Puls T, Van Fraeyenhoven L
Dept. of ENT, Head and Neck Surgery, AZ St.-Elisabeth, Herentals, Belgium.
Acta Otorhinolaryngol Belg. 1997;51(3):185-9.
A case report of a large vestibular aqueduct syndrome (LVAS) as the sole radiographically detectable inner ear anomaly with a mixed hearing loss is presented. Hearing loss in LVAS is sensorineural with a conductive component in 15-27% of the cases. The hearing loss fluctuates and is progressive, resulting in the deterioration of the hearing after minor head trauma. The pathophysiology of the sensorineural hearing loss and the conductive component remains unknown. Endolymphatic sac surgery has been abandoned because of possible adverse results. In our case a complex anomaly was found in the stapes region without noticeable hearing gain after correction. In case of a progressive mixed hearing loss in children with a normal tympanic membrane, a CT-scan is mandatory to rule out LVAS before attempting middle ear surgery that is to no avail.
本文报告一例大前庭导水管综合征（LVAS），其为唯一可通过影像学检测到的内耳异常，并伴有混合性听力损失。LVAS所致听力损失为感音神经性，15% - 27%的病例伴有传导性成分。听力损失呈波动性且进行性加重，轻微头部外伤后听力会恶化。感音神经性听力损失及传导性成分的病理生理学机制尚不清楚。由于可能产生不良后果，内淋巴囊手术已被摒弃。在我们的病例中，镫骨区域发现复杂异常，矫正后听力未显著改善。对于鼓膜正常的儿童出现进行性混合性听力损失，在尝试进行无效的中耳手术前，必须进行CT扫描以排除LVAS。
