Bilateral Mondini malformations

Case contributed by Frank Gaillard
Diagnosis certain

CT through the temporal bones demonstrates a number of abnormalities of the labyrinth. The cochlea demonstrates only 1.5 turns. The basal turn is intact, however the more apical turns are absent replaced by a single cystic cavity. The vestibule is enlarged with normal semicircular canals and the vestibular aqueduct is enlarged.

Case Discussion

These features are characteristic of the Mondini malformation.

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