Mondini Dysplasia

Abstract
Mondini dysplasia is characterized by a short flat cochlea, large vestibule, wide, small or missing semicircular canals, and immature sensorineural structures. It may occur in association with anomalies of other organs or it may occur in isolation. In some cases the footplate of the stapes is defective, leading to spontaneous perilymphatic fistula and meningitis.