Rothschild M A, Wackym P A, Silvers A R, Som P M
Department of Otolaryngology, Mount Sinai Medical Center, New York, NY 10029, USA.
Int J Pediatr Otorhinolaryngol. 1999 Nov 5;50(3):219-24. doi: 10.1016/s0165-5876(99)00236-0.
Congenital primary stenosis of the internal auditory canal (IAC) may exist in isolation or along with a number of other osseous anomalies of the temporal bone. Most of the literature on IAC stenosis is concerned with its effect on the outcome of cochlear implantation (i.e. patients with profound bilateral hearing loss). In addition, some degree of canal asymmetry has been noted in patients with normal hearing, questioning the causal relationship of this finding to deafness. We describe two children with computed tomography (CT) documented severe primary unilateral narrowing of the IAC and an associated ipsilateral sensorineural hearing loss. Typical radiographic findings are described, and the relevant developmental pathology is discussed. The ipsilateral association of stenosis and hearing loss strengthens the link between narrowing of the IAC and deafness.
先天性内耳道（IAC）原发性狭窄可能单独存在，也可能与颞骨的其他一些骨质异常同时出现。关于IAC狭窄的大多数文献都关注其对人工耳蜗植入结果的影响（即双侧极重度听力损失患者）。此外，听力正常的患者也存在一定程度的耳道不对称，这对该发现与耳聋之间的因果关系提出了质疑。我们描述了两名儿童，计算机断层扫描（CT）显示其IAC严重原发性单侧狭窄，并伴有同侧感音神经性听力损失。描述了典型的影像学表现，并讨论了相关的发育病理学。狭窄与听力损失的同侧关联强化了IAC狭窄与耳聋之间的联系。
