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目的评估大前庭水管综合征伴随其他内耳异常的特征及其发生率。资料与方法采用64层螺旋CT对168例大前庭水管综合征患者采集颞骨数据并进行多平面重组,然后对内耳异常表现进行评估。结果CT显示的168例332耳大前庭水管综合征中,46耳(13.9%)为单纯大前庭水管,286耳(86.1%)伴有其他内耳异常。其中69.9%(232耳)伴有蜗轴发育不良,3%(10耳)伴有蜗轴缺如,29.8%(99耳)伴有阶间隔发育不全Ⅱ型,73.2%(243耳)伴有前庭扩大,53.6%(178耳)伴有外半规管发育不良。结论大多数大前庭水管综合征均伴有其他轻至重度内耳异常。高分辨率CT可较准确地对其进行评估。
Objective To evaluate the characteristics and incidence of abnormalities of the inner ear caused by the large vestibular aqueduct syndrome. Materials and Methods The 64-slice spiral CT was used to collect temporal bone data from 168 patients with large vestibular aqueduct syndrome and multiplanar reorganization. Then the abnormalities of the inner ear were evaluated. Results In 168 cases of 332 large vestibular aqueduct syndromes, 46 ears (13.9%) were pure vestibular aqueduct and 286 ears (86.1%) were accompanied by other internal ear abnormalities. Among them, 69.9% (232 ears) had hypoplasia of the worm shaft, 3% (10 ears) with absence of the worm shaft, 29.8% (99 ears) with type Ⅱ septal insufficiency, 73.2% (243 ears) Vestibular enlargement, 53.6% (178 ears) with lateral semicircular canal dysplasia. Conclusions Most major vestibular aqueduct syndrome is associated with other mild to severe internal ear abnormalities. High-resolution CT can be more accurately assessed.