cochlearimplantinchildren.JacotEetal.IntJPediatrOtorhinolaryngol.(2009)