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Conservative Management of Bilateral Vestibular Schwannomas in Neurofibromatosis Type 2 Patients: Hearing and Tumor Growth Results

Peyre, Matthieu MD*,‡; Goutagny, Stéphane MD, PhD*,‡; Bah, Alpha MD*,‡; Bernardeschi, Daniele MD‡,§; Larroque, Béatrice MD, PhD; Sterkers, Olivier MD‡,§; Kalamarides, Michel MD, PhD*,‡

doi: 10.1227/NEU.0b013e31828bae28
Research-Human-Clinical Studies: Editor's Choice
Editor's Choice

BACKGROUND: As new treatment modalities develop for the management of vestibular schwannomas (VS) in patients with neurofibromatosis type 2, it remains crucial to ascertain the natural history of the disease.

OBJECTIVE: To determine the relationship between hearing and tumor growth in patients undergoing conservative VS management.

METHODS: Patients harboring bilateral VS with at least 1 year of radiological follow-up were selected. Conservative management was proposed based on the small tumor size and/or serviceable hearing at presentation. Tumor size was calculated by using the 2-component box model and reported as mean tumor diameter. Hearing was evaluated by using pure-tone average and the American Academy of Otololaryngologists and Head and Neck Surgery classification.

RESULTS: Forty-six patients harboring 92 VS were included. The mean clinical and radiological follow-up times were 6.0 and 4.2 years, respectively. The mean tumor diameter was 13 mm at presentation and 20 mm at the end of follow-up. Mean tumor growth rate was 1.8 mm/year. During follow-up, 17 patients (37%) underwent surgery for VS. Surgery-free rate for VS was 88% at 5 years. The number of patients with at least 1 serviceable ear was 39 (85%) at presentation and 34 (74%) at the end of follow-up, including 22 (66%) with binaural serviceable hearing maintained. There was no statistical correlation between tumor growth rate and preservation of serviceable hearing. Tumor growth rates and age at presentation were inversely correlated.

CONCLUSION: This study illustrates the high variability among neurofibromatosis type 2 patients regarding hearing status and VS growth rate and justifies the choice of initial conservative management in selected cases.

ABBREVIATIONS: AAO-HNS, American Academy of Otololaryngologists and Head and Neck Surgery classification

MTD, mean tumor diameter

NF2, neurofibromatosis type 2

PTA, pure-tone average

SDS, speech discrimination score

VS, vestibular schwannomas

*AP-HP, Hôpital Beaujon, Department of Neurosurgery, Clichy, France;

Université Paris 7 - Denis Diderot, Paris, France;

§AP-HP, Hôpital Beaujon, Department of ENT Surgery, Clichy, France;

AP-HP, Hôpital Beaujon, Epidemiology, Biostatistics and Clinical Research Unit, Clichy, France

Correspondence: Michel Kalamarides, MD, PhD, Department of Neurosurgery, APHP, Hopital Beaujon, 100 bvd du general Leclerc, 92110 Clichy, France. E-mail:

Received July 13, 2012

Accepted January 29, 2013

Copyright © by the Congress of Neurological Surgeons