Summary

A 30-year-old man with a 10-year history of neurofibromatosis type 2 (NF-2) and minimal hearing in his left ear, presented with rapidly progressive disease and risk of total hearing loss. He was started on imatinib and achieved stable disease for 4 months, after which the drug was ceased due to toxicity. He was then treated with bevacizumab for 8 months with a best response of stable disease.