Profound unilateral left-sided sensorineural hearing loss.
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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.
Cochlear aplasia is a rare entity, however the imaging findings are characteristic.