Abstract: Objective To evaluate the surgical method of Mondini dysplasia with cerebrospinal otorrhea via translabyrithine approach. Methods Two patients were diagnosed having Mondini dysplasia accompanied with defection of fundus of intenal acoustic canal (IAC) using CT scan and MRI. Both patients received surgical treatment via translabyrinthine approach to repair cerebrospinal otorrhea with muscle and fibrin glue. Results In the two patients the leakage stopped after the first surgical closure. After followingup for 6 months postoperatively, neither of them recurred. Conclusion Patients with hearing loss and recurrence of meningitis should be highly suspicious of Mondini dysplasia with spontaneous cerebrospinal otorrhea. The diagnosis mainly depends on symptoms, CT scan, MRI, and neurological test.Cerebrospinal otorrhea accompanied with Mondini dysplasia can be surgically treated via translabyrinthine approach. 