Outcomes of Cochlear Implanta3on in Children with CHARGE Syndrome Meredith A. Holcomb, AuD, CCC-A Medical University of South Carolina Clinical Assistant Professor, Department of OTO-HNS Clinical Director, Cochlear Implant Program CI2016 – InternaJonal Cochlear Implant Symposium Toronto, ON May 13, 2016
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OutcomesofCochlearImplanta3oninChildrenwithCHARGESyndrome
MeredithA.Holcomb,AuD,CCC-AMedicalUniversityofSouthCarolina
ClinicalAssistantProfessor,DepartmentofOTO-HNSClinicalDirector,CochlearImplantProgram
CI2016–InternaJonalCochlearImplantSymposium
Toronto,ONMay13,2016
Authors&Disclosures
• MeredithHolcomb,AuD– AdvancedBionicsAudiologyAdvisoryBoard– MedElCorporaJon:2ResearchGrants
• TedMeyer,MD,PhD– PresidentElect,NaJonalAGBell
• DavidWhite,MD– MedElPediatricSurgicalAdvisoryBoard
• TedMcRackan,MD
ObjecJve
• Todescribethesurgical,audiometric,andlanguageoutcomesofcochlearimplantaJoninchildrenwithCHARGEsyndrome
Study Design/Methods• IRBapprovedretrospecJvechartreviewofchildrenwithCHARGEsyndrome
whoreceivedacochlearimplantattheMedicalUniversityofSouthCarolinabetween2001-2016
• Dataanalyzed:– DiagnosJcABRresults– MRIand/orCTscanresults– Pre-operaJveaudiogram– Intra-operaJveECAP(NRT/NRI/ART)– Post-operaJveECAP(NRT/NRI/ART)– Post-operaJveCIaudiogram– Languageoutcomes
• 5children(10ears)idenJfiedforinclusioninthisstudy
Subject#1Ear ABR Audio Aided
AudioAbnormalCT MRI CI OR
NRTPost-opNRT
Post-opAudio
Right NR NR NR X Absent8th
Le` NR NR NR X Deficient8th
X NR NR NR
AgeatCI:22mosDevice:CAN5SurgicalComplicaJons:NoneCIOutcome:Non-userLanguageOutcome:ASL
Subject#2Ear ABR Audio Aided
AudioAbnormalCT MRI CI OR
NRIPost-opNRI
Post-opAudio
Right 90dB@4kHz
NR NR X Deficient8th
X Present Present 25-30dBHL
Le` NR NR NR X Absent8th
Age at CI: 51 mos Device: AB HR90k Surgical Complications: Yes CI Outcome: Consistent User Language Outcome: ASL (Autism/ADHD)
Subject#3Ear ABR Audio Aided
AudioAbnormalCT MRI CI OR
ARTPost-opART
Post-opAudio
Right NR NR NR X Deficient8th
X NR NR ?
Le` NR NR NR X Absent8th
AgeatCI:21mosDevice:MEConcertFlex24SurgicalComplicaJons:NoneCIOutcome:?LanguageOutcome:?
Subject#4Ear ABR Audio Aided
AudioAbnormalCT MRI CI OR
NRTPost-opNRT
Post-opAudio
Right NR NR NR X Deficient8th
X NR NR NR
Le` NR NR NR X Absent8th
AgeatCI:27mosDevice:CACI24RE-CASurgicalComplicaJons:NoneCIOutcome:Non-userLanguageOutcome:ASL
Subject#5Ear ABR Audio Aided
AudioAbnormalCT MRI CI OR
NRTPost-opNRT
Post-opAudio
Right ModtoprofSNHL
ModtoprofSNHL
20-60dBHL
X Present8th
X present present 15-20dBHL
Le` NR NR NR X Deficient8th
AgeatCI:60mosDevice:CACI24RE-CASurgicalComplicaJons:NoneCIOutcome:ConsistentuserLanguageOutcome:Oral
OverallResults
• 8of10totalears(80%)–– NoresponseonABRtesJng– hypoplasJcorabsentauditorynervesonMRIscans– Ofthese,4/4chosenforimplantaJondidnotsJmulatewiththeCI
• FullinserJonofinternalCIdevicewasachievedinallfiveimplantaJons,although1wascomplicated.
OverallResults
• 4ofthe5paJents(80%)usedASLastheirmainmodeofcommunicaJon,andonechild(20%)wasanoralcommunicatorwithspeechandlanguagescoresinthenormalrange.
• 2paJentsarenon-usersofCI,2paJentsareconsistentusers,and1islosttofollow-up.– Forthenon-users,pre-opABRwasnoresponse,MRIrevealedhypoplasJcorabsentauditorynerves,andintra-operaJveandpost-operaJveECAPresponseswereabsent.
Conclusions
• CochlearimplantaJonisanappropriateopJonoftreatmentofprofoundSNHLinchildrenwithCHARGEsyndrome.– MRIisnecessarytoevaluatestatusofauditorynervesforthispopulaJon.
– Outcomeslimitedbystatusofcochlearnerve.
• AnoresponseABRmaypredictpooroutcomeswithCIforchildrenwithCHARGEsyndrome.
ThankYou!
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