听神经病患者的临床听力学特征分析

Analysis for the clinical audiological characteristics in patients with auditory neuropathy

目的总结52例听神经病(AN)患者的临床表现特点和听力学特征,分析听力学检测在AN诊断中的意义并探讨其病损部位。方法回顾性分析52例AN患者的临床表现、纯音听力、声导抗、言语识别率、听性脑干反应(ABR)、畸变产物耳声发射(DPOAE)及对侧白噪声抑制试验的结果。结果本组52例AV患者纯音听阈测试结果均为骨、气导一致性下降的感音神经性聋。两耳间的差异无统计学意义(P>0.05),言语识别率明显差于纯音听力;鼓室导抗图为"A"型,镫骨肌声反射未引出,ABR不能引出或严重异常;52例(104耳)DPOAE全部正常引出;对侧声抑制减弱或消失。影像学检查(HRCT或MR I)无异常发现。结论AN的临床表现和系统的听力学检查对认识和明确诊断有重要意义。AN的病损部位可能在耳蜗神经传入通路和耳蜗传出周围通路。

Objective To summarize the features of clinical manifestation and audiological characteristics in patients with auditory neuropathy （AN） in order to analyze the significance of andiometry detection in the diagnosis of AN, and to investigate its lesion site. Methods The audiological data of 52 AN patients were retrospective reviewed. The patients＇ clinical findings, pure tone threshold and the examination results by acoustic impedance audiometry, speech audiometry, auditory brainstem response （ABR）, distortion product otoacoustic emission （DPOAE） and contralateral suppression were analyzed. Results In all the patients, the ratio of genders was 11.70 （ male ： female）. The patients＇ range was between 5 and 31 years, and averaged age was （ 16 ± 7） years. All the patients complained of bilateral gradual hearing loss. The patients could not discriminate speech correctly, especially in noisy environment. Among 52 patients 28 patients （50.85%） complained of last or discontinuous tinnitus, g patients developed evidence for a peripheral neuropathy. Most patients＇ audiograms showed a low frequency loss（93.3% ,97/104）. The patients＇speech hearing was worse obviously than the pure tone hearing. ABR was absent or abnormal severely,but DPOAE in all the patients were normally recorded. Acoustic stapedius reflex was absent and the contralateral suppression effect of DPOAE was disappeared. Tympanogram curve was ＂A＂ curve, and ipsilateral and contralateral acoustic stapedius reflexes were absent or present at some frequency, however,the acoustic reflex threshold was increased. Conclusion The clinical manifestation and systematic audiometry detection play an important role in the diagnosis of AN. The main lesion sites may be located in the afferent pathway of cochlear nerve and efferent pathway of cochlea.