GJB2基因突变患者人工耳蜗术后听神经功能研究

刘学铭; 罗建芬; 晁秀华; 王睿婕; 樊兆民; 徐磊; 王海波

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GJB2基因突变患者人工耳蜗术后听神经功能研究

临床研究 | 更新时间：2026-10-28

- GJB2基因突变患者人工耳蜗术后听神经功能研究
- The Study of Auditory Nerve Function in Patients with GJB2 Gene Mutation after Cochlear Implantation
- 中国听力语言康复科学杂志 2022年20卷第5期页码：344-348
- 作者机构：
  
  山东大学山东省耳鼻喉医院,济南,250023
- 作者简介：
- 基金信息：
  
  国家自然科学基金面上项目（82071053）;国家自然科学基金青年基金（81800905）
- DOI：
  中图分类号： R764.9
- 纸质出版：2022
- 稿件说明：
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刘学铭, 罗建芬, 晁秀华, 等. GJB2基因突变患者人工耳蜗术后听神经功能研究[J]. 中国听力语言康复科学杂志, 2022,20(5):344-348.

刘学铭, 罗建芬, 晁秀华, et al. The Study of Auditory Nerve Function in Patients with GJB2 Gene Mutation after Cochlear Implantation[J]. 2022, 20(5): 344-348.
刘学铭, 罗建芬, 晁秀华, 等. GJB2基因突变患者人工耳蜗术后听神经功能研究[J]. 中国听力语言康复科学杂志, 2022,20(5):344-348. DOI：

刘学铭, 罗建芬, 晁秀华, et al. The Study of Auditory Nerve Function in Patients with GJB2 Gene Mutation after Cochlear Implantation[J]. 2022, 20(5): 344-348. DOI：

摘要

目的研究GJB2基因突变患者人工耳蜗植入（CI）术后听神经电生理功能情况，并与未查到常见耳聋基因突变的CI患者听神经电生理功能进行比较，探讨影响GJB2基因突变患者耳蜗术后效果的客观因素。方法收集2018～2019年在山东省耳鼻喉医院进行人工耳蜗植入手术，并明确GJB2基因纯合突变患者24名作为GJB2组；未查到常见耳聋基因突变并排除耳蜗畸形、听神经病者为对照组，共28名。所有参与者均使用Cochlear

Nucleus

CI产品。选取3

19号电极分别代表耳蜗高频、中频、低频，分别记录电诱发复合动作电位（electrically evoked compaund actionpotential

ECAP）阈值，最大舒适水平下ECAP的振幅，最大振幅下ECAP波形N1的潜伏期，ECAP输入/输出（I/O）曲线的斜率，比较两组患者听神经功能之间的区别

以及组内不同电极间听神经功能的区别。结果 GJB2组内不同电极间耳蜗中转电极ECAP阈值高于底转与顶转；ECAP的最大振幅在耳蜗中转处最低，顶转和底转处高于中转；ECAP的最大振幅下N1潜伏期及ECAP增益曲线的坡度在耳蜗底转、中转、顶转处无显著性差异。两组在ECAP阈值，ECAP最大振幅下N1潜伏期无明显差异（P>0.05）;GJB2组底转电极的ECAP最大振幅高于对照组，表明GJB2组耳蜗底转的神经节细胞功能更好；GJB2组每个电极ECAP增益曲线的坡度均大于对照组，但组间无显著差异（P>0.05）。结论 GJB2基因纯合突变的CI患者比未查到常见耳聋基因突变患者具有更多的残余螺旋神经节细胞，耳蜗底转的神经节细胞功能更好。ECAP神经功能检测可作为有效的评估听神经功能的工具，用于预测CI术后效果。

Abstract

Objective To study the electrophysiological function of the auditory nerve in patients with GJB2 gene mutation after cochlear implantation（CI）

and to compare the electrophysiological function of the auditory nerve in CI patients with no common deafness gene mutation

and to explore the objective influence on

the cochlear postoperative effect of patients with GJB2 gene mutation. Methods Collected 24 patients with GJB2 homozygous mutation who underwent cochlear implant surgery at Shandong Otolaryngology Hospital from 2018 to 2019 as the experimental group; a total of 28 patients in the control group

with no common deafness gene mutation and excluded the cochlea deformities

auditory neuropathy.All the participants used Cochlear

Nucleus

CI products. Selected electrodes 3

and 19 to represent the high frequency

intermediate frequency

and low frequency of the cochlea

respectively

and recorded the electrically evoked compound action potential（ECAP） threshold

the amplitude of the ECAP at the maximum comfort level

the latency of N1 at the maximum amplitude

and the ECAP input/output（I/O） function

compared the difference between the two groups and the difference of the auditory nerve function between the different electrodes in the group. Results The comparison between the different electrodes in the GJB2 group found that the ECAP threshold of the cochlear middle turn was higher than the basal turn and the apical turn; the maximum amplitude of ECAP was the lowest at the middle turn

the apical turn were the basal turn are higher than the middle turn; there was no significant difference in the N1 latency and the slope of the ECAP at the cochlear basal turn

middle turn

and apical turn. Comparing the GJB2 group and the control group

there was no significant difference between the two groups in the ECAP threshold and the N1 latency at the maximum ECAP amplitude

the maximum ECAP amplitude of the basal turn in the GJB2 group was higher than that of the control group

indicating that the ganglion cells of the basal turn in the GJB2 group had better function; the slope of the ECAP of each electrode in the GJB2 group was greater than that of the control group

but the difference between the groups was not statistically significant. Conclusions This study found that CI patients with GJB2 gene mutat

ions had more residual spiral ganglion cells than patients with no common deafness gene mutation

and the ganglion cells of basal turn in GJB2 group had better function than that in control group. ECAP test can be used as an effective tool to evaluate auditory nerve function and used to predict the postoperative effect of CI.

关键词

Keywords

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