大前庭导水管综合征9例临床分析

Clinical analysis of 9 cases of large vestibular aqueduct syndrome

目的:探讨大前庭导水管综合征(LVAS)的诊断、听力学表现及治疗。方法:回顾分析我科2000至2005年9例LVAS的病史、听力学及前庭功能检查、影像学检查及治疗结果。结果:9例均为双耳发病,并呈进行性听力下降,8例纯音测听虽以感音神经性聋为主,但低频段存在一定的气骨导差,保守治疗远期效果不理想。结论:对于儿童不明原因的听力下降,首先应排除先天性疾病,合并其他因素造成的听力下降会增加LVAS诊断的难度。对LVAS进行性听力下降者应尽早选配助听器,并考虑行人工耳蜗植入术。

Objective： To explore the diagnosis, hearing and treatment of large vestibular aqueduct syndrome （LVAS）. Method： A retrospective analysis was conducted on the history, auditory and vestibular function, imaging examination, and treatment in 9 cases from 2000 to 2005. Results： All 9 cases presented bilateral large vestibular aqueduct syndrome, and showed progressive hearing loss. Although sensorineural hearing loss was predominant, conductive hearing loss existed in low-tone frequencies in 8 cases. Long-term results of conservative treatment were not ideal. Conclusions： Hearing loss in children with uncertain reasons should be excluded from congenital diseases. Other factors combined can increase difficulties in diagnosis of LVAS. Hearing-aids should be used in patients with progressive hearing loss as early as possible and cochlear implantation should be considered.