伴有大前庭水管的突发性聋(附病例报告及文献复习)

Sudden deafness with large vestibular aqueduct (case report and literatures review)

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 study the clinical feature of large vestibular aqueduct syndrome and diagnostic basis of large vestibular aqueduct on high resolution computer tomography. Method: Report a 18 year old man who presented a sudden deafness in right ear and his left ear arised the same disorder before 11 months. Since childhood his both ears have occurred sensorineural hearing loss, which causations were reserved unknown. The temporal bone axially oriented high resolution computer tomography scanning showed a enlarged vestibular aqueduct without associated cochlear anomalies of both ears. In addition the literatures about this disorder are reviewed. Result: The large vestibular aqueduct syndrome without association cochlear anomalies may occurs acquired sensorineural hearing loss and appears to be at high risk for sudden deafness. Conclusion: It is important to identify followed by high-resolution computer tomography whether a large vestibular aqueduct syndrome when a causal uncertain sensorineural hearing loss or/and a sudden deafness onset in childhood is represented.