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Rigshospitalet - a part of Copenhagen University Hospital
E-pub ahead of print

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

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  1. Cochlear implantation in a 10-year old boy with Pendred syndrome and extremely enlarged endolymphatic sacs

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  2. Auditory verbal habilitation is associated with improved outcome for children with cochlear implant

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  1. Vestibular Function in Pendred Syndrome: Intact High Frequency VOR and Saccular Hypersensitivity

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  2. Natural History of Growing Sporadic Vestibular Schwannomas During Observation: An International Multi-Institutional Study

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  3. Long-Term Vestibular Outcomes in Cochlear Implant Recipients

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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 languageEnglish
JournalCochlear Implants International
Pages (from-to)1-6
Number of pages6
ISSN1467-0100
DOIs
Publication statusE-pub ahead of print - 9 Sep 2021

    Research areas

  • Auditory Implant, Branchio-Oto-Renal Syndrome, Cochlear implants, Correction of Hearing Impairment, Hearing Aids, Hearing Loss, Surgery, Treatment outcome

ID: 67832665