Tian Luchen, West Niels, Cayé-Thomasen Per
Department of Otorhinolaryngology Head & Neck Surgery and Audiology, Rigshospitalet, University Hospital of Copenhagen, Copenhagen Ø, Denmark.
Faculty of Health and Medical Sciences, University Hospital of Copenhagen, Copenhagen Ø, Denmark.
Cochlear Implants Int. 2022 Jan;23(1):52-57. doi: 10.1080/14670100.2021.1973209. Epub 2021 Sep 9.
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.
鳃耳肾综合征（BOR）表现为鳃瘘、耳部异常和肾脏发育异常。治疗主要以对症治疗为主。BOR最常见的后果是轻至重度感音神经性、传导性或混合性听力损失，传统助听器（HA）对此提供的益处有限。针对与BOR相关的听力损失进行人工耳蜗植入是一种新兴的治疗方法。现有文献仍然有限，主要围绕儿科病例。
本文首次介绍了一名接受双侧人工耳蜗植入（CI）的成人/非儿科BOR患者的病例，包括术前和术后的客观和主观听力结果。此外，还对已报道的BOR/BO患者人工耳蜗植入病例进行了概述。
回顾了该病例患者从初次就诊于我们的三级中心到最新随访的病历。检索Medline数据库得到了四篇关于BOR/BO患者人工耳蜗植入的论文，构成了该主题文献综述的基础。
尽管患者存在内耳畸形，但双侧CI术后听力结果良好。
该病例患者的结果与未选择的内耳解剖结构正常的成人非综合征性CI接受者所报告的结果相当。
对于传统HA受益不足的BOR患者，应考虑进行人工耳蜗植入。
