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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 composition, imaging and clinical audiology of congenital inner ear deformities in infants and children with congenital sensorineural hearing loss (SNHL). Methods: A retrospective analysis was performed on 860 cases of congenital SNHL patients diagnosed and treated by Department of Otorhinolaryngology, Shanghai Children’s Medical Center affiliated to Shanghai Jiao Tong University School of Medicine. The congenital deformities of the inner ear were found by high resolution CT and MRI of the temporal bone The audiology and imaging data of 125 cases (225 ears) of children. Results: Among 860 children with congenital SNHL, 14.5% had congenital malformation of the inner ear. 98 cases (78.4%) were bilateral and 27 cases (21.6%) were unilateral. There were 167 ears (74.2%) in 225 ears with severe deafness, 36 ears (16%) with severe deafness and 22 ears (9.8%) with moderate deafness. In the group of inner ear deformities, vestibular aqueduct was the most common (75.6%), followed by vestibular deformity (32%) and again cochlear vestibular deformity (23.1%). The Mondini deformity was the most common (55.8%) in the cochlear vestibular deformity, followed by the common cavity deformity (28.9%). Very severe deafness in the inner ear deformity involving the cochlea was significantly more than those in the inner ear deformity without involvement of the cochlea. CONCLUSION: To understand the congenital deformity of congenital inner ear in congenital SNHL of infants and children in China is of great significance to the etiological diagnosis of congenital SNHL and the formulation of intervention strategies including hearing aid, cochlear implantation and its prognosis.