Natural history of vestibular schwannoma growth and hearing decline in newly diagnosed neurofibromatosis type 2 patients.

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
Scott R PlotkinWilliam H Slattery

Abstract

To determine the rate of growth in vestibular schwannomas and the rate of hearing decline in neurofibromatosis type 2 (NF2) patients not undergoing active treatment Prospective study. Data were collected at 10 NF2 centers, including hospital-based, academic, and tertiary care centers. 120 NF2 patients with 200 vestibular schwannomas. Hearing decline, defined as a decrease in word recognition score outside the 95% critical difference compared with baseline, and radiographic progression, defined as a 20% or greater increase in tumor volume compared with baseline. During a total of 313.4 patient-years of follow-up, the rate of hearing decline was 5% at 1 year, 13% at 2 years, and 16% at 3 years; the rate of tumor progression was 31% at 1 year, 64% at 2 years, and 79% at 3 years. For this cohort, the median time to tumor progression (14 mo) was significantly shorter than the median time to hearing decline (62.0 mo). These data provide potentially useful information for the design of clinical trials for NF2 vestibular schwannoma.

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