During the past 20 years, there have been significant advances in cochlear implants as the treatment of choice for profoundly hearing-impaired children. The increasing application of cochlear implant has brought with it an increase in the investigational use of computed tomography and magnetic resonance imaging. In this article, the author reviews (a) the anatomy and embryology of the inner ear, (b) the pathological changes associated with congenital sensorineural hearing loss (SNHL), and (c) the spectrum of imaging findings in patients with SNHL.