Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point of care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

For more information, click below.


Subscribers log in here


Vestibular schwannoma (acoustic neuroma)

INTRODUCTION

Vestibular schwannomas (also known as acoustic neuromas, acoustic schwannomas, acoustic neurinomas, or vestibular neurilemomas) are Schwann cell-derived tumors that commonly arise from the vestibular portion of the eighth cranial nerve (figure 1).

Vestibular schwannomas account for approximately 8 percent of intracranial tumors in adults and 80 to 90 percent of tumors of the cerebellopontine angle (CPA). In comparison, they are rare in children, except for patients with neurofibromatosis type 2 (NF2). (See "Neurofibromatosis type 2".)

The epidemiology, pathogenesis, clinical presentation, diagnosis, and management of patients with vestibular schwannomas will be reviewed here.

EPIDEMIOLOGY

The overall incidence of vestibular schwannomas is about one per 100,000 person-years. However, the incidence appears to be increasing, due at least in part to the incidental diagnosis of asymptomatic lesions with the widespread use of magnetic resonance imaging (MRI) and computed tomography [1,2]. As an example, a retrospective analysis of 46,000 MRI scans done for other reasons identified eight unsuspected vestibular schwannomas (0.02 percent) [1], and autopsy studies suggest that the prevalence may be even higher [3,4].

The median age at diagnosis is approximately 50 years [2]. The tumors are unilateral in more than 90 percent of cases [5], affecting the right and left sides with equal frequency. Bilateral vestibular schwannomas are primarily limited to patients with neurofibromatosis type 2 [4]. (See "Neurofibromatosis type 2", section on 'Vestibular schwannomas'.)

                     

Subscribers log in here

To continue reading this article you must have access through your hospital or your group practice, log in to your personal subscription, or purchase a personal subscription. For more information, click below.
Literature review current through: Apr 2012. | This topic last updated: Oct 31, 2011.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use (click here) ©2012 UpToDate, Inc.
References
Top
  1. Lin D, Hegarty JL, Fischbein NJ, Jackler RK. The prevalence of "incidental" acoustic neuroma. Arch Otolaryngol Head Neck Surg 2005; 131:241.
  2. Propp JM, McCarthy BJ, Davis FG, Preston-Martin S. Descriptive epidemiology of vestibular schwannomas. Neuro Oncol 2006; 8:1.
  3. Tos M, Stangerup SE, Cayé-Thomasen P, et al. What is the real incidence of vestibular schwannoma? Arch Otolaryngol Head Neck Surg 2004; 130:216.
  4. Eldridge R, Parry D. Vestibular schwannoma (acoustic neuroma). Consensus development conference. Neurosurgery 1992; 30:962.
  5. Edwards CG, Schwartzbaum JA, Lönn S, et al. Exposure to loud noise and risk of acoustic neuroma. Am J Epidemiol 2006; 163:327.
  6. Sughrue ME, Yeung AH, Rutkowski MJ, et al. Molecular biology of familial and sporadic vestibular schwannomas: implications for novel therapeutics. J Neurosurg 2011; 114:359.
  7. Preston-Martin S, Thomas DC, Wright WE, Henderson BE. Noise trauma in the aetiology of acoustic neuromas in men in Los Angeles County, 1978-1985. Br J Cancer 1989; 59:783.
  8. Preston-Martin S, Mack WJ. Neoplasms of the nervous system. In: Cancer Epidemiology and Prevention, 2nd, Shottenfeld D, Fraumeni JF (Eds), Oxford University Press, New York 1996.
  9. Ryals BM, Rubel EW. Hair cell regeneration after acoustic trauma in adult Coturnix quail. Science 1988; 240:1774.
  10. Hamernik RP, Turrentine G, Roberto M, et al. Anatomical correlates of impulse noise-induced mechanical damage in the cochlea. Hear Res 1984; 13:229.
  11. Schneider AB, Ron E, Lubin J, et al. Acoustic neuromas following childhood radiation treatment for benign conditions of the head and neck. Neuro Oncol 2008; 10:73.
  12. Shore-Freedman E, Abrahams C, Recant W, Schneider AB. Neurilemomas and salivary gland tumors of the head and neck following childhood irradiation. Cancer 1983; 51:2159.
  13. Bäcklund LM, Grandér D, Brandt L, et al. Parathyroid adenoma and primary CNS tumors. Int J Cancer 2005; 113:866.
  14. Rouleau GA, Merel P, Lutchman M, et al. Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature 1993; 363:515.
  15. Matthies C, Samii M. Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation. Neurosurgery 1997; 40:1.
  16. Doyle KJ. Is there still a role for auditory brainstem response audiometry in the diagnosis of acoustic neuroma? Arch Otolaryngol Head Neck Surg 1999; 125:232.
  17. Wilson DF, Hodgson RS, Gustafson MF, et al. The sensitivity of auditory brainstem response testing in small acoustic neuromas. Laryngoscope 1992; 102:961.
  18. Matthies C, Samii M, Krebs S. Management of vestibular schwannomas (acoustic neuromas): radiological features in 202 cases--their value for diagnosis and their predictive importance. Neurosurgery 1997; 40:469.
  19. Marx SV, Langman AW, Crane RC. Accuracy of fast spin echo magnetic resonance imaging in the diagnosis of vestibular schwannoma. Am J Otolaryngol 1999; 20:211.
  20. Sughrue ME, Kaur R, Rutkowski MJ, et al. Extent of resection and the long-term durability of vestibular schwannoma surgery. J Neurosurg 2011; 114:1218.
  21. Lanman TH, Brackmann DE, Hitselberger WE, Subin B. Report of 190 consecutive cases of large acoustic tumors (vestibular schwannoma) removed via the translabyrinthine approach. J Neurosurg 1999; 90:617.
  22. Barker FG 2nd, Carter BS, Ojemann RG, et al. Surgical excision of acoustic neuroma: patient outcome and provider caseload. Laryngoscope 2003; 113:1332.
  23. Gormley WB, Sekhar LN, Wright DC, et al. Acoustic neuromas: results of current surgical management. Neurosurgery 1997; 41:50.
  24. Samii M, Matthies C. Management of 1000 vestibular schwannomas (acoustic neuromas): surgical management and results with an emphasis on complications and how to avoid them. Neurosurgery 1997; 40:11.
  25. Falcioni M, Mulder JJ, Taibah A, et al. No cerebrospinal fluid leaks in translabyrinthine vestibular schwannoma removal: reappraisal of 200 consecutive patients. Am J Otol 1999; 20:660.
  26. Darrouzet V, Martel J, Enée V, et al. Vestibular schwannoma surgery outcomes: our multidisciplinary experience in 400 cases over 17 years. Laryngoscope 2004; 114:681.
  27. Ohta S, Yokoyama T, Nishizawa S, Uemura K. Regrowth of the residual tumour after acoustic neurinoma surgery. Br J Neurosurg 1998; 12:419.
  28. Cerullo L, Grutsch J, Osterdock R. Recurrence of vestibular (acoustic) schwannomas in surgical patients where preservation of facial and cochlear nerve is the priority. Br J Neurosurg 1998; 12:547.
  29. Samii M, Matthies C. Management of 1000 vestibular schwannomas (acoustic neuromas): the facial nerve--preservation and restitution of function. Neurosurgery 1997; 40:684.
  30. Anderson DE, Leonetti J, Wind JJ, et al. Resection of large vestibular schwannomas: facial nerve preservation in the context of surgical approach and patient-assessed outcome. J Neurosurg 2005; 102:643.
  31. Ryzenman JM, Pensak ML, Tew JM Jr. Headache: a quality of life analysis in a cohort of 1,657 patients undergoing acoustic neuroma surgery, results from the acoustic neuroma association. Laryngoscope 2005; 115:703.
  32. Schankin CJ, Gall C, Straube A. Headache syndromes after acoustic neuroma surgery and their implications for quality of life. Cephalalgia 2009; 29:760.
  33. Sughrue ME, Yang I, Aranda D, et al. Beyond audiofacial morbidity after vestibular schwannoma surgery. J Neurosurg 2011; 114:367.
  34. Murphy ES, Suh JH. Radiotherapy for vestibular schwannomas: a critical review. Int J Radiat Oncol Biol Phys 2011; 79:985.
  35. Lunsford LD, Niranjan A, Flickinger JC, et al. Radiosurgery of vestibular schwannomas: summary of experience in 829 cases. J Neurosurg 2005; 102 Suppl:195.
  36. Link MJ, Cohen PL, Breneman JC, Tew JM Jr. Malignant squamous degeneration of a cerebellopontine angle epidermoid tumor. Case report. J Neurosurg 2002; 97:1237.
  37. Shin M, Ueki K, Kurita H, Kirino T. Malignant transformation of a vestibular schwannoma after gamma knife radiosurgery. Lancet 2002; 360:309.
  38. Kondziolka D, Lunsford LD, McLaughlin MR, Flickinger JC. Long-term outcomes after radiosurgery for acoustic neuromas. N Engl J Med 1998; 339:1426.
  39. Chopra R, Kondziolka D, Niranjan A, et al. Long-term follow-up of acoustic schwannoma radiosurgery with marginal tumor doses of 12 to 13 Gy. Int J Radiat Oncol Biol Phys 2007; 68:845.
  40. Roche PH, Régis J, Devèze A, et al. [Surgical removal of unilateral vestibular schwannomas after failed Gamma Knife radiosurgery]. Neurochirurgie 2004; 50:383.
  41. Subach BR, Kondziolka D, Lunsford LD, et al. Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis Type 2. J Neurosurg 1999; 90:815.
  42. Ito K, Shin M, Matsuzaki M, et al. Risk factors for neurological complications after acoustic neurinoma radiosurgery: refinement from further experiences. Int J Radiat Oncol Biol Phys 2000; 48:75.
  43. Phi JH, Kim DG, Chung HT, et al. Radiosurgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2: tumor control and hearing preservation. Cancer 2009; 115:390.
  44. Combs SE, Welzel T, Schulz-Ertner D, et al. Differences in clinical results after LINAC-based single-dose radiosurgery versus fractionated stereotactic radiotherapy for patients with vestibular schwannomas. Int J Radiat Oncol Biol Phys 2010; 76:193.
  45. Williams JA. Fractionated stereotactic radiotherapy for acoustic neuromas. Int J Radiat Oncol Biol Phys 2002; 54:500.
  46. Koh ES, Millar BA, Ménard C, et al. Fractionated stereotactic radiotherapy for acoustic neuroma: single-institution experience at The Princess Margaret Hospital. Cancer 2007; 109:1203.
  47. Andrews DW, Suarez O, Goldman HW, et al. Stereotactic radiosurgery and fractionated stereotactic radiotherapy for the treatment of acoustic schwannomas: comparative observations of 125 patients treated at one institution. Int J Radiat Oncol Biol Phys 2001; 50:1265.
  48. Kapoor S, Batra S, Carson K, et al. Long-term outcomes of vestibular schwannomas treated with fractionated stereotactic radiotherapy: an institutional experience. Int J Radiat Oncol Biol Phys 2011; 81:647.
  49. Weber DC, Chan AW, Bussiere MR, et al. Proton beam radiosurgery for vestibular schwannoma: tumor control and cranial nerve toxicity. Neurosurgery 2003; 53:577.
  50. Bakkouri WE, Kania RE, Guichard JP, et al. Conservative management of 386 cases of unilateral vestibular schwannoma: tumor growth and consequences for treatment. J Neurosurg 2009; 110:662.
  51. Smouha EE, Yoo M, Mohr K, Davis RP. Conservative management of acoustic neuroma: a meta-analysis and proposed treatment algorithm. Laryngoscope 2005; 115:450.
  52. Pulec JL. Acoustic neuroma surgery in geriatric patients. Ear Nose Throat J 1999; 78:429.
  53. Samii M, Tatagiba M, Matthies C. Acoustic neurinoma in the elderly: factors predictive of postoperative outcome. Neurosurgery 1992; 31:615.
  54. Sughrue ME, Yang I, Aranda D, et al. The natural history of untreated sporadic vestibular schwannomas: a comprehensive review of hearing outcomes. J Neurosurg 2010; 112:163.
  55. Sughrue ME, Kane AJ, Kaur R, et al. A prospective study of hearing preservation in untreated vestibular schwannomas. J Neurosurg 2011; 114:381.