Abstract

Neurofibromatosis type 2 (NF2) is a genetically inherited disorder characterized by the presence of multiple central nervous system tumors most characteristic being vestibular schwannomas with or without peripheral manifestations in the form of cataract or cutaneous neurofibromas. Unlike its type 1 counterpart NF2 is an uncommon disorder. We here describe a classical case of neurofibromatosis type 2 with florid clinical manifestations and characteristic neuroimaging features. We also briefly describe the literature pertaining to this rare disorder. The case also emphasizes the fact that NF2 should be considered in the list of differentials for sensorineural deafness especially when it is bilateral.

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