先天性内耳发育畸形的高分辨率CT表现

Appearance of high resolution CT in congenital inner ear malformations

目的探讨先天性内耳畸形的高分辨率CT表现。方法回顾性分析29例(46耳)婴幼儿先天性内耳发育畸形患者的CT表现,所有患者均做多层螺旋高分辨率CT横断面扫描及多平面重建,必要者利用容积再现技术对骨迷路进行三维重建。结果29例先天性内耳发育畸形患者双侧畸形者17例,单侧畸形者12例,内耳发育畸形共计46耳。具体分布如下:(1)Michel型(2耳),(2)耳蜗未发育(6耳),(3)共同腔畸形(5耳),(4)不完全分隔Ⅰ型(5耳),(5)不完全分隔Ⅱ型(传统Mondini型)(17耳),(6)耳蜗形态正常仅前庭及(或)半规管畸形(9耳),(7)单纯内耳道畸形(2耳)。结论高分辨率CT对先天性内耳骨迷路畸形具有重要的诊断价值,并可为人工耳蜗植入术适应证的选择提供重要依据。

Objective： To discuss the high resolution CT （HRCT） appearance of the congenital inner ear malformations. Methods ： The HRCT appearance of 29 cases （46 ears） with congenital inner ear malformations were analyzed retrospectively. The axial images and multi - planar reconstruction were performed in each patient, and the 3D reconstruction of inner ear labrinth were done with volume rendering technique. Results： Seventeen patients were bilateral inner ear malformations among 29 patients. There are 46 ears with inner ear malformations. The malformations were （1） Michel deformity （2 ear） , （2） cochlear aplasia （6 ears） , （3） common cavity deformity （5 ears）, （4） incomplete partition Ⅰ（5 ears）, （5） incomplete partition Ⅱ （traditional Mondini deformity） （ 17 ears）, （6） vestibular and/or semicircular canal malformaitions （9 ears）, （7） internal auditory canal malformations （2 ears）. Conclusion ： HRCT is very useful to the diagnosis of the cingenital inner ear malformations, and can provide an important evidence for the cochlear implantation.