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Rigshospitalet - a part of Copenhagen University Hospital
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Cochlear implantation in Branchiootorenal syndrome - case report and review of the literature

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@article{4e364670031a47b5a0bed92ea01243cb,
title = "Cochlear implantation in Branchiootorenal syndrome - case report and review of the literature",
abstract = "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.",
keywords = "Auditory Implant, Branchio-Oto-Renal Syndrome, Cochlear implants, Correction of Hearing Impairment, Hearing Aids, Hearing Loss, Surgery, Treatment outcome",
author = "Luchen Tian and Niels West and Per Cay{\'e}-Thomasen",
year = "2021",
month = sep,
day = "9",
doi = "10.1080/14670100.2021.1973209",
language = "English",
pages = "1--6",
journal = "Cochlear Implants International",
issn = "1467-0100",
publisher = "Taylor & Francis",

}

RIS

TY - JOUR

T1 - Cochlear implantation in Branchiootorenal syndrome - case report and review of the literature

AU - Tian, Luchen

AU - West, Niels

AU - Cayé-Thomasen, Per

PY - 2021/9/9

Y1 - 2021/9/9

N2 - 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.

AB - 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.

KW - Auditory Implant

KW - Branchio-Oto-Renal Syndrome

KW - Cochlear implants

KW - Correction of Hearing Impairment

KW - Hearing Aids

KW - Hearing Loss

KW - Surgery

KW - Treatment outcome

UR - http://www.scopus.com/inward/record.url?scp=85114651895&partnerID=8YFLogxK

U2 - 10.1080/14670100.2021.1973209

DO - 10.1080/14670100.2021.1973209

M3 - Journal article

C2 - 34498539

SP - 1

EP - 6

JO - Cochlear Implants International

JF - Cochlear Implants International

SN - 1467-0100

ER -

ID: 67832665