Congenital defects of the inner ear


 * Anomalies of the inner ear are caused by disorders of otocyst formation . Different types of labyrinth hypoplasia arise due to a disorder in the development of the otocyst.

Mondini anomaly

 * A normal snail has 2.5 turns. In the case of Mondini anomaly, the cochlea has only 1 turn and there is a pathological communication between the scala vestibuli and the scala tympani with simultaneous expansion of the saccus and ductus endolymphaticus.
 * Hearing impairment varies in extent.
 * Diagnosis is mainly based on CT.