Objective: To improve the ability to diagnosis the Mondini dysplasis, 48 cases of Mondini dysplasis were reported.
Method: The clinical data of 48 cases were collected. The clinical manifestation and the examination of CT and MRI and treatment of the Mondini dysplasia were discussed.
Result: Most of them were deaf when they were babies. The prolonged speaking and unsteading were important clinical feature.
Conclusion: The diagnosis of the disease is based on the examination of the hight resolution tomography and magnetic resonance image of the temporal bone. Hearing aid is common management to the disease. A part of patients should be considered cochlear implantation.