中文摘要：

目的探讨小儿听神经病患者的听性稳态反应（auditorysteady-stateresponse，ASSR）特征及临床应用价值。方法研究对象为30例（60耳）诊断为双侧听神经病的患儿（观察组），其ABR双侧100dBnHL未引出反应，DPOAE和耳蜗微音电位（CM）均可引出，中耳功能正常。另选择30例（60耳）诊断为双侧极重度感音神经性聋的患儿为对照组，其ABR双侧100dBnHL未引出反应，DPOAE和CM均未引出，中耳功能正常。应用美国智听IHSASSR系统对两组患儿进行ASSR测试，刺激声为调幅调制声，载波频率分别为500、1000、2000和4000Hz，调制频率分别为77-103Hz，耳机型号为ER-3A插入式气导耳机。比较两组患儿各频率ASSR反应阈。结果观察组500、1000、2000和4000HzASSR反应阈分别为81．1±12．5、84．6±13．0、74．7±13．2和62．7±8．3dBnHL，对照组各频率ASSR反应阈分别为90．8±5．9、104．3±6．4、106．9±4．9和108．2±10．3dBnHL，观察组各频率ASSR反应阈均低于对照组，差异有显著统计学意义（P〈O．01）。结论虽然小儿听神经病患者ABR测试不能引出反应，但ASSR仍可引出反应，且其反应阈低于极重度感音神经性聋患儿；ASSR可引出反应的听神经病患儿可能可以排除听神经缺失的病变。

英文摘要：

Objective To investigate the characteristics and clinical significance of auditory steady--state re- sponses （ASSRs） in auditory neuropathy （AN） among children. Methods 30 children （60 ears） diagnosed as AN, showing auditory brainstem responses （ABRs） beirg absent, distortion product otoacoustic emissions （DPOAEs） and Cochlear microphonics （CMs） present, and middle ear normal. They were aged from 4 to 70 months with an av- erage age of 25± 17 months and received the ASSR test after sedated by chloridehy drate. 30 SNHL children （60 ears） made up the control group with ABRs, DPOAEs and CMs absent, and middle ear normal, aged from 5 to 62 months with an average age of 25 18 months. IHS ASSR system was used in the test with the ER- 3A insert earphones. We tested 4 {requencies （500, 1 000, 2 000, and 4 000 Hz）, each frequency on both ears with amplitude modulate （AM） stimulus at the modulate rate of 77-103 Hz. The ASSR thresholds were measured in both children with AN and SNHL. Results The threshold in children with AN were 81.1± 12.5, 84.6! 13.0, 74.7 ± 13.2 and 62.7±8.3 dB nHL at 500, 1 000, 2 000 and 4 000 Hz. The thresholds in children with SNHL were 90.8±5.9, 104.3：k6.4, 106.9±4.9 and 108.2±10.3 dB nHL. The threshold of AN group were significantly lower than those in control group （P〈0.01）. Oonclusion Authough ABRs were absent in AN children, there existed some responses of ASSR, especially in 2 000 Hz and 4 000 Hz.