(A806) Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Systematic Review

Introduction: Keratitis-Ichthyosis-Deafness (KID) syndrome is a rare congenital disorder identified by a triad of vascularizing keratitis, hyperkeratosis, and profound sensorineural hearing loss. This study aims to assess the unique challenges and outcomes associated with cochlear implantation (CI) in this patient population.

Methods: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol, PubMed, Web of Science, and Embase were queried from each database’s inception to present day (November 18, 2024) for articles describing cochlear implantation outcomes in patients with Keratitis-Ichthyosis-Deafness syndrome. Article titles and abstracts were reviewed and selected or removed based on the following inclusion criteria: 1) patients diagnosed with KID Syndrome; 2) patients with no prior diagnosis of other ear-related skin conditions; 3) case reports, studies, or series; and 4) published in English. A qualitative synthesis of post-operative complications, long-term outcomes, and consistent use of CI, was then employed for all of the studies.

Results: Of 224 abstracts initially identified, 9 met the inclusion criteria, comprising 13 patients and 17 ears. The average pediatric age at first implantation was 3.4 years (14 months-8 years). 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).

Conclusions: CI in patients with KID syndrome presents distinctive challenges but is an important treatment approach given progressive vision changes that render lip-reading and sign language less effective. Current literature suggests that CI in KID is a safe solution. 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.