Inner ear malformation accounts for approximately 20% of reported cases of congenital sensorineural deafness. It results
from developmental arrest in different stages of embryogenesis. Aplasia of one of the semicircular canals with or without cochlear
malformation is well known and has been reported in journals. However; to our knowledge, aplasia of two semicircular canals have
been rarely reported, and for this reason the clinical reflection of this anomaly is poorly understood. This case will be hopefully used
to gain an in-depth understanding of illness and help in the diagnosis and proper management of future cases. In this paper we
present a report concerns a 2-year-old boy with no family history of hearing loss, presenting with bilateral profound sensorineural
hearing loss with no other congenital anomalies. CT scan and MRI shows dysplastic bilateral vestibules, bilateral hypoplastic cochlea
and bilateral lateral and posterior semicircular canal (SCC) aplasia.