目的:分析在儿童感音神经性耳聋(sensorineural hearing loss,SNHL)中先天性内耳畸形患儿的影像学及临床特征.方法:回顾分析860例SNHL患儿中因听力障碍(109例) 、外耳畸形(3例)、面部异常(13例)来就诊的125例(225耳)患儿的临床资料.结果:109例(87.2%)患儿因听力障碍就诊,母亲妊娠早期有感冒史52例(41.6%).166耳(73.8%)极重度耳聋,累及双侧100例(80%).颞骨高分辨率CT或磁共振成像(MRI)显示其中同时合并外耳及中耳畸形3耳,合并中耳畸形13耳.Michel 畸形2例(4耳),耳蜗不发育1例(1耳),共同腔畸形10例(14耳),不完全分隔-I型2例(2耳),耳蜗前庭发育不良2例(2耳),不完全分隔-II型(Mondini 畸形)19例(29耳).累及前庭导水管扩大91例(170耳)(75.6%),半规管畸形30例(46耳)(20.6%),内听道畸形35例(49耳)(21.7%).结论:前庭导水管扩大是儿童SNHL中最常见的内耳影像学异常.患儿多为双侧重度听力损失或全聋.对于先天性内耳畸形的患儿行高分辨率CT检查非常重要.%Objective: To study the clinical features of inner ear malformations with sensorineural hearing loss(SNHL)in children Methods: Retrospective chart review, 860 patients with SNHL,of which 125 children(225 ears) with inner ear malformations diagnosed with temporal bone computed tomography (CT) scans who referred to the Ear, Nose and Throat (ENT) Department of Children Medical Center from February 2005 to January 2010,109 cases due to hearing loss and other 3 cases due to external ear malformations, 13 cases due to face abnormality, were retrospectively studied. Results: A total of 109(87.2％) cases were found hearing loss, 52 cases (41.6％)mothers were noted to have caught a cold in first trimester of pregnancy. Hearing loss was typically bilateral in 100 ears(80％ ) ,and profound sensorineural hearing loss in 166 ears (73.8％). Of the total, 3 ears were accompanied by outer and middle ear malformations, 13 ears accompanied by middle ear malformations. Among the 124 patients, 2(4 ears)were found to be Michel deformity, 1 (1 ears) was found to be Cochlear aplasia, 10( 14 ears)were found to be common cavity deformity,2(2 ears)incomplete partition,2(2 ear) cochleovestibular hypoplaisa,19(29 ears)incomplete partition Ⅱ (Mondini deformity),91 (170 ears) accompanied by large vestibular aqueduct, 30(46 ears)semicircular canal deformity,internal auditory canal deformity 35(49ears). Conclusions: The patients with large vestibular aqueduct are more in patients with inner ear malformations than in patients with other inner ear malformations. Hearing loss is typically bilateralor total deafness.CT examinations are of great importance for the diagnosis and treatment of children with inner ear malformations.
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