A pediatric medulloblastoma presenting as isolated sensorineural hearing loss: Case report and review of the literature

Medulloblastoma is the most common pediatric malignant brain tumor and carries a relatively grim prognosis despite recent advances in multimodality therapy. Delays in diagnosis and treatment initiation may contribute to worst outcomes. Signs of increased intracranial pressure and ataxia are known presentations of posterior fossa tumors, but sensorineural hearing loss (SNHL) is a seldom reported symptom. We report the case of a 2-year-old girl who had progressive unilateral SNHL since birth, which was later found to have a posterior fossa medulloblastoma when a head magnetic resonance imaging was ordered following subsequent progression to bilateral hearing impair. We further report our review of 17 additional cases of medulloblastoma presenting with SNHL and their associated head and neck findings. The present study provides insight into the current state of the literature on this rare symptom of a commonly encountered diagnosis, while highlighting the need to consider dedicated brain imaging in pediatric unilateral SNHL when a syndromic etiology or inner ear anomaly is not readily identified on initial investigations.