合并其他内耳畸形的大前庭水管综合征患者的短潜伏期负反应特征分析

Characteristics Analysis of the Acoustically Evoked Short Latency Negative Response of Large Vestibular Aqueduct Syndrome with Other Inner Ear Malformations

目的探讨合并其他内耳畸形的大前庭水管综合征(LVAS)患者ABR的声诱发短潜伏期负反应(ASNR特征,为LVAS患者的临床诊断提供参考。方法对176例(339耳)LVAS患者的ABR检测结果进行分析,按照ABR引出ASNR与否分为两组,比较ASNR引出率的差异。结果合并其他内耳畸形的LVAS中,以合并前庭增宽畸形居多,Mondini畸形次之;ASNR的引出率在单纯/合并其他内耳畸形LVAS患者之间的差异无统计学意义(P=0.448>0.05);不同性别、年龄、听力损失程度的合并其他内耳畸形LVAS患者ASNR引出率差异无统计学意义(P分别为0.342、0.928、0.166,P>0.05);合并不同内耳畸形LVAS患者的ASNR引出率差异有统计学意义(P=0.045<0.05)。在仅合并前庭增宽畸形的LVAS患者中,有/无ASNR组患者的前庭宽度差异无统计学意义(P=0.996>0.05);在合并两种或两种以上畸形(包含前庭增宽畸形)的LVAS患者中,有/无ASNR组患者的前庭宽度差异有统计学意义(P=0.002<0.01)。结论 ASNR的引出率与单纯/合并其他内耳畸形LVAS无关;合并其他内耳畸形LVAS患者ASNR引出率与性别、年龄、听力损失程度无关,与畸形类型有关;在合并两种或两种以上畸形(包含前庭增宽畸形)LVAS患者中,无ASNR组的前庭增宽畸形程度大于有ASNR组。

Objective To explore the ASNR characteristics of LVAS with other inner ear malformations and provide references for its clinical diagnosis.Methods ABR tests were performed in 176 patients with LVAS,according to the presence or absence of ASNR,they were divided into two groups.The differences of the extraction rate of ASNR between the two groups was compared.Results The majority of LVAS with other inner ear malformations were those with vestibular broadening malformation,followed by Mondini malformation.There was no statistically significant difference in the extraction rate of ASNR between simple LVAS and LVAS with other inner ear malformations(P=0.448>0.05).There was no statistically significant difference in the extraction rate of ASNR in LVAS with other inner ear malformations patients with different genders,ages and the degree of hearing loss(P=0.342、0.928、0.166,P>0.05).There were statistically significant difference in ASNR extraction rates in LVAS with different inner ear malformations(P=0.045<0.05).In LVAS patients with only vestibular broadening deformity,the size of the vestibules in the two groups was not statistically significant difference(P=0.996>0.05).In LVAS patients with two or more deformities(including vestibular broadening deformity),the size of the vestibules in the two groups was statistically significant difference(P=0.002<0.01).Conclusion The extraction rate of ASNR was independent of simple LVAS or LVAS with other inner ear malformations.The extraction rate of ASNR in LVAS with other inner ear malformations was not related to gender,age and the degree of hearing loss,but was related to the type of deformity.In LVAS patients with two or more deformities(including vestibular broadening deformity),the degree of vestibular broadening deformity in the group without ASNR was greater than that in the group with ASNR.