中文摘要：

目的 分析不同声刺激模式对大前庭导水管综合征短潜伏期负反应（acoustically evoked short latency negative response,ASNR）引出率的影响。方法 选取从2015年6月-2016年3月在上海交通大学医学院附属新华医院耳鼻咽喉头颈外科临床听力障碍诊治中心门诊经高分辨率颞骨CT影像学确诊为大前庭导水管综合征患者共计30例（56耳）,所有患者依次完成气导click ABR、骨导ABR、500 Hz频率特异性（TB-500 Hz）ABR 3种声刺激模式下的听力脑干反应检测,应用SPSS统计软件分析3种刺激声模式下的ASNR的整体引出率及单一声刺激模式下ASNR引出率的差异性。结果 （1）30例确诊为大前庭导水管综合征的患者中,其中男14例（46.4%）,女16例（53.6%）;年龄分布在4个月至7岁,平均（21.4±19.1）个月;患者听力损失程度轻度2例,中度10例,重度15例,极重度3例;（2）30例（56耳）中,有30耳引出ASNR,整体引出率为53.6%;（3）各不同声刺激模式下ASNR引出率分别为：click ABR有27耳引出（48.2%）、骨导ABR有耳引出（5.4%）、TB-500 Hz ABR有15耳引出（26.8%）;（4）χ2检验显示3种不同声刺激模式下ASNR引出率具有明显统计学差异（P〈0.05）;多个样本率的χ2检验显示：click和TB-500 Hz ABR声刺激下模式下的ASNR引出率没有统计学差异（P=0.019〉0.0167）,与骨导ABR声刺激模式下ASNR引出率具有明显的统计学差异。结论 click声刺激模式能获得相对更高的ASNR引出率,ASNR的引出高度提示大前庭导水管综合征可能。

英文摘要：

Objective To study the effect of acoustic stimulation mode on prevalence of acoustically evoked short latency negative responses （ASNlq） in children with large vestibular aqueduct syndrome （LVAS）. Methods 30 children （56 ears） diagnosed as LVAS with high-resolution computerized tomography （HRCT） of temporal bone were included in our study. Ordinal cliek-ABR, tone burst ABR, bone ABR tests were performed to 56 ears. The ASNR waves were recorded during the ABR test, and the prevalence was analyzed with SPSS software. Results ① Of all the 56 ears, 26 （46.4%） were male and 30 （53.6%） were female, with an average age of （21.4 ± 19. 1 ） months （ ranged from 4 months to 7 years）. As for the degree of hearing loss, 22 ears （39.3%） were mild or moderate, 27 （48.2%） were moderately severe, 7 （ 12. 5% ） were severe and profound.② ASNR waves were presented in 30 ears with a total prevalence of 53.5 %. ③The prevalence of ASNR by the click stimuli was 48.2% （27/56） , which was higher than those by tone burst stimuli （26.8% , 15/56） and bone stimuli （5.4% , 3/56）. χ^2 test showed that the differences of prevalence among different stimuli were all statistically significant （ all P 〈 0.05 ）. The χ^2 test of multiple sampling rates showed that the difference between prevalence by click stimuli and tone burst stimuli was statistically insignificant, while those between click stimuli and bone stimuli, tone burst stimuli and bone stimuli were both statistically significant. Conclusion Click stimulation mode may result in a high prevalence of ASNR. The presence of ASNR is highly indicative of LVAS.