Cochlear implantation in Branchiootorenal syndrome–case report and review of the literature
Research output: Contribution to journal › Journal article › Research › peer-review
Introduction: 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. Objectives: 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. Methods: 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. Results: Excellent hearing outcomes were achieved after bilateral CI despite the patient’s inner ear malformation. Discussion: The case patient’s outcomes were comparable to results reported for unselected adult, non-syndromic CI-recipients with normal inner ear anatomy. Conclusion: BOR patients experiencing insufficient benefit from conventional HA should be considered for cochlear implantation.
Original language | English |
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Journal | Cochlear Implants International |
Volume | 23 |
Issue number | 1 |
Pages (from-to) | 52-57 |
Number of pages | 6 |
ISSN | 1467-0100 |
DOIs | |
Publication status | Published - 2022 |
Bibliographical note
Publisher Copyright:
© 2021 Informa UK Limited, trading as Taylor & Francis Group.
- Auditory Implant, Branchio-Oto-Renal Syndrome, Cochlear implants, Correction of Hearing Impairment, Hearing Aids, Hearing Loss, Surgery, Treatment outcome
Research areas
ID: 314072505