大前庭水管综合征与其它重度-极重度聋患者的声诱发短潜伏期负反应波的比较

A Comparison of the Auditory Characteristics of ASNR among Patients with LVA and other Patients with Severe to Profound Deafness

目的通过分析大前庭水管综合征(largevestibularaqueductsyndrome,LVAS)患者的声诱发短潜伏期负反应(acoustically evoked short latency negative response,ASNR)的临床特征,并与无LVAS的其它内耳畸形和不伴有内耳畸形的重度-极重度耳聋患者比较,探索ASNR在LVAS诊断中的临床价值。方法回顾性分析2014年4月~2020年12月在我院耳鼻咽喉头颈外科就诊,经颞骨CT或内听道MRI确诊为单纯LVAS、无LVAS的其它内耳畸形,以及无内耳畸形的重度-极重度感音性聋患者在95dBnHL气导短声ABR(click-ABR)刺激声条件下ASNR的引出率、幅值、潜伏期和阈值。结果(1)单纯LVAS组、无LVAS的其它内耳畸形组,以及无内耳畸形的重度-极重度耳聋组的ASNR引出率分别为56.90%(66/116耳)、14.5%(8/55耳)、9.7%(10/103耳),差异具有显著差异(P<0.05),LVAS组患者ASNR的引出率最高;(2)3组ASNR的平均阈值分别为88.71±8.15dBnHL、93.50±2.42dBnHL和89.17±7.68dBnHL,无显著差异(P>0.05);(3)3组在95dBnHL刺激声条件下ASNR的平均潜伏期分别为2.98±0.25ms、2.83±0.34ms和3.17±0.21ms,有显著差异(P<0.05),其它内耳畸形组患者的ASNR潜伏期最短;(4)3组在95dBnHL刺激声条件下ASNR的平均幅值分别为0.36±0.14μV、0.20±0.05μV和0.18±0.05μV,有显著差异(P<0.05)LVAS组幅值最高。结论与其它内耳畸形和不伴有内耳畸形的重度-极重度耳聋患者相比,LVAS患者的ASNR引出率和幅值明显增高。婴幼儿click-ABR检测中出现ASNR且幅值明显增高时,高度提示LVAS。

Objective To analyze and compare the auditory characteristics of acoustic evoked short latency negative response(ASNR)among patients with large vestibular aqueduct syndrome(LVAS)other inner ear malformations and severe-profound deafness patients without inner ear malformations,and further to explore the clinical value of ASNR in the diagnosis of LVAS.Methods Carried out a retrospective analysis of the air conduction click ABR(Click-ABR)test results among patients with LVAS,other inner ear malformations without LVAS,and severe to profound deafness patients without any inner ear malformations that were diagnosed by temporal bone CT or inner ear MRI imaging in our hospital from April 2014 to December 2020.The ASNR presence,amplitude and latency under 95 dB nHL stimulation and threshold were compared and analyzed among these three groups.Results(1)The presence of ASNR among LVAS group,other inner ear malformations group and severe-profound deafness group was 56.90%(116 ears),14.5%(55 ears)and 9.7%(103 ears),respectively,the difference was statistically significant(P<0.05).The presence of ASNR was highest in LVAS group compared with the other two groups.(2)The average threshols of ASNR in these three groups were 88.71±8.15 dB nHL,93.50±2.42 dB nHL and 89.17±7.68 dB nHL,respectively.No statistical difference was found;(3)The average latency of ASNR under 95 dB nHL click-ABR stimulation was 2.98±0.25,2.83±0.34 ms and 3.17±0.21 ms,respectively.There was a significant difference(P<0.05).The latency of other inner ear malformations group was shortest compared with LVAS group and severe-profound deafness group.(4)The average amplitudes of ASNR under 95 dB nHL click-ABR stimulation was 0.36±0.14μV,0.20±0.05μV,0.18±0.05μV,respectively.The amplitude of LVAS group was highest compared with the other group with statistically significant difference(P<0.05).Conclusion The presence and amplitude of ASNR were significantly higher in patients with LVAS group than patients with other inner ear malformations group and severe-profound deafness group.It was highly recommended as LVAS when ASNR was appeared with high amplitude during click ABR test.