大前庭水管综合征患者短潜伏期负反应特征分析

Characteristics of the Acoustically Evoked Short Latency Negative Response in Patients with Large Vestibular Aqueduct Syndrome

目的探讨大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患者听性脑干反应(ABR)的声诱发短潜伏期负反应波(acoustically evoked short latency negative response,ASNR)特征,为LVAS患者的临床诊断提供参考。方法对228例(438耳)LVAS患者进行ABR检测,按照ABR引出ASNR与否分为两组,比较不同性别、年龄、耳别、听力下降程度LVAS患者ASNR引出率的差异。结果 228例(438耳)LVAS患者中ASNR引出率为57.08%(250/438),阈值为80～100 dB nHL。不同性别、年龄LVAS患者ASNR的引出率差异无统计学意义(均为P>0.05);不同听力下降程度LVAS患者ASNR的引出率差异有统计学意义(P<0.05),重度和极重度聋患耳ASNR引出率(60.10%,238/396)高于轻度和中度聋患耳(28.57%,12/42)。双耳患病的LAVS患者中,双耳ASNR引出率(45.24%,95/210)高于单耳引出率(26.19%,55/210)。100 dB nHL刺激强度下ASNR平均潜伏期为3.22±0.21 ms,平均振幅为0.39±0.23μV。不同刺激声强度下ASNR的潜伏期、振幅差异有统计学意义(P<0.001),随着刺激声强度增加,ASNR的潜伏期缩短、振幅增大。结论 LVAS患者ASNR引出与否与性别、年龄无关,与单/双耳、听力下降程度有关;随刺激声强度增加,ASNR的潜伏期缩短、振幅增大。

Objective To explore the characteristics of ASNR in patients with LVAS and provide a scientific basis for the clinical diagnosis of LVAS patients. Methods The ABR data of 228 LVAS patients were retrospectively analyzed. According to the presence or absence of ASNR in ABR, they were divided into two groups to compare the incidence of ASNR in LVAS patients with different genders,ages,ears and the degree of hearing loss. Results The occurrence rate of ASNR was 57.08%(250/438). The ASNR thresholds were 80~100 dB nHL.There were no statistically significant differences in the incidence of ASNR between different genders and ages LVAS patients ( P > 0.05 ). However, there was a statistically significant difference in the incidence of ASNR in the LVAS patients with different degree of hearing loss( P < 0.05 ). The incidence of ASNR in the doubles ears( 45.24%,95/210) was higher than that of in the single ears( 26.19%,55/210) in patients with double LVAS.The incidence of ASNR in the patients with severe and profound deafness( 60.10%,238/396) was higher than that in the patients with mild and moderate hearing loss( 28.57%,12/42).The average latency of ASNR in the 100 dB nHL stimulus intensity was 3.22 ± 0.21 ms, and the average amplitude was 0.39 ± 0.23 μV.The difference of the latency and amplitude of ASNR under different acoustically intensities was statistically significant( P < 0.001 ).As the intensity of stimulation increased, the latency of ASNR decreased and the amplitude increased. Conclusion The presence of ASNR in LVAS patients was not related to genders and ages, but related to the ear side and the degrees of hearing loss. As the intensity of stimulation increases, the latency of ASNR decrease and the amplitude increase.