Levenson M J, Parisier S C, Jacobs M, Edelstein D R
Department of Otolaryngology Head and Neck Surgery, Manhattan Eye, Ear & Throat Hospital, New York, NY 10021.
Arch Otolaryngol Head Neck Surg. 1989 Jan;115(1):54-8. doi: 10.1001/archotol.1989.01860250056026.
The large vestibular aqueduct as an isolated anomaly of the temporal bone has been previously identified radiologically, and its association with sensory neural hearing loss has been recognized. It has not, however, been defined as a distinct clinical entity in children. We studied 12 children, ages 3 to 9 years, with downward-fluctuating progressive high-frequency neurosensory hearing losses whose symptoms were thought to be related to the isolated enlargement of the vestibular aqueduct identified by high-resolution computed tomographic scanning. Previously it had been assumed that a large vestibular aqueduct is a temporal bone dysplasia that is a variant of the Mondini type of deformity and that the associated hearing loss is congenital in nature. Our clinical observations, however, indicate that the hearing loss in children with an isolated enlargement of the vestibular aqueduct is acquired during childhood. The natural history of this progressive deafness is reviewed, and a pathophysiologic hypothesis is presented.
大前庭导水管作为颞骨的一种孤立性异常，此前已通过放射学方法得以确认，并且其与感音神经性听力损失的关联也已得到认可。然而，它在儿童中尚未被定义为一种独特的临床实体。我们研究了12名年龄在3至9岁之间的儿童，他们患有高频感音神经性听力损失且呈进行性波动下降，其症状被认为与高分辨率计算机断层扫描所确定的前庭导水管孤立性扩大有关。此前曾认为大前庭导水管是一种颞骨发育异常，是Mondini型畸形的一种变体，且相关的听力损失本质上是先天性的。然而，我们的临床观察表明，前庭导水管孤立性扩大的儿童的听力损失是在儿童期获得的。本文回顾了这种进行性耳聋的自然病史，并提出了一种病理生理假说。
