UK and Ireland experience of cochlear implants in children with Down Syndrome

ObjectivesDown Syndrome (DS) is associated with a high incidence of hearing loss. The majority of hearing loss is conductive, but between 4 and 20% is sensorineural, which in the main is mild or moderate and is managed with conventional behind-the-ear hearing aids. Cochlear implantation is an elective invasive procedure, performed to provide some form of hearing rehabilitation in individuals with severe to profound sensorineural hearing loss, and initially candidacy criteria were strict—excluding patients with additional disabilities. With good results and expanding experience, more candidates with additional disabilities are now being implanted. A survey of UK and Ireland Cochlear Implant Programmes sought to identify the number of individuals with DS who have been implanted with a cochlear implant (CI) and to provide relevant information on outcomes of implantation in these individuals.MethodsE-mail survey of all programmes within the British Cochlear Implant Group (BCIG). Postal questionnaire to programmes identified to have implanted a child with Down Syndrome, with data collection on pre-operative assessment, surgical and post-operative outcomes. Case series review.ResultsThree of 23 BCIG programmes have implanted a child with Down Syndrome. Four children have received implants. No intraoperative or post-operative surgical complications were encountered. All children had middle ear disease, but no problems with implantation were associated with their middle ear condition. All children remain implant users, 12 months to 4 years post-implantation.ConclusionCochlear implantation is an option for a child with Down Syndrome and associated severe to profound sensorineural hearing loss. Clinicians caring for these children and their families should consider referral for assessment by a Cochlear Implant Programme.