Neurofibromatosis type 2 vestibular schwannoma treatment: a review of the literature, trends, and outcomes.

OBJECTIVE: Review the useful hearing preservation and tumor control outcomes of microsurgery (MS), stereotactic radiation (SR), conservative management (CM), and chemotherapy (ChT) for Neurofibromatosis type 2 vestibular schwannomas. DATA SOURCES: Ovid MEDLINE was used to conduct a thorough search of English-language publications dating from 1948 to February 2013. STUDY SELECTION: Patients must have had useful hearing at diagnosis and treated with one of the 4 treatments as their primary therapy. All sporadic vestibular schwannoma cases were excluded. DATA EXTRACTION: A total of 19 articles were reviewed. Hearing preservation was defined as unchanged or improved useful hearing. Tumor control was defined as no change in size or tumor regression for SR, CM, and ChT-treated cases, and as no recurrence for MS treated cases. CONCLUSION: Microsurgery seems to have the worse overall results, while SR has very good tumor control with poor useful hearing preservation. If a patient qualifies for CM, he is likely to show the most satisfactory results with the least treatment available. A close follow-up on ChT clinical trials and possible side-effects is warranted as preliminary short-term results are quite favorable. Additional long-term studies are required for a better understanding of this disease.