搏动性耳鸣的病因有动脉性、静脉性和非血管性因素，鉴于先天性中枢神经系统畸形引起的搏动性耳鸣，以往文献未见记述，该文作者报告2例1型阿一希（Arnold－Chiari）二氏畸形和1例中脑小管狭窄所致的搏动性耳鸣，以上二种先天性畸形通过矢状位MRI检查作出确诊，并证实耳鸣均继 The causes of pulsatile tinnitus are arterial, venous and non-vascular factors. In view of the pulsatile tinnitus caused by congenital central nervous system deformity, which has not been described in the prior literature, the authors report two cases of Arnold- Chiari deformity and one case of middle cerebral tubule stenosis caused by pulsatile tinnitus, the above two congenital malformations by sagittal MRI examination confirmed and confirmed tinnitus are following