22例听神经病患者临床及听力学特征分析

The Clinical and Audiological Characteristics of 22 Patients with Auditory Neuropathy

孙素芬;索利敏;司楠楠;宋颖慧;董美;靳玲;张磊;杨洁;赵长青;

1:山西医科大学

2:山西医科大学第二医院耳鼻咽喉头颈外科

3:山西省长治医学院附属和平医院耳鼻咽喉头颈外科

摘要
目的探讨听神经病(auditory neuropathy,AN)患者的临床表现特点和听力学特征,减少漏诊与误诊。方法回顾性分析22例听神经病患者的临床资料,分析性别、发病年龄、患耳侧别等,并对其纯音测听、声导抗、听性脑干反应(auditory brainstem responses,ABR)、畸变产物耳声发射(distortion product otoacoustic emissions,DPOAE)及40 Hz听觉事件相关电位(40 Hz-AERP)等结果进行分析。结果 22例AN患者中,男8例,女14例,发病年龄小于10岁者6例, 10~20岁者15例,大于20岁者1例,平均发病年龄14.23±6.73岁。双侧发病19例,其中14例患者ABR未引出,5例患者反应阈45~95 dB nHL;14例患者双耳DPOAE各频率均可引出,4例部分频率引出,1例各频率均未引出;听阈图多呈低频下降型,以轻、中度听力损失为主;15例鼓室导抗图为A型,3例As型,1例C型;15例镫骨肌声反射同侧及对侧均未引出,4例均可引出;40 Hz听觉事件相关电位7例双侧均未引出,4例单侧可引出,8例双侧均可引出。3例单侧发病患者中2例患耳ABR未引出,1例反应阈95 dB nHL;3例患耳听阈图为平坦型,平均听阈值分别为11.25、70、100 dB HL;3例患耳40 Hz-AERP及DPOAE均可引出。结论本组听神经病患者起病早,发病缓慢,典型听神经病患者听阈图多为低频下降型,DPOAE可引出,ABR严重异常或缺失。
关键词
听神经病;听性脑干反应;畸变产物耳声发射;纯音听阈
基金项目(Foundation):
山西省回国留学人员科研资助项目(2017-118);; 山西省重点研发计划项目(201803D31122)
作者
孙素芬;索利敏;司楠楠;宋颖慧;董美;靳玲;张磊;杨洁;赵长青;
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