Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2.

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Published in Am J Med Genet A on December 02, 2011

Authors

Jaishri O Blakeley1, D Gareth Evans, John Adler, Derald Brackmann, Ruihong Chen, Rosalie E Ferner, C Oliver Hanemann, Gordon Harris, Susan M Huson, Abraham Jacob, Michel Kalamarides, Matthias A Karajannis, Bruce R Korf, Victor-Felix Mautner, Andrea I McClatchey, Harry Miao, Scott R Plotkin, William Slattery, Anat O Stemmer-Rachamimov, D Bradley Welling, Patrick Y Wen, Brigitte Widemann, Kim Hunter-Schaedle, Marco Giovannini

Author Affiliations

1: Johns Hopkins University, Baltimore, Maryland 21204, USA. jblakel3@jhmi.edu

Associated clinical trials:

Concentration and Activity of Lapatinib in Vestibular Schwannomas | NCT00863122

Bevacizumab for Symptomatic Vestibular Schwannoma in Neurofibromatosis Type 2 (NF2) | NCT01207687

Lapatinib Study for Children and Adults With Neurofibromatosis Type 2 (NF2) and NF2-Related Tumors | NCT00973739

A Study of Nilotinib in Growing Vestibular Schwannomas | NCT01201538

PTC299 for Treatment of Neurofibromatosis Type 2 | NCT00911248

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Committee on Hearing and Equilibrium guidelines for the evaluation of hearing preservation in acoustic neuroma (vestibular schwannoma). American Academy of Otolaryngology-Head and Neck Surgery Foundation, INC. Otolaryngol Head Neck Surg (1995) 2.08

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