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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 those with neurofibromatosis. (See "Classification of brain tumors".)

The epidemiology, clinical presentation, diagnosis, and treatment of vestibular schwannomas will be reviewed here.

EPIDEMIOLOGY AND RISK FACTORS

The overall incidence of vestibular schwannomas is about one per 100,000 person-years. However, the incidence appears to be increasing, probably 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 autosomal dominant neurofibromatosis [4].
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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, Caye-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 [see comments]. Neurosurgery 1992; 30:962.
  5. Edwards, CG, Schwartzbaum, JA, Lonn, S, et al. Exposure to loud noise and risk of acoustic neuroma. Am J Epidemiol 2006; 163:327.
  6. Preston-Martin, S, Thomas, DC, Wright, WE, Henderson, BE. Noise trauma in the etiology of acoustic neuromas in men in Los Angeles County, 1978-1985. Br J Cancer 1989; 59:783.
  7. Preston-Martin, S, Mack, WJ. Neoplasms of the nervous system. In: Cancer Epidemiology and Prevention, 2nd ed, Shottenfeld, D, Fraumeni, JF (Eds), Oxford University Press, New York, 1996.
  8. Ryals, BM, Rubel, EW. Hair cell regeneration after acoustic trauma in adult Coturnix quail. Science 1988; 240:1774.
  9. Hamernik, RP, Turrentine, G, Roberto, M, et al. Anatomical correlates of impulse noise-induced mechanical damage in the cochlea. Hear Res 1984; 13:229.
  10. 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.
  11. 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.
  12. Backlund, LM, Grander, D, Brandt, L, et al. Parathyroid adenoma and primary CNS tumors. Int J Cancer 2005; 113:866.
  13. 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.
  14. Matthies, C, Samii, M. Management of 1000 vestibular schwannomas (acoustic neuromas): Clinical presentation. Neurosurgery 1997; 40:1.
  15. 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.
  16. Wilson, DF, Hodgson, RS, Gustafson, MF, et al. The sensitivity of auditory brainstem response testing in small acoustic neuromas [see comments]. Laryngoscope 1992; 102:961.
  17. 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.
  18. 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.
  19. Mirz, F, Jorgensen, B, Fiirgaard, B, et al. Investigations into the natural history of vestibular schwannomas. Clin Otolaryngol 1999; 24:13.
  20. Fucci, MJ, Buchman, CA, Brackmann, DE, Berliner, KI. Acoustic tumor growth: implications for treatment choices. Am J Otol 1999; 20:495.
  21. Modugno, GC, Pirodda, A, Ferri, GG, et al. Small acoustic neuromas: Monitoring the growth rate by MRI. Acta Neurochir (Wien) 1999; 141:1063.
  22. Ohta, S, Yokoyama, T, Nishizawa, S. Massive haemorrhage into acoustic neurinoma related to rapid growth of the tumour. Br J Neurosurg 1998; 12:455.
  23. 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.
  24. Barker FG, 2nd, Carter, BS, Ojemann, RG, et al. Surgical excision of acoustic neuroma: patient outcome and provider caseload. Laryngoscope 2003; 113:1332.
  25. Gormley, WB, Sekhar, LN, Wright, DC, et al. Acoustic neuromas: results of current surgical management [see comments]. Neurosurgery 1997; 41:50.
  26. 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.
  27. 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.
  28. Darrouzet, V, Martel, J, Enee, V, et al. Vestibular schwannoma surgery outcomes: our multidisciplinary experience in 400 cases over 17 years. Laryngoscope 2004; 114:681.
  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. Ohta, S, Yokoyama, T, Nishizawa, S, Uemura, K. Regrowth of the residual tumour after acoustic neurinoma surgery. Br J Neurosurg 1998; 12:419.
  34. 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.
  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 [see comments]. 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, Regis, J, Deveze, 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. Williams, JA. Fractionated stereotactic radiotherapy for acoustic neuromas. Int J Radiat Oncol Biol Phys 2002; 54:500.
  45. 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.
  46. Koh, ES, Millar, BA, Menard, C, et al. Fractionated stereotactic radiotherapy for acoustic neuroma: single-institution experience at the princess margaret hospital. Cancer 2007; 109:1203.
  47. Combs, SE, Volk, S, Schulz-Ertner, D, et al. Management of acoustic neuromas with fractionated stereotactic radiotherapy (FSRT): Long-term results in 106 patients treated in a single institution. Int J Radiat Oncol Biol Phys 2005; 63:75.
  48. Chan, AW, Black, P, Ojemann, RG, et al. Stereotactic radiotherapy for vestibular schwannomas: favorable outcome with minimal toxicity. Neurosurgery 2005; 57:60.
  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.
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