Demir Ozgün Ilhan, Cakmakci Handan, Erdag Taner Kemal, Men Süleyman
Department of Radiology, Faculty of Medicine, Dokuz Eylül University, 35340 Balçova, Izmir, Turkey.
Pediatr Radiol. 2005 Dec;35(12):1220-3. doi: 10.1007/s00247-005-1547-y. Epub 2005 Aug 4.
Narrow duplicated internal auditory canal (IAC) is a rare malformation of the temporal bone that is associated with ipsilateral congenital sensorineural hearing loss. This may be an isolated finding or a part of a syndrome. Radiological examination should demonstrate aplasia or hypoplasia of the neural components of the narrow IAC, to guide the surgical approach. We report a 7-year-old boy with Klippel-Feil syndrome with a narrow double IAC with no sensorineural hearing loss but with conductive hearing loss. In this patient, the IAC consisted of two separate narrow bony canals clearly seen on 3D temporal bone CT and one nerve that was delineated on MRI. The contralateral external auditory canal was stenotic and the ossicles were dysplastic.
狭窄重复的内耳道（IAC）是一种罕见的颞骨畸形，与同侧先天性感音神经性听力损失相关。这可能是一个孤立的发现，也可能是综合征的一部分。影像学检查应显示狭窄IAC神经成分的发育不全或发育不良，以指导手术方法。我们报告一名7岁患有Klippel-Feil综合征的男孩，其双侧IAC狭窄但无感音神经性听力损失，仅有传导性听力损失。在该患者中，IAC由在三维颞骨CT上清晰可见的两条独立狭窄骨管和在MRI上显示的一条神经组成。对侧外耳道狭窄，听小骨发育异常。
