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

作　　者：孙素芬 索利敏[2] 司楠楠 宋颖慧 董美 靳玲[2] 张磊[2] 杨洁[2] 赵长青[2] Sun Sufen;Suo Limin;Si Nannan;Song Yinghui;Dong Mei;Jin Ling;Zhang Lei;Yang Jie;Zhao Changqing(Shanxi Medical University,Taiyuan,030001,China)

机构地区：[1]山西医科大学,太原030001 [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

出　　处：《听力学及言语疾病杂志》2020年第2期122-127,共6页Journal of Audiology and Speech Pathology

基　　金：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)。

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。Objective To investigate the clinical and audiological characteristics of patients with auditory neuropathy(AN).Methods The clinical data of 22 patients with AN were analyzed retrospectively.The results of pure tone audiometric,acoustic immittance,auditory brainstem response(ABR),distortion product otoacoustic emission(DPOAE)and 40 Hz auditory event-related potential were analyzed.Results Among the 22 patients,8 were male,14 were female;6 were younger than 10 years old,15 were 10 to 20 years old,and 1 was older than 20 years old.The average age was 14.23±6.73 years old.There were 19 cases of bilateral onset,of which 14 cases of ARB were not elicited but 5 cases were elicited.The reaction threshold was 45~95 dB nHL.All the frequencies of binaural DPOAE were induced in 14 patients,partial frequencies were induced in 4 patients,but no frequency was induced in 1 patient.The hearing thresholds were mostly sloping low-frequency.Most of them had mild and moderate hearing loss.Five cases showed type A tympanograms,3 cases of type As and 1 case of type C.A total of 15 cases of acoustic reflexes were absent from the ipsilateral and the contralateral side while only 4 showed reflexes.A total of 7 cases had no 40 Hz auditory event related potentials,but 4 cases were unilaterally present,and 8 cases were present on the both sides.Three patients had unilateral onset,of whom 2 cases showed no ABR responses,and 1 case had a response at 95 dB nHL.Three patients had flat hearing thresholds with an average hearing threshold of 11.25,70,and 100 dB HL,and 3 patients showed 40 Hz auditory event-related potentials and DPOAE responses.Conclusion This group of patients with auditory neuropathy had early onset but slow progress.Most of the typical auditory neuropathy patients had low-frequency descending audiogram.Although DPOAE could be induced,ABR was seriously abnormal or absent.

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 

分 类 号：R764.4[医药卫生—耳鼻咽喉科]