Bilateral Cochlear Nerve Hypoplasia with Vestibular Nerve Agenesis: A Rare Cause of Congenital Sensorineural Hearing Loss

Bilateral cochlear nerve hypoplasia associated with bilateral vestibular nerve agenesis is an exceptionally rare neuroimaging finding and an uncommon cause of congenital sensorineural hearing loss (SNHL). We present the case of a 50-year-old patient with a long-standing history of bilateral hearing loss since childhood, who underwent MRI of the internal auditory canals (IACs) for reassessment. High-resolution T2-weighted MRI demonstrated bilateral cochlear nerve hypoplasia, more pronounced on the right, and complete agenesis of both superior and inferior vestibular nerves bilaterally. This article emphasizes detailed imaging features, clinical significance, diagnostic pitfalls, and differentials. A comprehensive review of updated imaging literature supports recognition and appropriate classification of cochleovestibular nerve anomalies.