Unilateral sensorineural hearing loss is a common symptom of vestibular schwannomas
in adolescent patients with neurofibromatosis type 2 or sporadic vestibular schwannomas
and is often the initial clinical feature. While rare cases of sensorineural impairment
presenting as vision or hearing loss due to metastatic medulloblastoma are known,
hearing loss as an isolated presenting symptom of primary malignant neuroepithelial
tumors of the central nervous system has not been reported in the pediatric population
so far. We present two adolescents with unilateral hearing loss due to cochlear nerve
dysfunction as the only symptom of a primary nonmetastatic medulloblastoma of the
WNT signaling pathway family members subgroup.
Keywords
unilateral hearing loss - medulloblastoma - MRI
