Pearce Alexa N, Eckard Peter, Mangan Andrew R, Baggett Alaina, Dornhoffer John, Saadi Robert A
Department of Otolaryngology-Head and Neck Surgery, Division of Head and Neck Oncology and Surgery, University of Arkansas for Medical Sciences, Little Rock, AR, United States of America.
Department of Otolaryngology-Head and Neck Surgery, Division of Head and Neck Oncology and Surgery, University of Arkansas for Medical Sciences, Little Rock, AR, United States of America.
Am J Otolaryngol. 2025 Sep-Oct;46(5):104694. doi: 10.1016/j.amjoto.2025.104694. Epub 2025 Jun 23.
Our goal was to review the current literature to better understand outcomes following cochlear implantation (CI) in patients with cochlear nerve deficiency (CND).
PubMed, MED-LINE, and Google Scholar.
Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Protocol, PubMed, MED-LINE, and Google Scholar were queried for articles published from January 2000 to December 2023 describing cochlear implantation in patients with cochlear nerve agenesis or deficiency.
Of 1820 articles initially identified, 28 articles met inclusion criteria, amounting to 514 patients and 513 cochlear implantations. Among the 24 studies that differentiated between cochlear nerve hypoplasia (CNH) and aplasia (CNA), there were 154 ears with hypoplasia and 364 ears with aplasia. The most common outcome measured was Categories of Auditory Performance; other outcomes used by multiple studies included hearing threshold, Meaningful Auditory Integration Scale, Meaningful Use of Speech Scale, and Speech Intelligibility Rating. The average pre-operative CAP was 0.5, and the average post-operative CAP was 3.6. The average pre-operative hearing threshold was 101.3 dB, and 85.5 % of participants had no response on pre-operative auditory brain response. The average post-operative aided hearing threshold was 42.5 dB, with 4.2 % of patients showing no response.
CI in children with CND offers the potential for significant improvement in hearing threshold and speech development, but outcomes are variable in those with more profound nerve hypoplasia or aplasia. Families should be counseled pre-operatively on the possibility of limited results and the potential benefit of alternative treatments like auditory brainstem implantation.
我们的目标是回顾当前文献，以更好地了解耳蜗神经缺如（CND）患者人工耳蜗植入（CI）后的结果。
PubMed、MEDLINE和谷歌学术。
按照系统评价和Meta分析的首选报告项目协议，在PubMed、MEDLINE和谷歌学术中检索2000年1月至2023年12月发表的描述耳蜗神经发育不全或缺如患者人工耳蜗植入的文章。
在最初识别的1820篇文章中，28篇符合纳入标准，共计514例患者和513次人工耳蜗植入。在24项区分耳蜗神经发育不良（CNH）和发育不全（CNA）的研究中，有154耳为发育不良，364耳为发育不全。最常测量的结果是听觉表现类别；多项研究使用的其他结果包括听力阈值、有意义听觉整合量表、言语有意义使用量表和言语可懂度评分。术前平均CAP为0.5，术后平均CAP为3.6。术前平均听力阈值为101.3dB，85.5%的参与者术前听觉脑干反应无反应。术后平均助听听阈为42.5dB，4.2%的患者无反应。
CND儿童的CI有可能显著改善听力阈值和言语发育，但对于神经发育不全或发育不全更严重的患者，结果存在差异。术前应向家属咨询结果有限的可能性以及听觉脑干植入等替代治疗的潜在益处。
