Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Abstract

Objective: 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

Study Design: Prospective study.

Setting: Data were collected at 10 NF2 centers, including hospital-based, academic, and tertiary care centers.

Patients: 120 NF2 patients with 200 vestibular schwannomas.

Outcome Measures: 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.

Results: 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).

Conclusion: These data provide potentially useful information for the design of clinical trials for NF2 vestibular schwannoma.