Natural History of Vestibular Schwannomas in Neurofibromatosis Type 2 (NF2)

摘要

Neurofibromatosis 2 (NF2) is an autosomal disorder characterized by the development of multiple nervous system tumors such as vestibular schwannomas. The purpose of the study is to define the growth rate and clinical course of vestibular schwannomas in NF2- affected individuals. We developed an international consortium of clinical centers with expertise in NF2, standardized the radiographic analysis of the vestibular schwannomas, assessed the patients' audiological functioning, and analyzed molecular, pathological, and clinical features of the disease over the course of 3 years. With 106 enrolled subjects, we exceeded our target goal of 100 study participants. This was accomplished by recruiting additional study sites and expanding the inclusion criteria. Baseline audiological data and baseline MRI were collected for 76% and 72% of enrolled subjects, respectively. We have follow-up data, representing the largest collection of longitudinal prospectively collected data on NF2 patients.