DOI: https://doi.org/10.36347/sjmcr.2025.v13i06.016

Pages: 1370-1372

Congenital profound deafness is often linked to significant anomalies of the inner ear and auditory nerve pathways, necessitating detailed imaging prior to cochlear implantation. We present the case of an 8-year-old female patient with bilateral severe hearing loss who underwent magnetic resonance imaging of the brain and temporal bones as part of the preoperative workup. Imaging revealed marked bilateral stenosis of the internal auditory canals with absence of identifiable cochleovestibular nerve fibers, aberrant courses of the facial and vestibular nerves, and enlargement of the vestibular structures, notably the vestibules and lateral semicircular canals, more evident on the right side. No abnormalities were detected in the brain parenchyma, midline, or brainstem. This report highlights the complex anatomical variations encountered in such cases and discusses their critical impact on surgical decision-making and cochlear implant feasibility.