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Published on: March 24, 2023
Cochlear implantation in children with keratitis-ichthyosis-deafness (KID) syndrome: outcomes in three cases
Elizabeth J Barker1, Robert Js Briggs
1Department of Otolaryngology, The University of Melbourne, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia. ebarker@bionicear.org
Insights
Cochlear implants can effectively improve hearing in children with rare keratitis-ichthyosis-deafness (KID) syndrome. Despite complications from the condition, outcomes show significant benefits for these patients.
Area of Science:
- Otolaryngology
- Genetics
- Dermatology
Background:
- Keratitis-ichthyosis-deafness (KID) syndrome is a rare genodermatosis.
- It is associated with connexin-26 gene mutations.
- KID syndrome affects skin, hair, vision, and hearing.
Observation:
- Three children with KID syndrome underwent cochlear implantation.
- Ichthyotic ear canal epithelium and keratosis obturans complicated assessment and fitting.
- Eczematous dermatitis and otitis media presented additional challenges.
Findings:
- Cochlear implantation was performed in three children with KID syndrome.
- Despite complications, the cochlear implants were effective.
- All cases required additional medical management.
Implications:
- Cochlear implantation is a viable option for hearing restoration in KID syndrome.
- Management of associated dermatological and otological issues is crucial for successful outcomes.
- Further research into optimizing cochlear implant protocols for rare genetic syndromes is warranted.
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.