Cochlear implantation in children with keratitis-ichthyosis-deafness (KID) syndrome: outcomes in three cases

Authors

  • Elizabeth J Barker,

    Corresponding author
    1. Department of Otolaryngology, The University of Melbourne, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia
    • School of Audiology, 172 Victoria Pde, East Melbourne 3002, Australia. Phone: +61 3 9667 7506. Fax: +61 3 9662 3312
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  • Robert JS Briggs

    1. Department of Otolaryngology, The University of Melbourne, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia
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Abstract

Three children with keratitis-ichthyosis-deafness (KID) syndrome received cochlear implants at the Royal Victorian Eye and Ear Hospital. KID syndrome is a rare genodermatosis associated with mutation of the connexin-26 gene with characteristics affecting skin, hair, vision and hearing. Ichthyotic involvement of the ear canal epithelium and associated non-erosive keratosis obturans complicate hearing assessment and aid fitting. The tendency to eczematous dermatitis and otitis media is an additional problem with cochlear implantation. All cases have required additional medical management, however the outcomes show that the cochlear implant can be effective in these patients. Copyright © 2008 John Wiley & Sons, Ltd.

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