大前庭导水管综合征

目的:探讨大前庭导水管综合征的发病、临床表现及防治措施。方法:对18例经高分辨率CT证实为双侧大前庭导水管综合征患者的诊疗过程进行回顾性分析。结果:同一患者，管径大患耳较管径小患耳的听力损失重；不同患者管径大小与听力损失程度无关。耳声发射测定结果波动大，经颞骨CT扫描证实均为大前庭导水管综合征。结论:大前庭导水管综合征是一种独立的疾病，极易误诊，对临床上有波动性进行性听力下降、轻微的头颅外伤引起严重的听力下降的患者或声发射测定结果波动较大的感音神经性聋患者,应常规进行颞骨高分辨率CT扫描,以明确诊断，对已经证实的患者应积极进行干预治疗，尽可能地保存患者的残余听力。

Large vestibular aqueduct syndrome

ABSTRACT］Objective: To explore the clinical manifestation, prevention and treatment of large vestbular aqueduct syndrome （LVAS）. Methods: The clinical process of diagnosis and treatment of 18 patients with LVAS confirmed by high resolution CT were retrospectively analyzed. Results: The large caliber resulted in more hearing loss of the ear than the small one in the same patient; for different patients, the caliber size had no relationship with the hearing loss; the results of otoacoustic emission（OAE） was unstable; all patients were proved to have LVAS by CT scan of the temporal bone. Conclusions: The LVAS may be often misdiagnosed. The patients with fluctuative and progressive hearing loss should be examined by high resolution CT scan of the temporal bone. Early diagnosis plays an important role in preserving the patients′ remaining hearing.