The case shows bilateral symmetric inner ear malformations:
- The external contour of the cochlea is normal in size and the cochlea is distinct from the vestibule, but the internal architecture of the cochlea is abnormal. The cochlear apex has a cystic appearance due to absence of the modiolus and interscalar septa at the level of the middle and upper turns. However, the basal turn is, for the most part, partitioned by the interscalar septum. The cochlear aperture/fossette is not enlarged.
- The vestibule is fused with the broad lateral semicircular canal in a single globular cavity. The lateral semicircular canal bony island is absent. The posterior and superior semicircular canals are normally formed.
- The vestibular aqueduct is not enlarged.
- The internal auditory canal is normal in size.
- The facial nerve course is normal.
- The middle ear and mastoid are normally developed.