Sensorineural hearing loss (SNHL) can be caused by dysfunction of cochlea, the vestibulocochlear nerve or central auditory pathways. Imaging of the temporal bone and the inner ear of patients with SNHL has become an important part of evaluation for the presence of developmental anomalies of the inner ear. In this case, we presented a 14-year-old male patient with SNHL total at left side and 50% at right side. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed left sided cochlear dysplasia/hypoplasia with lateral semicircular canal-utriculosaccular cavity. T2-weighted oblique sagittal MR images demonstrated absence of cochlear nerve at the left site. Patient also has a history of meningitis. © 2003 Elsevier Ireland Ltd. All rights reserved.