Waardenburg综合征患者人工耳蜗术前EABR检测及术后生活质量评估  

Detection of EABR before Cochlear Implantation for Deafness with Waardenburg Syndrome and Postoperative Quality of Life Assessment

作　　者：王斌[1] 郭晓会 曹克利[1] 魏朝刚[3] 高志强[1] 王素菊[1] WANG Bin;GUO Xiao-hui;CAO Ke-li;WEI Chao-gang;GAO Zhi-qiang;WANG Su-ju

机构地区：[1]中国医学科学院北京协和医院耳鼻喉科,北京100730 [2]解放军总医院第三医学中心眼科医学部,北京100853 [3]北京大学第一医院耳鼻咽喉头颈外科,北京100034

出　　处：《中国听力语言康复科学杂志》2022年第4期289-293,共5页Chinese Scientific Journal of Hearing and Speech Rehabilitation

基　　金：北京市科委首都临床特色应用研究基金(Z171100001017218)。

摘　　要：目的探讨Waardenburg综合征(waardenburg syndrome,WS)患者人工耳蜗植入术前采用电刺激听觉脑干诱发电位(EABR)检测方法评估听觉通路的可行性及患者术后效果的评估。方法收集2008年~2018年在我院行人工耳蜗手术的WS患者10例,回顾性分析患者术前听力资料、合并眼病情况,术前行EABR检测听觉通路功能,随访术后神经反应遥测(neural response telemetry,NRT)测试结果及开机调试结果,使用听力植入体儿童的家庭生活质量问卷(children using hearing implants quality of life,CuHIQol)评估植入者术后生活质量。另选择相同听觉年龄的耳蜗结构正常植入患者10例作为对照组,对两组测试结果进行统计学分析。结果10例WS合并双侧极重度神经性聋患者术前EABR测试听觉通路功能好,EABR波形分化2~4级,植入不同类型人工耳蜗,V波平均阈值179.35±12.21 CL,平均动态范围26.28±11.45 CL,Ⅴ波I/O曲线斜率0.026±0.015和对照组统计分析有显著差异;20例患者术后无面瘫、脑脊液漏等手术并发症;10例WS患者术后均获得不同程度电听性反应,开机测试NRT引出率30%,低于对照组90%;开机12月平均调机C值WS组177.57±20.45 CL;对照组167.85±22.38 CL(P<0.05);两组患者的EABR阈值和开机C值有显著相关性(P<0.05)。WS组患者在父母期望、家庭影响及生活质量3方面得分均较术前明显提高,CuHIQol评分术前总分37.55±7.81,术后59.04±7.63(P<0.05)。两组患者术前EABR检测V波I/O曲线斜率和术后CuHIQol有显著相关性(P<0.05)。结论Waardengburg综合征伴双侧极重度神经性聋患者术前生活质量差,人工耳蜗植入术前EABR检测可以筛选手术适应症,患者植入人工耳蜗后生活质量显著好于术前,独立生活能力和自信心均有明显提高。Objective To explore the method of electrically evoked auditory brainstem responses(EABR)evaluation of auditory pathway before cochlear implantation in patients with deafness combined with Waardenburg syndrome(WS)and the effect of quality of life rehabilitation after cochlear implantation.Methods 10 cases of deaf patients with WS who underwent cochlear implantation in our hospital from 2008 to 2018 were collected,and the preoperative hearing data and eye diseases were retrospectively analyzed.The function of auditory pathway was detected by EABR.The results of postoperative NRT test and mapping were followed up.Hearing Implants Quality of life questionnaire(CuHIQol)was used to evaluate the quality of life of the patients.Another 10 patients with normal cochlear structure of the same age were selected as the normal control group,and the test results were compared and analyzed.Results For the patients with severe sensorineural hearing loss combined with WS,the function of auditory pathway was good by EABR test preoperation,and cochlear implants with different types of electrodes were implanted.The wave differentiation of EABR was grade 2-4 in the WS group.There were significant differences in the average threshold of V wave 179.35±12.21 CL,the average dynamic range 26.28±11.45 CL,and the slope of V wave I/O curve(0.026±0.015)between the two groups.There were no postoperative complications,such as facial paralysis and cerebrospinal fluid leakage in 20 patients.Ten patients with deafness combined with WS had different degrees of electroacoustic response after operation,and the NRT extraction rate(3/10)was 30%,which was lower than 90%in the control group(9/10).The average C value of 12 months after operation was a significant difference between the two groups(177.57±20.45CL vs 167.85±22.38CL,P<0.05).There was a significant correlation between the EABR threshold and C value of the two groups(P<0.05).The CuHIQol score of 10 patients with WS was 37.55±7.81 preoperatively and 59.04±7.63 postoperatively(P<0.05),sign

关 键 词：WAARDENBURG综合征 耳聋 人工耳蜗 电刺激脑干听觉诱发电位 生活质量 

分 类 号：R764.9[医药卫生—耳鼻咽喉科]