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

作　　者：刘学铭 罗建芬[1] 晁秀华 王睿婕[1] 樊兆民[1] 徐磊[1] 王海波[1] LIU Xue-ming;LUO Jian-fen;CHAO Xiu-hua;WANG Rui-jie;FAN Zhao-min;XU Lei;WANG Hai-bo

机构地区：[1]山东大学山东省耳鼻喉医院,济南250023

出　　处：《中国听力语言康复科学杂志》2022年第5期344-348,共5页Chinese Scientific Journal of Hearing and Speech Rehabilitation

基　　金：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)。

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^(TM)CI产品。选取3,12,19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。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 CochlearNucleusCI products. Selected electrodes 3, 12, 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 tur

关 键 词：GJB2基因突变 人工耳蜗植入 电诱发复合动作电位 

分 类 号：R764.9[医药卫生—耳鼻咽喉科]