中文摘要：

目的 分析听神经病谱系障碍患者（auditory neuropathy spectrum,ANSD）首诊与复诊的听力学特征,预测ANSD患者临床听力学转归,并建立有效的随访方案。方法 对1997年4月至2014年3月间确诊为ANSD的22例（40耳）患者进行随访,随访期1-17年,对比分析患者首诊及末次复诊的听力学特征。听力学检查包括：纯音测听、言语识别率测试（SDS）、畸变产物耳声发射测试（DPOAE）,声导抗测试,听性脑干反应测试（ABR）。结果 对比患者首诊与末次复诊听力学特点：1不同随访期组间两两比较,纯音听阈未见明显变化（P〉0.05）;2随访期1-3年组与随访期〉7年组间言语识别率有统计学意义（P〈0.05）;3首诊患者的DPOAE均正常或大部分正常引出,复诊中低频DPOAE的引出率降低,其中随访期为1-3年的患者全频DPOAE引出率下降;4镫骨肌反射引出率由20%下降为5%;5ABR波形无明显变化,均为2耳（5%）引出V波。结论 1随着随访期的延长,ANSD患者言语识别率、DPOAE引出率、镫骨肌反射引出率下降,部分患者将表现出感音神经性耳聋的临床听力学特征。患者中主诉有言语识别障碍的,仍有可能为ANSD;2随访方案仍需继续完善,以更好地为患者提供帮助,并在一定程度上提高复诊率。

英文摘要：

Objective To analyse the audiological characteristics of patients with auditory neuropathy spectrum disorder （ANSD） for gaining a better understanding of the audiological characteristics prognosis of ANSD patients. As well as to make a scheme for patients＇ further consulation, facilitating the patients＇ further consulation. Methods 22 patients （40ears） with ANSD were enrolled, all of whom had received further consulation within 17 years after the first consulation. Pure tone test, speech-discrimination score test （SDS）, distortion product otoacoustic emission （DPOAE）, acoustic immitance testingand auditory brainstem response （ABR） were performed on these patients. Results The differences between the first consulation and the further consulation are as follows： ①There were no significant changes in pure tone average（P〉0.05）. ②The SDS of the patients who came back for further consulation in 3 years differ from the ones who came back in 7 years（P〈0.05）. ③Most patients had almost all the sensitive DPOAE in the first consulation, while they had worse results in the low frecequeces in the further consulation. The whole frequences of the DPOAE became worse in the patients who came back for further consula- tion in 3 years. @The acoustic reflex （AR） results decrease from 20% to 5%. （~）There were no significant changes in the ABR. Conclusion ①With the development of the disease, we can see the SDS, the DPOAE results and the AR results be- came worse. As a result, a part of the patients with ANSD would perform like the ones with sensorineural hearing loss （SNHL）. So we must pay attention to those patients who complain that they can hear the sound without understanding what others are talking about. ②It is necessary to improve the scheme of patients＇ further consultation, easing the follow-up study and further understanding the clinical prognosis of ANSD.