impact of congenital bilateral vestibular loss (cbvl) on

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

0

2

4

6

8

10

12

14

16

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)