Cochlear aplasia

Case contributed by Dr Jane McEniery

Presentation

Profound unilateral left-sided sensorineural hearing loss.

Patient Data

Age: 8 weeks
Gender: Male
MRI

The left cochlea is absent. The left vestibule and semicircular canals are dysplastic, with a globular appearance.  The left cochlear nerve is absent, and the left internal acoustic meatus is smaller than the right. The right inner ear structures have a normal appearance. The right facial nerve and vestibulocochlear nerves appear normal.

Case Discussion

Cochlear aplasia is a rare entity, however the imaging findings are characteristic.  

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Case information

rID: 69218
Published: 29th Apr 2020
Last edited: 29th Apr 2020
Inclusion in quiz mode: Included

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