Cochlear implantation in patients with keratitis-ichthyosis-deafness syndrome: A systematic review

Objective

Keratitis-Ichthyosis-Deafness (KID) syndrome is a rare congenital disorder characterized by vascularizing keratitis, hyperkeratosis, and profound sensorineural hearing loss. Skin debris and chronic otitis may render conventional hearing aids ineffective, and visual impairment may preclude use of sign language. This study assesses the unique challenges and outcomes associated with cochlear implantation (CI) in this patient population.

Data sources

Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol, PubMed, Web of Science, and Embase were queried from each database's inception to present day (November 2024) for articles describing CI outcomes in KID syndrome patients.

Review methods

Studies were examined for participant demographics, age at time of CI, lateralization of CI, previous hearing aid use, CI model, and follow-up length. A qualitative synthesis of post-operative complications and long-term outcomes was employed for all studies.

Results

Of 224 abstracts initially identified, 9 met the inclusion criteria, comprising 13 patients and 17 ears. Notable complications included postoperative infection (30.8 %, n = 4) and superficial wound dehiscence (15.4 %; n = 2). One patient required revision surgery with local tissue rearrangement, and one other patient was offered revision due to complications but refused. Ten ears (58.8 %) had very limited or no complications after CI. The average hearing improvement was 45 dB (28 dB–60 dB).

Conclusion

CI in patients with KID syndrome presents distinctive challenges but is a safe and effective treatment option. Close monitoring of the postoperative incision is advised to expediently manage any wound healing issues, but long-term results appear to be successful in most cases.