Presentation
Profound unilateral left-sided sensorineural hearing loss.
Patient Data
Age: 8 weeks
Gender: Male
From the case:
Cochlear aplasia
{"current_user":null,"step_through_annotations":true,"access":{"can_edit":false,"can_download":true,"can_toggle_annotations":true,"can_feature":false,"can_examine_pipeline_reports":false,"can_pin":false},"extraPropsURL":"/studies/78999/annotated_viewer_json?iframe=true\u0026lang=us"}
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.