Cochlear Implantation in children with CHARGE syndrome

CHARGE syndrome is a rare congenital condition that manifests with anomalies of coloboma, heart defects, choanal atresia, mental retardation, genitourinary and ear anomalies that can affect almost any part of the auditory pathway. In those patients with a significant sensorineural hearing loss, cochlear implantation has become a potential therapeutic option.

Chart review of three cases from the Southern Cochlear Implant Programme.

All patients met clinical diagnostic criteria of CHARGE syndrome, and had abnormal inner ear anatomy with profound sensorineural hearing loss. One child had previously undergone cochlear implantation which was unsuccessful due to increasing non-auditory stimulation. All patients had successful cochlear implantation with full insertion of a Nucleus Freedom Implant with contour Advance. All patients showed improvement in their audiological function; one child has high functioning verbal communication, one child uses both sign and verbal communication with improved speech quality and ability to speech read, and one child responds reliably to sound, understands short phrases and attempts to vocalize, but this is limited by tracheostomy.

Cochlear implantation faces numerous challenges in children with CHARGE syndrome, but with appropriate patient selection can result in successful audiologic and quality of life outcomes.