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Branchiootorenal syndrome (BOR) manifests with branchial fistulae, otological anomalies and renal dysplasia. Management remains largely symptomatic. The most common consequence of BOR is mild-to-profound sensorineural, conductive or mixed hearing loss, where conventional hearing aids (HA) provide limited benefit. Cochlear implantation for BOR-associated hearing loss is an emerging treatment. Existing literature remains limited, centring around paediatric cases. This paper is the first to present a case of an adult/non-paediatric BOR patient receiving bilateral cochlear implants (CI), including pre- and postoperative objective and subjective hearing outcomes. Additionally, an overview of reported cases of cochlear implantation in BOR/BO patients is given. A review of the case patient’s medical charts from initial visit at our tertiary center to latest follow-up was conducted. A search of the Medline database resulted in four papers on cochlear implantation in BOR/BO patients, forming the basis of a literature review on this topic. Excellent hearing outcomes were achieved after bilateral CI despite the patient’s inner ear malformation. The case patient’s outcomes were comparable to results reported for unselected adult, non-syndromic CI-recipients with normal inner ear anatomy. BOR patients experiencing insufficient benefit from conventional HA should be considered for cochlear implantation.

Cochlear implantation in Branchiootorenal syndrome – case report and review of the literature