儿童单侧感音性听力损失的听力学及影像学特征149例分析

Analysis of audiological and imaging characteristics of 149 children with unilateral sensorineural hearing loss

目的 探讨儿童单侧感音性听力损失的听力学和影像学表现,分析其病因。方法 回顾性分析149例1月龄~14岁的单侧感音性听力损失患者的就诊途径、听力学和颞骨影像学检查结果。结果 149例患者95.97%为重度、极重度听力损失,即单侧聋。38.26%就诊患儿是由于听力筛查未通过被发现,20.13%患儿自觉单侧听力差就诊发现。影像学检查正常47例,异常102例,其中内耳结构/蜗神经发育异常96例(64.43%),颞骨骨折6例。结论 儿童单侧感音性听力损失多为重度及极重度聋,影像学检查发现蜗神经发育异常及内耳发育异常比例较高,可认为儿童单侧感音性听力损失与先天发育异常关系密切,因此儿童单侧感音性听力损失患儿应常规行影像学检查以明确病因。

Objective To analyze the clinical audiological data and imaging manifestations of unilateral sensorineural hearing loss(USHL) in children, and explore its etiology. Methods A total of 149 patients with USHL, aged from one month to 14 years, were analyzed retrospectively. Results 95.97% of the analyzed patients had severe or extremely severe hearing loss, namely single-sided deafness. 38.26% of the patients with USHL were detected through hearing examinations after their hearing screenings were not passed suffered from unilateral hearing loss as per the findings 20.13% children were found by perceiving unicat eral hearing loss. No obvious imaging abnormalities were found in 47 patients. Among the other 102 patients with imaging abnormalities, one had complete dysplasia(Michel malformation), one had dysplasia of the cochlea, and three had common cavity malformation. Two patients had dysplasia of the cochlea and seven patients had dysplasia of the cochlea. These included four patients with IP-Ⅰ malformation, two with IP-Ⅱ malformation, and one with IP-Ⅲ malformation. Seven patients had vestibular aqueduct enlargement, while 28 patients had bony internal auditory canal stenosis or cochlear foramina stenosis. One patient had internal auditory canal enlargement, while 46 patients had cochlear dysplasia, including 10 patients with cochlear nerve absence and 26 with cochlear nerve tenaciousness. Conclusion Most children with USHL experience severe and extremely severe deafness. The proportion of cochlear neurodysplasia and cochlear dysplasia are high in children with USHL. It is also closely related to congenital dysplasia. Therefore, routine temporal bone imaging examinations should be performed for children with USHL.