title: 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
  
  
  METHODS
  Prospective study.
  
  
  METHODS
  Data were collected at 10 NF2 centers, including hospital-based, academic, and tertiary care centers.
  
  
  METHODS
  120 NF2 patients with 200 vestibular schwannomas.
  
  
  METHODS
  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).
  
  
  CONCLUSIONS
  These data provide potentially useful information for the design of clinical trials for NF2 vestibular schwannoma.
authors: *Department of Neurology and Cancer Center, †Biostatistics Center, and ‡Neurosurgical Service, Massachusetts General Hospital, Boston, Massachusetts; and §Neurotology, House Clinic, Los Angeles, California, U.S.A.
authors: Scott R
authors: SR
authors: Plotkin
authors: Vanessa L
authors: VL
authors: Merker
authors: Alona
authors: A
authors: Muzikansky
authors: Fred G
authors: FG
authors: Barker
authors: William
authors: W
authors: Slattery
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D034381:Hearing Loss / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000473:pathology*; D009464:Neuroma, Acoustic / Q000473:pathology*; D011446:Prospective Studies; D055815:Young Adult