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Audiologic and radiographic response of NF2-related vestibular schwannoma to erlotinib therapy


Background A 48-year-old man presented to a neurologist with complaints of bilateral hearing loss and tinnitus. The patient was a member of a large family affected by neurofibromatosis type 2 and first noted hearing loss 10 years before presentation.

Investigations Medical and neurological examination, MRI scan of the brain and spinal cord, pure-tone audiometry, NU–6 monosyllabic word test with phoneme scoring, City University of New York topic-related sentences test, noise/voice test of minimal auditory capability battery.

Diagnosis Progressive neurofibromatosis-type-2-related vestibular schwannomas.

Management Annual cranial MRI and audiology, surgical resection of right vestibular schwannoma, high-power behind-the-ear hearing aid, erlotinib therapy for progressive left vestibular schwannoma.

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Figure 1: Cranial MRI scans of the patient before and after treatment with erlotinib.
Figure 2: Volumetric analysis of bilateral vestibular schwannomas.


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This work was supported in part by grants from the Department of Defense NF050202 and from Neurofibromatosis, Inc., New England (SRP), and by a grant from the Children's Tumor Foundation (GJH). The authors wish to acknowledge the past members of the Massachusetts General Hospital Neurofibromatosis Clinic including Priscilla Short, MD, Mia MacCollin, MD, Catherine Bove, RN, and Robert Martuza, MD, for their care of patients with NF2. Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Correspondence to Scott R Plotkin.

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Plotkin, S., Singh, M., O'Donnell, C. et al. Audiologic and radiographic response of NF2-related vestibular schwannoma to erlotinib therapy. Nat Rev Clin Oncol 5, 487–491 (2008).

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