231 Outcomes of Cochlear Implantation in Patients with Waardenburg Syndrome: A Systematic Review and Narrative Synthesis

Abstract

Aim This systematic review aims to establish outcomes of cochlear implantation (CI) in patients with a diagnosis of Waardenburg syndrome (WS). Method A systematic review and narrative synthesis were undertaken. Databases searched: Medline, PubMed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits placed on language or year of publication. A review conducted in accordance with the PRISMA statement. Results Searches identified 160 abstracts and 157 full texts. Of these, 22 studies met inclusion criteria reporting outcomes in 191 patients and at least 209 implants. Hearing outcomes of those receiving cochlear implantation were generally good. Five studies included genetic analysis of one or more of the participants. A total of 10 intra- or post-operative complications were reported. The methodological quality of included studies was modest, mainly comprising case reports and non-controlled case series with small cohort size. All studies were OCEBM grade III-IV. Conclusions Hearing outcomes following CI in Waardenburg syndrome are generally good with the majority of patients experiencing improvement in audiometry, speech perception, and speech intelligibility. Cochlear implant teams must use their clinical expertise to assess individual patients’ needs in order to perform cochlear implantation at the optimum age and provide thorough rehabilitation to maximise implantation benefits.