耳科临床经常遇到一些原因不明的感音神经性耳聋，对此类疾病的诊断伴随听力学以及放射学等学科的进展，近年来不断提高。自１９１７年Ｍｏｎｄｉｎｉ发现有些耳聋是先天性内耳发育不良所致，至今对Ｍｏｎｄｉｎｉ畸形伴前庭导水管扩大的报道很少，现将我们诊断的１例报道... Ear clinical often encounter some unexplained sensorineural deafness, the diagnosis of such diseases with hearing and radiology and other disciplines progress in recent years continue to improve. Mondini found some deafness since 1917 is due to congenital inner ear dysplasia, so far the Mondini malformation with vestibular aqueduct enlargement is rarely reported, we now report a case of diagnosis ...