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目的:探讨不伴耳蜗畸形的大前庭水管综合征的临床表现和高分辨率CT对大前庭管的诊断。方法:报告1例18岁男性患者从2岁时开始患双耳感音神经性听力损失,2001年1月和11月两耳先后出现突发性聋;颞骨轴位高分辨率CT扫描示双侧前庭水管扩大,不伴耳蜗畸形。此外并进行了文献复习。结果:不伴耳蜗畸形的大前庭水管综合征可出现后天性感音神经性听力损失,并有突发性聋的危险。结论:对儿童时期发生的不明原因的感音神经性听力损失或(和)突发性聋,应行高分辨率CT扫描,以了解是否存在不伴耳蜗畸形的大前庭水管综合征。
Objective: To investigate the clinical manifestations of large vestibular aqueduct syndrome without cochlear malformation and the diagnosis of large vestibular canal with high resolution CT. METHODS: One 18-year-old male patient was reported to have binaural sensorineural hearing loss starting at 2 years of age. Sudden deafness occurred in both ears in January and November 2001, and a high resolution CT scan of the temporal bone axis showed double Side of the vestibular water pipes to expand, not with cochlea deformity. In addition, a literature review was conducted. Results: Large vestibular aqueduct syndrome without cochlear malformation may cause acquired sensorineural hearing loss, and there is the risk of sudden deafness. CONCLUSION: High-resolution CT scans of unexplained sensorineural hearing loss or sudden deafness during childhood should be performed to understand the presence or absence of a large vestibular aqueduct syndrome without cochlear deformity.