Download 13th International Conference on Cochlear Implants and Other

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CHARGE syndrome and paediatric cochlear implant outcomes
Birman C. , Gibson W. , Elliott E.
University of Sydney, Sydney Medical School, Children's Hospital at Westmead, Department of ENT, SCIC, Sydney, Australia, 2Macquarie
University, Sydney, Australia, 3Sydney University, Sydney Medical School, SCIC, Sydney, Australia, 4Sydney University, Sydney Medical
School, Department of Paediatrics and Child Health, Children's Hosptial at Westmead, Sydney, Australia
Introduction: Charge syndrome is a complex cluster of congenital abnormalities, which can impact on cochlear
implant outcomes. Associated factors include hearing loss, abnormal inner ear anatomy, developmental delay
and cranial nerve hypoplasias. These children may have absent or hypoplastic auditory nerves.
Method: The Sydney Cochlear Implant Centre (SCIC) data were searched for cochlear implant children with
CHARGE syndrome, aged 16 and less. Data were collected regarding clinical history; hearing assessments; MRI
and CT scans; transtympanic electrical Auditory Brainstem Response; intraoperative findings; and language
outcomes in terms of main language used and Categories of Auditory Performance scores (0-7 ranking).
Results: Nine children were identified. All six prelingual profoundly deaf children had hypoplastic or absent
auditory nerves bilaterally on MRI scanning. Intraoperative anatomy and surgery was difficult in half of these
cases. Sign language was used as the main mode of communication in all of these children. Two, implanted
early, did have spoken language also. CAP scores ranged from 0-5. The three children with progressive hearing
loss had better auditory nerve findings on MRI scan. All preoperatively only used verbal language and continued
to with their cochlear implants, with CAP scores of 6.
Conclusion: Congenital profound hearing loss in children with CHARGE syndrome is associated with
hypoplasia or absent auditory nerves, affecting outcomes with cochlear implants. These children do best with a
bilingual early intervention, using sign language and verbal language, to ensure best language outcomes of the
children. Progressive hearing loss CHARGE syndrome children did well with cochlear implants.