Presentation
Right sensorineural hearing loss since birth.
Patient Data
Age: Adult
Gender: Male
From the case:
Cochlear hypoplasia
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Stenotic right internal auditory canal. Cystic right cochlear apex with only 1.5 turns. The cochlear aperture is narrow and hypoplastic.
Hypoplastic right vestibule with an absent lateral semicircular canal.
Both vestibular aqueducts are normal.
The bony facial canals are well seen on both sides being relatively narrower on the right side.
Traces of mucosal thickening are seen at both maxillary and ethmoidal sinuses.
Case Discussion
This case demonstrates cochlear hypoplasia as one of the inner ear malformations that consist of only one turn or a partial cochlear turn, abnormally small internal acoustic canal; the vestibule and semicircular canals are usually malformed.
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