Department of Otolaryngology-Head and Neck Surgery, Shandong Provincial ENT Hospital, Cheeloo College of Medicine, Shandong University, Jinan, P.R. China.
Ear Hear. 2023;44(3):558-565. doi: 10.1097/AUD.0000000000001299. Epub 2022 Dec 8.
In this study, we aimed to (1) review the long-term outcomes of cochlear implantation in children with cochlear nerve aplasia and (2) compare the development of their auditory and speech abilities to children with normal-sized cochlear nerves.
This is a retrospective case-control study. Patients who underwent unilateral cochlear implant (CI) surgery in a tertiary referral center from September 2012 to December 2018 were reviewed. The study group included 55 children with cochlear nerve aplasia diagnosed using preoperative images. The control group included 35 children with normal-sized cochlear nerves. The control group did not differ from the study group in terms of age at implantation, pre-implantation auditory and speech abilities, or the electrode array type. Cochlear implantation outcomes were assessed using a test battery, including the Categories of Auditory Performance (CAP) score, the Speech Intelligibility Rating (SIR) score, behavioral audiometry, and closed- or open-set speech recognition tests. The development of auditory and speech abilities was compared between the two groups using Generalized Linear Mixed-effect Models.
The mean duration of CI usage was 4.5 years (SD = 1.5, range = 2.0 to 9.5) in the study group. The CAP scores, SIR scores, and aided hearing thresholds improved significantly post-implantation in the study group, but were significantly poorer than those in the control group. Generalized Linear Mixed-effect Models showed that the development of CAP and SIR scores was significantly slower in the study group than in the control group. Overall, 27 (49%) children with cochlear nerve aplasia had some degree of open-set speech perception skills, but the monosyllabic and bisyllabic word recognition rates were significantly lower than those in the control group.
For children with cochlear nerve aplasia, auditory perception and speech intelligibility continued to improve in the long-term follow-up, but this progress was significantly slower than in children with normal-sized cochlear nerves. Most children with cochlear nerve aplasia could obtain the ability of common phrase perception and understanding simple spoken language with consistent CI usage and auditory rehabilitation.
本研究旨在：(1) 回顾儿童耳蜗神经发育不良患者人工耳蜗植入的长期效果；(2) 将其听觉和言语能力的发展与正常耳蜗神经患者进行比较。
这是一项回顾性病例对照研究。对 2012 年 9 月至 2018 年 12 月在一家三级转诊中心接受单侧人工耳蜗植入（CI）手术的患者进行了回顾性分析。研究组包括 55 例术前影像学诊断为耳蜗神经发育不良的患者。对照组包括 35 例耳蜗神经正常的患者。两组在植入年龄、植入前的听觉和言语能力以及电极阵列类型方面无差异。采用一系列测试评估人工耳蜗植入的效果，包括听觉能力分类（CAP）评分、言语可懂度评分（SIR）、行为测听和闭合或开放式言语识别测试。采用广义线性混合效应模型比较两组患者听觉和言语能力的发展。
研究组 CI 使用的平均时间为 4.5 年（SD=1.5，范围 2.0 至 9.5）。研究组植入后 CAP 评分、SIR 评分和助听听阈显著提高，但明显低于对照组。广义线性混合效应模型显示，研究组 CAP 和 SIR 评分的发展速度明显慢于对照组。总体而言，27 例（49%）耳蜗神经发育不良患儿具有一定程度的开放式言语感知能力，但单音节和双音节词识别率明显低于对照组。
对于耳蜗神经发育不良的儿童，在长期随访中，听觉感知和言语清晰度仍在不断提高，但与正常耳蜗神经儿童相比，这一进展明显较慢。大多数耳蜗神经发育不良患儿在持续使用人工耳蜗和听觉康复的情况下，能够获得对常用短语的感知能力和理解简单口语的能力。
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