Introduction:

Vestibulo Cochlear aplasia is an embryological defect in the formation of the inner ear that causes a sensorineural hearing loss. We report the case of an 8 year-old boy with left sided hearing loss, not previously detected. On neurological examination, a hearing loss in the left ear was found. Through imaging studies, the diagnosis is confirmed.

Conclusion:

When hearing loss is suspected, imaging studies are the ideal method for detecting pathological findings in the inner ear.