Valvassori和Clemis 1978年命名大前庭导水管综合征。本文报导17例(33耳)系未合并其它内耳畸形的,均经X线矢状面断层及轴位高分辨CT检查,测试总脚与外口间中点直径超过2mm者(正常0.4～1.0mm),双耳16例,单耳1例。其前庭导水管(VA)直径达2～6mm,临床表现为双耳渐进性感音性聋。6例生后即表现耳聋;8例5岁前发现耳聋;1例8岁 Valvassori and Clemis 1978 Named Large Vestibular Aqueduct Syndrome. This article reports 17 cases (33 ears) without other deformities of the inner ear, both by X-ray sagittal plane axial and high resolution CT examination, test the total diameter between the foot and the mouth outside the diameter of more than 2mm (normal 0.4 ~ 1.0 mm), 16 cases of binaural and 1 case of monaural. The vestibular aqueduct (VA) diameter of 2 ~ 6mm, the clinical manifestations of binaural sensory hearing loss. 6 cases showed deafness after birth; 8 cases were found deafness before 5 years old; 1 case was 8 years old