耳蜗和蜗神经发育情况对蜗神经发育不良患儿人工耳蜗植入术后长期效果的影响

doi:10.3969/j.issn.1006-7795.2024.06.005

首都医科大学学报

• 内耳畸形人工耳蜗植入 • 上一篇下一篇

耳蜗和蜗神经发育情况对蜗神经发育不良患儿人工耳蜗植入术后长期效果的影响

高振橙¹, 陆思萌¹, 魏兴梅¹, 薛书锦¹, 张李芳², 王静娴¹, 马春玲¹, 孔颖¹, 拱月³, 谢静⁴, 陈彪¹, 陈婧媛¹, 李永新^1*

1.首都医科大学附属北京同仁医院耳鼻咽喉头颈外科，北京 100730； 2. 北京社会管理职业学院（民政部培训中心）康复工程学院，北京 102600； 3. 北京大学人民医院，北京 100044； 4. 杭州市第一人民医院耳鼻喉科，杭州 310006

收稿日期:2024-08-26 出版日期:2024-12-21 发布日期:2024-12-18
通讯作者: 李永新 E-mail:entlyx@sina.com
基金资助:
国家重点研发计划项目（2022YFC2402705），北京市自然科学基金项目（7244308），北京市医院管理中心青年人才培养“青苗”计划项目（QML20230204）。

The impact of cochlear and cochlear nerve development on long-term outcomes of cochlear implantation in children with cochlear nerve deficiency

Gao Zhencheng¹, Lu Simeng¹, Wei Xingmei¹, Xue Shujin¹, Zhang Lifang², Wang Jingxian¹, Ma Chunling¹, Kong Ying¹, Gong Yue³, Xie Jing⁴, Chen Biao¹, Chen Jingyuan¹, Li Yongxin^1*

1.Department of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China; 2. Department of Rehabilitation Engineering, Beijing College of Social Administration (Training Center of Ministry of Civil Affairs), Beijing 102600, China; 3. Peking University People's Hospital, Beijing 100044, China; 4. Department of Otorhinolaryngology, Hangzhou First People's Hospital, Hangzhou 310006, China

Received:2024-08-26 Online:2024-12-21 Published:2024-12-18
Supported by:
This study was supported by National Key Research and Development Program of China (2022YFC2402705), Natural Science Foundation of Beijing (7244308), Beijing Municipal Administration of Hospitals' Youth Programme (QML20230204).

摘要/Abstract

摘要： 目的探讨耳蜗畸形程度和前庭蜗神经发育情况对蜗神经发育不良（cochlear nerve deficiency，CND）患儿人工耳蜗植入（cochlear implantation，CI）术后听觉言语效果及听觉言语发育速度的影响。方法利用听觉行为分级（Categories of Auditory Performance，CAP）问卷和言语可懂度分级（Speech Intelligibility Rating, SIR）问卷评估CND患儿CI术前以及术后开机5年内的听觉言语效果。按耳蜗发育情况将患儿分为共同腔（common cavity，CC）组、耳蜗发育不全（cochlear hypoplasia，CH）组、不完全分隔（incomplete partition，IP）组和正常耳蜗（normal cochlea，NC）组；按内听道内神经束数量将患儿分为CND I级、Ⅱ级、Ⅲ级和Ⅳ级组（神经束1~4）。利用广义估计方程（generalized estimating equation, GEE）分别比较不同耳蜗类型及不同前庭蜗神经发育程度的CND患儿CI术前以及术后开机5年内的听觉言语效果。以CAP=5分，SIR=3分为事件终点进行听觉言语发育的Kaplan-Meier生存分析。结果除CI术后5年CC组和NC组CAP和SIR得分存在统计学差异外，其余不同耳蜗类型的各组 CND 患儿 CI 术前以及术后开机 5 年内的 CAP 和 SIR 得分和听觉言语发育速度不存在统计学差异。不同内听道内神经束数量的各组 CND 患儿 CI 术前 CAP 和 SIR 得分不存在显著差异。但在术后开机5年内的 CAP 和 SIR 得分以及听觉言语发育速度方面，多数组间表现出显著差异。结论前庭蜗神经发育程度是决定CND患儿CI术后短期及长期听觉言语效果及听觉言语发育速度的关键因素。是否伴有耳蜗畸形以及畸形种类并不直接影响CND患儿CI术后效果。

关键词: 蜗神经发育不良, 人工耳蜗植入, 听觉行为分级, 言语可懂度分级, 广义估计方程, Kaplan-Meier生存分析

Abstract: Objective To investigate the impact of cochlear types and the developmental status of the vestibulocochlear nerve on auditory and speech outcomes and the rate of auditory speech development in children with cochlear nerve deficiency (CND) after cochlear implantation (CI). Methods The auditory and speech outcomes of CND children were assessed before CI and within five years post-operation with the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) questionnaires. Children were categorized by cochlear type into common cavity (CC), cochlear hypoplasia (CH), incomplete partition (IP), and normal cochlea (NC) groups. They were also grouped by the number of nerve bundles in the internal auditory canal into CND I, Ⅱ, Ⅲ, and Ⅳ groups (nerve bundles 1, 2, 3, and 4, respectively). With generalized estimating equations (GEE), auditory speech outcomes were compared among different cochlear types and vestibulocochlear nerve development levels both pre-CI and within five years post-activation. Kaplan-Meier survival analysis was used to evaluate the auditory and speech development with CAP=5 and SIR=3 as event endpoints. Results Except for the statistically significant differences in CAP and SIR scores between the CC and NC groups 5 years after CI surgery, there are no statistically significant differences in CAP and SIR scores before CI surgery and within 5 years post-activation among CND children with different cochlear types. The rate of auditory and speech development also showed no significant differences among these groups. Among CND children with varying degrees of vestibulocochlear nerve development, there were no significant differences in CAP and SIR scores before CI, but significant differences were observed among most groups within 5 years post-operation. The rate of auditory and speech development showed significant differences among these groups. Conclusions The development level of the vestibulocochlear nerve was a key factor in determining both the short-term and long-term auditory speech outcomes and the rate of speech development in children with CND after CI. The presence and type of cochlear malformation did not directly impact the post-operative outcomes in CND children.

Key words: cochlear nerve deficiency, cochlear implantation, categories of auditory performance, speech intelligibility rating, generalized estimating equation, Kaplan-Meier survival analysis

中图分类号:

R762

高振橙, 陆思萌, 魏兴梅, 薛书锦, 张李芳, 王静娴, 马春玲, 孔颖, 拱月, 谢静, 陈彪, 陈婧媛, 李永新. 耳蜗和蜗神经发育情况对蜗神经发育不良患儿人工耳蜗植入术后长期效果的影响[J]. 首都医科大学学报, doi: 10.3969/j.issn.1006-7795.2024.06.005.

Gao Zhencheng, Lu Simeng, Wei Xingmei, Xue Shujin, Zhang Lifang, Wang Jingxian, Ma Chunling, Kong Ying, Gong Yue, Xie Jing, Chen Biao, Chen Jingyuan, Li Yongxin. The impact of cochlear and cochlear nerve development on long-term outcomes of cochlear implantation in children with cochlear nerve deficiency[J]. Journal of Capital Medical University, doi: 10.3969/j.issn.1006-7795.2024.06.005.

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[2]	薛书锦, 魏兴梅, 高振橙, 孔颖, 陆思萌, 陈彪, 石颖, 崔丹默, 李永新. 耳蜗发育不全解剖学特点及人工耳蜗植入术后效果研究[J]. 首都医科大学学报, 2024, 45(6): 956-965.
[3]	魏兴梅, 李永新. 内耳畸形人工耳蜗植入进展[J]. 首都医科大学学报, 2024, 45(6): 1001-1007.

耳蜗和蜗神经发育情况对蜗神经发育不良患儿人工耳蜗植入术后长期效果的影响

The impact of cochlear and cochlear nerve development on long-term outcomes of cochlear implantation in children with cochlear nerve deficiency

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