Lee Seung Young, Cha Sang-Hoon, Jeon Min Hee, Bae Il Heon, Han Gi Seok, Kim Sung Jin, Park Kil Sun
Department of Radiology, Chungbuk National University Hospital, 410 Seongbong-ro, Heungduk-gu, Cheongju, Chungbuk 361-711, Korea.
J Comput Assist Tomogr. 2009 Jul-Aug;33(4):565-70. doi: 10.1097/RCT.0b013e31818d8ba5.
The narrow duplicated internal auditory canal (IAC) is a very rare malformation, and there is no report about the narrow triplicated IAC in the literature. It has been believed to be associated with congenital sensorineural hearing loss and to be a result of aplasia or hypoplasia of the vestibulocochlear nerve or the cochlear branch. We present 2 cases of unilateral narrow duplicated IAC and 1 case of unilateral narrow triplicated IAC with ipsilateral sensorineural hearing loss. The separated, accessory bony canals delineated on high-resolution computed tomography do not mean the presence of nerve fibers. In previously presented cases and in our case, the separated small canals were vacant and without a cochlear nerve fiber. We present new common radiological findings as follows: the bony canal to the cochlea is very thin and the connection with the modiolus of the cochlea is interrupted in narrow separated IAC.
狭窄的重复内耳道(IAC)是一种非常罕见的畸形,文献中尚无关于狭窄的三重IAC的报道。人们一直认为它与先天性感音神经性听力损失有关,是前庭蜗神经或蜗神经分支发育不全或发育不良的结果。我们报告2例单侧狭窄重复IAC和1例单侧狭窄三重IAC伴同侧感音神经性听力损失。高分辨率计算机断层扫描显示的分离的副骨管并不意味着存在神经纤维。在之前报道的病例以及我们的病例中,分离的小管道是空的,没有蜗神经纤维。我们提出如下新发现:在狭窄分离的IAC中,通向耳蜗的骨管非常薄,且与耳蜗蜗轴的连接中断。