前庭导水管扩大患儿影像与听力结果分析

Analysis of imaging and hearing results in children with enlarged vestibular aqueduct

目的回顾性分析前庭导水管扩大患儿的耳部影像表现及新生儿听力筛查结果及听力测试结果,探讨单纯前庭导水管扩大及合并其他内耳畸形的患儿的新生儿听力筛查通过率及听力损失程度是否有差异。方法2009年1月至2019年12月在首都医科大学附属北京同仁医院儿童听力诊断中心就诊,电子计算机断层扫描(computed tomography,CT)显示有前庭导水管扩大伴或不伴其他内耳畸形的患儿92例(182耳),入组者均接受过新生儿听力筛查。收集患儿新生儿听力筛查、听力学测试结果及耳部影像学资料。根据影像学表现分为3组:A组为单纯前庭导水管扩大;B组为Mondini畸形伴前庭导水管扩大及前庭池扩大即不完全分隔Ⅱ型(incomplete partition typeⅡ,IP-Ⅱ);C组为前庭导水管扩大伴前庭池扩大或半规管畸形。采用卡方检验对比组间的新生儿听力筛查通过率;采用Kruskal-Wallis秩和检验对比组间听力损失程度。结果92例患儿首次就诊年龄为1.5~65个月,其中男性47例(51.1%)、女性45例(48.9%)。182耳中,A组占40.11%(73/182)、B组占26.37%(48/182)、C组占33.52%(61/182),B+C组约占59.89%。3组新生儿听力筛查通过率比较,差异无统计学意义(χ^(2)=1.122,P=0.571);伴与不伴Mondini畸形间比较,差异无统计学意义(χ^(2)=0.401,P=0.526)。3组听力损失程度及伴与不伴Mondini畸形组间比较,差异均无统计学意义(P>0.05)。结论前庭导水管扩大合并其他内耳畸形约占60%;无论前庭导水管扩大伴与不伴其他内耳畸形,新生儿听力筛查通过率及听力损失程度均差异无统计学意义,提示前庭导水管扩大所致的迟发性听力损失与伴随的其他畸形类型无关。

Objective To explore the differences in the pass rate of neonatal hearing screening and degree of hearing loss between the ears with simple enlarged vestibular aqueduct(EVA)and EVA accompanied by other inner ear malformations.Methods The objects of this study were children diagnosed with enlarged vestibular aqueduct by computed tomography in Children's Hearing Diagnosis Center,Beijing Tongren Hospital,Capital Medical University and accepted newborn hearing screening(NHS)from January 2009 to December 2019.There are a total of 182 ears of 92 cases.The results of their NHS,clinical hearing tests and imaging of ear were collected,and the children were grouped according to image performance.The ears in group A with EVA alone,group B with EVA and Mondini deformity(vestibular pool enlarged may be included simultaneously)(IP-Ⅱ),and group C with EVA and enlarged vestibular pool or semicircular canal malformation without Mondini malformation.The difference in the pass rate of NHS between different groups was analyzed with chi-square test.Kruskal Wallis rank sum test was used to compare the degree of hearing loss between different groups.Results The age of 92 patients is between 1.5-65.0 months,47 males(51.1%)and 45 females(48.9%).There were 73 ears in group A(40.11%,73/182),48 ears in group B(26.37%,48/182),and 61 ears in group C(33.52%,61/182).Group B and group C account for 59.89%in total.The pass rate of NHS showed no significant difference between A,B,and C groups(χ^(2)=1.122,P=0.571),and between B and A+C groups(χ^(2)=0.401,P=0.526).The difference of the degree of hearing loss between different group showed no statistical significance(P>0.05).Conclusion Most children(about 60%)enrolled in this study had EVA accompanied by other inner ear malformation.The pass rate of newborn hearing screening and the degree of hearing loss showed no significant difference between the groups.Whether vestibular aqueduct enlargement is accompanied by other inner ear malformations could lead to delayed hearing loss.