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耳蜗神经发育不全或发育不良的儿科患者人工耳蜗植入的临床观点

Clinical Perspectives on Cochlear Implantation in Pediatric Patients with Cochlear Nerve Aplasia or Hypoplasia.

作者信息

Raynor Ava, Perez Sara, Worthington Megan, Shafiro Valeriy

机构信息

Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, USA.

Communication Disorders and Sciences, Rush University, 600 S. Paulina Street, Chicago, IL 60612, USA.

出版信息

Audiol Res. 2025 Aug 5;15(4):96. doi: 10.3390/audiolres15040096.

DOI:10.3390/audiolres15040096
PMID:40863022
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12382941/
Abstract

: Cochlear implantation (CI) in pediatric patients with cochlear nerve deficiencies (CND) remains controversial due to a highly variable clinical population, lack of evidence-based guidelines, and mixed research findings. This study assessed current clinical perspectives and practices regarding CI candidacy in children with CND among hearing healthcare professionals in the USA. : An anonymous 19-question online survey was distributed to CI clinicians nationwide. The survey assessed professional background, experience with aplasia and hypoplasia, and perspectives on CI versus auditory brainstem implant (ABI) candidacy, including imaging practices and outcome expectations. Both multiple-choice and open-ended responses were analyzed to identify trends and reasoning. : Seventy-two responses were analyzed. Most clinicians supported CI for hypoplasia (60.2%) and, to a lesser extent, for aplasia (41.7%), with audiologists more likely than neurotologists to favor CI. Respondents cited lower risk, accessibility, and the potential for benefit as reasons to attempt CI before ABI. However, many emphasized a case-by-case approach, incorporating imaging, electrophysiological testing, and family counseling. Only 22.2% considered structural factors the best predictors of CI success. : Overall, hearing health professionals in the USA tend to favor CI as a first-line option, while acknowledging the limitations of current diagnostic tools and the importance of individualized, multidisciplinary decision-making in CI candidacy for children with CND. Findings reveal a high variability in clinical perspectives on CI implantation for pediatric aplasia and hypoplasia and a lack of clinical consensus, highlighting the need for more standardized assessment and imaging protocols to provide greater consistency across centers and enable the development of evidence-based guidelines.

摘要

由于临床人群差异很大、缺乏循证指南以及研究结果不一,儿童蜗神经发育不全(CND)患者的人工耳蜗植入(CI)仍然存在争议。本研究评估了美国听力保健专业人员对于CND儿童CI候选资格的当前临床观点和做法。

一项包含19个问题的匿名在线调查已分发给全国的CI临床医生。该调查评估了专业背景、关于发育不全和发育不良的经验,以及对CI与听觉脑干植入(ABI)候选资格的看法,包括成像做法和结果预期。对多项选择和开放式回答都进行了分析,以确定趋势和理由。

分析了72份回复。大多数临床医生支持对发育不良进行CI植入(60.2%),对发育不全的支持程度稍低(41.7%),听力学家比神经耳科学家更倾向于CI。受访者将较低的风险、可及性以及获益潜力作为在ABI之前尝试CI的理由。然而,许多人强调要采取逐案处理的方法,纳入成像、电生理测试和家庭咨询。只有22.2%的人认为结构因素是CI成功的最佳预测指标。

总体而言,美国的听力保健专业人员倾向于将CI作为一线选择,同时承认当前诊断工具的局限性以及在CND儿童CI候选资格方面进行个体化、多学科决策的重要性。研究结果显示,对于小儿发育不全和发育不良的CI植入,临床观点存在很大差异,且缺乏临床共识,这凸显了需要更标准化的评估和成像方案,以在各中心之间提供更大的一致性,并推动循证指南的制定。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4995/12382941/d266bd17b73c/audiolres-15-00096-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4995/12382941/d266bd17b73c/audiolres-15-00096-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4995/12382941/d266bd17b73c/audiolres-15-00096-g001.jpg

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本文引用的文献

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Int J Audiol. 2025 Jul;64(7):685-694. doi: 10.1080/14992027.2025.2454439. Epub 2025 Jan 27.
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Eur Arch Otorhinolaryngol. 2025 May;282(5):2203-2215. doi: 10.1007/s00405-024-09087-w. Epub 2024 Dec 10.
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Laryngoscope. 2025 Apr;135(4):1259-1266. doi: 10.1002/lary.31888. Epub 2024 Nov 9.
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A Systematic Review and Meta-analysis Examining Outcomes of Cochlear Implantation in Children With Bilateral Cochlear Nerve Deficiency.双侧耳蜗神经发育不良儿童人工耳蜗植入术后结局的系统评价和荟萃分析
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