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Cochlear Implantation in Children With CHARGE Syndrome: Therapeutic Decisions and Outcomes
Author(s) -
Lanson Biana G.,
Green Janet E.,
Roland J Thomas,
Lalwani Anil K.,
Waltzman Susan B.
Publication year - 2007
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1097/mlg.0b013e31806009c9
Subject(s) - charge syndrome , choanal atresia , medicine , coloboma , sensorineural hearing loss , audiology , hearing loss , atresia , cochlear implant , pediatrics , cochlear implantation , surgery , ophthalmology , psychiatry
Objectives: 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. Study Design: 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.