Imaging of the inner ear structures is becoming essential 
nowadays as the increasing number of cases of cochlear implants 
being performed globally. We report here a case of a 19-year-old 
boy who had congenital hearing loss and was being evaluated in 
our service for possible cochlear implantation. Audiometry 
showed profound mixed sensorineural hearing loss of the right 
side and mild to moderate conductive hearing loss in the left side. 
The magnetic resonance imaging revealed only two nerves in the 
narrowed right inner auditory canal: one in the anterior superior 
quadrant, identified as the facial nerve, and one on the posterior 
quadrants, representing the incomplete separation of the superior 
and inferior vestibular nerves which is an anatomical variant. 
Bilateral lateral semicircular canals were not visualized and there 
is dysplastic vestibules morphology. The diagnosis for the patient 
was right cochlear nerve agenesis with bilateral lateral 
semicircular canals aplasia.