Journal of Radiology
Open Access

Abstract

We report a case of a newborn with left unilateral sensorineural hearing loss (SNHL) that progressed to profound SNHL by 16 months. The child then presented acutely at age 3 with right sudden profound SNHL, rendering her bilaterally deaf. MRI revealed a mass lesion within the fourth ventricle, determined to be Group-3 medulloblastoma. SNHL has been reported in 14 other cases of medulloblastoma. Out of all patients with reported post-treatment outcomes, 8/13 (61%) had permanent neurological sequelae, including one death and 5/13 (38%) was able to return to previous neurological baseline. This is the youngest and the first case presenting with asymmetric SNHL that progressed into bilateral profound SNHL. Medulloblastoma is an uncommon but important etiology to exclude in cases of progressive unilateral or sudden SNHL. MRI is the imaging of choice in these children to ensure excellent visualization of inner ear and nerve structures and particularly the posterior fossa in order to not miss critical diagnoses that are time sensitive.

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