极重度听损大前庭水管综合征婴幼儿患者ABR和ASSR结果分析

An Analysis of the ABR and ASSR Results in Profoundly Hearing-impaired Infants with Large Vestibular Aqueduct Syndrome

目的通过对极重度听力损失的大前庭水管综合征婴幼儿进行听性脑干反应（ABR）和多频稳态反应（ASSR）测试，探讨其ABR检查过程中出现的声诱发短潜伏期负向波（acoustically evoked short latency negative response，ASNR）及其ASSR特点。方法回顾性研究22例（42耳）极重度听力损失的大前庭水管综合征（LVAS）婴幼儿，另选择28例（56耳）极重度听力损失的非LVAS的感音神经性耳聋婴幼儿作为对照组，研究其ABR及ASSR相关性。结果①LVAS婴幼儿组ABR测试中，36耳（85．7％）记录到AsNR，在109．6 dB nHL刺激强度引出的ASNR平均潜伏期在3．04±0．22msT对照组中婴幼儿无一例记录到ASNR。②LVAS婴幼儿组ASSRNI]试在250、500和1000Hz引出率明显高于对照组，两组间差异有统计学意义（P〈0．01）。结论相比于普通的极重度感音神经性耳聋患儿，极重度听损LVAS患儿进行听力学检查时，ABR容易出现ASNR负向波，ASSR测试结果显示较高的中低频的电位反应引出率，此结果可用于临床辅助诊断大前庭水管综合征。

Objective To perform auditory brainstem response（ABR） and auditory steady-state response（ASSR） tests on profoundly hearing-impaired infants with large vestibular aqueduct syndrome（LVAS） and to discuss the acoustically evoked short latency negative response （ASNR） during ABR test and the features of ASSR. Methods The clinical data of 22 profoundly hearing-impaired infants （42 ears） with LVAS were retrospectively analyzed and 28 infants （56 ears） with profound sensorineural hearing loss but no LVAS were studied as the control group. The correlations between ABR and ASSR results were analyzed with SPSS 18.0 software. Results （1） ASNR was elicited in 36 ears（85.7%） in the LVAS group and the average latency of ASNR elicited by the stimulus of 109.6 dB nil1 was 3.04±0.22 ms, but no ASNR was elicited in the control group.（2）The detection rate of ASSR in the LVAS group was higher than that in the control group at 250,500 and 1000 Hz and the difference was significant（P〈0.01）.Conclusion ASNR is more likely to be recorded in profoundly hearing- impaired children with LVAS than those without LVAS during ABR test. The detection rate of ASSR is higher at the low and middle frequencies. These results can help in the clinical diagnosis of LVAS.