Management of neurofibromatosis type 2

Neurofibromatosis type 2 (NF2) is characterized by bilateral eighth cranial nerve schwannomas although some patients may have also other spinal and intracranial tumors. The disease has an autosomal-dominant mode of inheritance. The relationships between the heterogeneity within the NF2 gene mutations and the severity of the phenotype are not yet perfectly defined. The aim of the study is to report on our series of 13 patients with NF2 managed by our two departments in close collaboration. Treatment options have included observation, surgical intervention for complete tumor removal: middle fossa approach, retrosigmoid approach, combined middle fossa and retrosigmoid approach, translabyrinthine approach and translabyrinthine approach with auditory brainstem implantation.