摘要
目的:分析婴幼儿、儿童先天性感音神经性聋(SNHL)中先天性内耳畸形的构成、影像学及临床听力学特征。方法:回顾性分析2005-02-2010-01上海交通大学医学院附属上海儿童医学中心耳鼻咽喉科诊治的860例先天性SNHL患儿中,经颞骨高分辨率CT及MRI发现有先天性内耳畸形的125例(225耳)患儿的听力学及影像学资料。结果:860例先天性SNHL患儿中有先天性内耳畸形者占14.5%;累及双侧98例(78.4%),单侧27例(21.6%)。225耳中167耳(74.2%)为极重度聋,36耳(16%)为重度聋,22耳(9.8%)为中度聋。该组内耳畸形中,前庭水管扩大最多见(75.6%),其次为前庭畸形(32%),再次为耳蜗前庭畸形(23.1%)。耳蜗前庭畸形中以Mondini畸形最常见(55.8%),其次为共同腔畸形(28.9%)。累及耳蜗的内耳畸形中极重度聋者明显多于未累及耳蜗的内耳畸形中极重度聋者。结论:对了解中国婴幼儿、儿童先天性SNHL中先天性内耳畸形的构成,对先天性SNHL的病因诊断以及对包括助听器、耳蜗植入等在内的干预策略的制订及其预后有一定意义。
Objective：To analyze the constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss（SNHL）.Method：Imaging and auditory data of 125 cases（225 ears） in 860 pediatric patients with congenital SNHL who referred to ENT department of Shanghai Children Medical Center from February 2005 to January 2010 were retrospectively studied.Result：Congenital malformations of inner ear accounted for 14.5% in 860 pediatric patients with congenital SNHL. Bilateral ear was involved in 98 cases（78.4%）, unilateral in 27cases（21.6%）. One hundred and sixty-seven ears （74.2%） were identified as profound deafness, 36 ears （16%） severe deafness and 22 ears （9.8%） moderate deafness in 225 ears, respectively. In present group, large vestibular aqueduct （75.6%） was the most common and next was vestibular deformity （32%）, then cochleovestibular deformity （23.1%）. Mondini deformity（55.8%） was the most common and common cavity deformity（28.9%）next in cochleovestibular deformity. Profound deafness in cochlea involved congenital malformations of inner ear was significantly more than those in no cochlea involved malformations.Conclusion：The results are of importance for understanding the constitute of congenital malformations of inner ear in pediatric patients with congenital SNHL in China, for etiological diagnosis of congenital SNHL, for intervention including hearing aids or cochlear implant and prognosis.
出处
《临床耳鼻咽喉头颈外科杂志》
CAS
CSCD
北大核心
2011年第1期1-5,共5页
Journal of Clinical Otorhinolaryngology Head And Neck Surgery
关键词
内耳畸形
先天性
听力损失
感音神经性
congenital malformations
inner ear
hearing loss
sensorineural
