Presentation
Sensorineural hearing loss
Patient Data
Both cochleae show anomalous form with one and a half turns only, representing incomplete partition between the middle and apical parts. The basal turns are normal in size bilaterally (1.5 mm in cross-section).
Enlarged vestibules are identified with a normal appearance of the semicircular canals depicted.
Dilated both internal auditory canals (6.7 mm on the right side and 5.4 mm on the left side).
An enlarged vestibular aqueduct bilaterally measures about 3.8 mm at the right side and 3.3 mm at the left side.
Various MRI sequences confirm the CT finding of well-appreciated bilateral endolymphatic sac dilatation (4.4 mm on the right side and 5.5 mm on the left side), as well as all the other relevant findings mentioned above.
Case Discussion
The combination of the bilaterally deformed, abnormal cochleae with only 1.5 turns—indicating failure of segmentation between the middle and apical turns, resulting in sac-like cochleae—as well as the enlarged vestibular aqueduct and dilated endolymphatic sac, is consistent with Mondini malformation.
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