Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes.
OBJECTIVE
Ear anomalies and deafness are associated with CHARGE syndrome, which also presents with a cluster of features including coloboma of the eye, heart defects, atresia of the choanae, developmental retardation, and genitourinary abnormalities. The aim of this study is to explore the viability of cochlear implantation in children with CHARGE syndrome and to assess the outcome.


METHODS
Retrospective chart review.


METHODS
Eleven children presenting with severe to profound sensorineural hearing loss associated with CHARGE syndrome were the subjects of this study. Routine audiometric measurements and the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were performed pre- and postoperatively. In addition, the degree of the subjects' cochlear deformity were measured and correlated to outcome.


RESULTS
All patients had varying degrees of ear anomalies, seven patients suffered from coloboma of the eyes, two had heart defects, five exhibited choanal atresia, eleven showed developmental retardation, and six had genitourinary abnormalities. Ten of the children underwent cochlear implantation with complete insertion of the electrode array without complication and were followed over a 3-month to a 7-year period. The eleventh child was not implanted because of severe retardation. All of the implanted children showed varying, but limited degrees, of auditory benefit as measured by routine audiometry and the IT-MAIS.


CONCLUSIONS
Careful treatment planning for children with sensorineural hearing loss and CHARGE syndrome can lead to varying, but limited degrees, of auditory benefit with no increase in surgical complications. Although the implant enhanced the children's 'connectivity' to the environment, it did not promote the development of oral language skills in this population.
Lanson
Biana G
BG
Department of Otolaryngology, NYU School of Medicine, New York, NY 10016, USA.
Green
Janet E
JE
Roland
J Thomas
JT
Lalwani
Anil K
AK
Waltzman
Susan B
SB
D000015:Abnormalities, Multiple; D002675:Child, Preschool; D002754:Choanal Atresia; D019929:Cochlear Implantation / Q000706:statistics & numerical data; D003103:Coloboma; D002658:Developmental Disabilities / Q000209:etiology*; D004423:Ear / Q000002:abnormalities*; D005260:Female; D005835:Genitalia / Q000002:abnormalities; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000601:surgery*; D006330:Heart Defects, Congenital; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome