Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

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

Nature Clinical Practice Oncology volume 5, pages 487–491 (2008)Cite this article

Abstract

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.

This is a preview of subscription content, access via your institution

Relevant articles

Open Access articles citing this article.

Access options

Rent or buy this article

Get just this article for as long as you need it

$39.95

Learn more

Prices may be subject to local taxes which are calculated during checkout

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

References

  1. Curto M et al. (2007) Contact-dependent inhibition of EGFR signaling by Nf2/Merlin. J Cell Biol 177: 893–903

    Article  CAS  Google Scholar 

  2. Sorensen AG et al. (2001) Comparison of diameter and perimeter methods for tumor volume calculation. J Clin Oncol 19: 551–557

    Article  CAS  Google Scholar 

  3. American National Standards Institute (2004) Methods for manual pure-tone threshold audiometry. S3.21 New York: ANSI

  4. Boothroyd A and Calkwell S (1981) Vibrotactile thresholds in pure-tone audiometry. In Hearing Measurement: a Book of Readings, edn 2 134–139 (Eds Chaiklin JB. et al.) New York: Addison Wesley

    Google Scholar 

  5. Halpin C: Measuring audiometric outcomes. In Evidence Based Otolaryngology 227–238 (Eds Shin J. et al.) New York: Springer, in press

    Chapter  Google Scholar 

  6. Owens E et al. (1982) Interim assessment of candidates for cochlear implants. Arch Otolaryngol 108: 478–483

    Article  CAS  Google Scholar 

  7. Boothroyd A et al. (1988) Voice fundamental frequency as an auditory supplement to the speechreading of sentences. Ear Hear 9: 306–312

    Article  CAS  Google Scholar 

  8. Farrell CJ and Plotkin SR (2007) Genetic causes of brain tumors: neurofibromatosis, tuberous sclerosis, von Hippel–Lindau, and other syndromes. Neurol Clin 25: 925–946

    Article  Google Scholar 

  9. Evans DG et al. (2005) Incidence of vestibular schwannoma and neurofibromatosis 2 in the North West of England over a 10 year period: higher incidence than previously thought. Otol Neurotol 26: 93–97

    PubMed  Google Scholar 

  10. Mulvihill JJ et al. (1990) NIH conference. Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis). An update. Ann Intern Med 113: 39–52

    Article  CAS  Google Scholar 

  11. National Institutes of Health Consensus Development Conference (1988) Neurofibromatosis. Conference statement. Arch Neurol 45: 575–578

  12. Baser ME et al. (2002) Evaluation of clinical diagnostic criteria for neurofibromatosis 2. Neurology 59: 1759–1765

    Article  CAS  Google Scholar 

  13. Evans DG et al. (2005) Management of the patient and family with neurofibromatosis 2: a consensus conference statement. Br J Neurosurg 19: 5–12

    Article  CAS  Google Scholar 

  14. Otto SR et al. (2002) Multichannel auditory brainstem implant: update on performance in 61 patients. J Neurosurg 96: 1063–1071

    Article  Google Scholar 

  15. Mathieu D et al. (2007) Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates. Neurosurgery 60: 460–468

    Article  Google Scholar 

  16. Rowe JG et al. (2003) Clinical experience with gamma knife stereotactic radiosurgery in the management of vestibular schwannomas secondary to type 2 neurofibromatosis. J Neurol Neurosurg Psychiatry 74: 1288–1293

    Article  CAS  Google Scholar 

  17. Evans DG et al. (2006) Malignant transformation and new primary tumours after therapeutic radiation for benign disease: substantial risks in certain tumour prone syndromes. J Med Genet 43: 289–294

    Article  CAS  Google Scholar 

  18. Shepherd FA et al. (2005) Erlotinib in previously treated non-small-cell lung cancer. N Engl J Med 353: 123–132

    Article  CAS  Google Scholar 

Download references

Acknowledgements

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.

Author information

Authors and Affiliations

  1. SR Plotkin is Assistant Professor of Neurology and Director of the Neurofibromatosis Clinic, MA Singh is family Nurse Practitioner and Research Nurse, and CC O'Donnell is the Clinical Research Coordinator, at the Neurofibromatosis Clinic, Massachusetts General Hospital.,

    Scott R Plotkin, Marybeth A Singh & Caroline C O'Donnell

  2. GJ Harris is Associate Professor of Radiology at Harvard Medical School, Director of the 3D-imaging Service at Massachusetts General Hospital, and Director of the Tumor Imaging Metrics Core for the Dana-Farber/Harvard Cancer Center.,

    Gordon J Harris

  3. and a member of the Department of Audiology at the Massachusetts Eye and Ear Infirmary, AI McClatchey is an Associate Professor of Pathology at Harvard Medical School and Massachusetts General Hospital, and C Halpin is Assistant Professor of Otology and Laryngology at Harvard Medical School, Boston, MA, USA.,

    Andrea I McClatchey & Chris Halpin

Authors
  1. Scott R Plotkin
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marybeth A Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Caroline C O'Donnell
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Gordon J Harris
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Andrea I McClatchey
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Chris Halpin
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Scott R Plotkin.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

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). https://doi.org/10.1038/ncponc1157

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncponc1157

This article is cited by

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing