Vestibular schwannoma (acoustic neuroma)
- John K Park, MD, PhD
John K Park, MD, PhD
- Santa Barbara Neuroscience Institute
- David M Vernick, MD
David M Vernick, MD
- Assistant Professor of Otolaryngology
- Harvard Medical School
- Naren Ramakrishna, MD, PhD
Naren Ramakrishna, MD, PhD
- Director of Neuro and Pediatric Radiation Oncology
- University of Florida Health Cancer Center at Orlando Health
- Professor of Radiation Oncology
- University of Central Florida College of Medicine
- Section Editors
- Jay S Loeffler, MD
Jay S Loeffler, MD
- Section Editor — Neurooncology
- Professor of Radiation Oncology
- Harvard Medical School
- Patrick Y Wen, MD
Patrick Y Wen, MD
- Section Editor — Neurooncology
- Professor of Neurology
- Harvard Medical School
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.
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) , and autopsy studies suggest that the prevalence may be even higher [3,4].
The median age at diagnosis is approximately 50 years . The tumors are unilateral in more than 90 percent of cases , affecting the right and left sides with equal frequency. Bilateral vestibular schwannomas are primarily limited to patients with neurofibromatosis type 2 . (See "Neurofibromatosis type 2", section on 'Vestibular schwannomas'.)
- Lin D, Hegarty JL, Fischbein NJ, Jackler RK. The prevalence of "incidental" acoustic neuroma. Arch Otolaryngol Head Neck Surg 2005; 131:241.
- Propp JM, McCarthy BJ, Davis FG, Preston-Martin S. Descriptive epidemiology of vestibular schwannomas. Neuro Oncol 2006; 8:1.
- 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.
- Eldridge R, Parry D. Vestibular schwannoma (acoustic neuroma). Consensus development conference. Neurosurgery 1992; 30:962.
- Edwards CG, Schwartzbaum JA, Lönn S, et al. Exposure to loud noise and risk of acoustic neuroma. Am J Epidemiol 2006; 163:327.
- 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.
- 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.
- 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.
- Bäcklund LM, Grandér D, Brandt L, et al. Parathyroid adenoma and primary CNS tumors. Int J Cancer 2005; 113:866.
- 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.
- Schlehofer B, Schlaefer K, Blettner M, et al. Environmental risk factors for sporadic acoustic neuroma (Interphone Study Group, Germany). Eur J Cancer 2007; 43:1741.
- Hours M, Bernard M, Arslan M, et al. Can loud noise cause acoustic neuroma? Analysis of the INTERPHONE study in France. Occup Environ Med 2009; 66:480.
- Edwards CG, Schwartzbaum JA, Nise G, et al. Occupational noise exposure and risk of acoustic neuroma. Am J Epidemiol 2007; 166:1252.
- Ryals BM, Rubel EW. Hair cell regeneration after acoustic trauma in adult Coturnix quail. Science 1988; 240:1774.
- Hamernik RP, Turrentine G, Roberto M, et al. Anatomical correlates of impulse noise-induced mechanical damage in the cochlea. Hear Res 1984; 13:229.
- Fisher JL, Pettersson D, Palmisano S, et al. Loud noise exposure and acoustic neuroma. Am J Epidemiol 2014; 180:58.
- 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.
- Matthies C, Samii M. Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation. Neurosurgery 1997; 40:1.
- Noonan KY, Rang C, Callahan K, et al. Nervus Intermedius Symptoms following Surgical or Radiation Therapy for Vestibular Schwannoma. Otolaryngol Head Neck Surg 2016; 155:657.
- 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.
- Wilson DF, Hodgson RS, Gustafson MF, et al. The sensitivity of auditory brainstem response testing in small acoustic neuromas. Laryngoscope 1992; 102:961.
- 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.
- 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.
- 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.
- 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.
- Barker FG 2nd, Carter BS, Ojemann RG, et al. Surgical excision of acoustic neuroma: patient outcome and provider caseload. Laryngoscope 2003; 113:1332.
- Gormley WB, Sekhar LN, Wright DC, et al. Acoustic neuromas: results of current surgical management. Neurosurgery 1997; 41:50.
- 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.
- 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.
- 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.
- Ohta S, Yokoyama T, Nishizawa S, Uemura K. Regrowth of the residual tumour after acoustic neurinoma surgery. Br J Neurosurg 1998; 12:419.
- 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.
- Samii M, Matthies C. Management of 1000 vestibular schwannomas (acoustic neuromas): the facial nerve--preservation and restitution of function. Neurosurgery 1997; 40:684.
- 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.
- 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.
- Schankin CJ, Gall C, Straube A. Headache syndromes after acoustic neuroma surgery and their implications for quality of life. Cephalalgia 2009; 29:760.
- Sughrue ME, Yang I, Aranda D, et al. Beyond audiofacial morbidity after vestibular schwannoma surgery. J Neurosurg 2011; 114:367.
- Muzevic D, Legcevic J, Splavski B, Cayé-Thomasen P. Stereotactic radiotherapy for vestibular schwannoma. Cochrane Database Syst Rev 2014; :CD009897.
- Murphy ES, Suh JH. Radiotherapy for vestibular schwannomas: a critical review. Int J Radiat Oncol Biol Phys 2011; 79:985.
- Kondziolka D, Lunsford LD, McLaughlin MR, Flickinger JC. Long-term outcomes after radiosurgery for acoustic neuromas. N Engl J Med 1998; 339:1426.
- Flickinger JC, Lunsford LD, Linskey ME, et al. Gamma knife radiosurgery for acoustic tumors: multivariate analysis of four year results. Radiother Oncol 1993; 27:91.
- Foote RL, Coffey RJ, Swanson JW, et al. Stereotactic radiosurgery using the gamma knife for acoustic neuromas. Int J Radiat Oncol Biol Phys 1995; 32:1153.
- Mendenhall WM, Friedman WA, Buatti JM, Bova FJ. Preliminary results of linear accelerator radiosurgery for acoustic schwannomas. J Neurosurg 1996; 85:1013.
- Yang I, Sughrue ME, Han SJ, et al. A comprehensive analysis of hearing preservation after radiosurgery for vestibular schwannoma. J Neurosurg 2010; 112:851.
- Roos DE, Potter AE, Brophy BP. Stereotactic radiosurgery for acoustic neuromas: what happens long term? Int J Radiat Oncol Biol Phys 2012; 82:1352.
- Hasegawa T, Kida Y, Kato T, et al. Long-term safety and efficacy of stereotactic radiosurgery for vestibular schwannomas: evaluation of 440 patients more than 10 years after treatment with Gamma Knife surgery. J Neurosurg 2013; 118:557.
- Carlson ML, Jacob JT, Pollock BE, et al. Long-term hearing outcomes following stereotactic radiosurgery for vestibular schwannoma: patterns of hearing loss and variables influencing audiometric decline. J Neurosurg 2013; 118:579.
- Roos DE, Potter AE, Zacest AC. Hearing preservation after low dose linac radiosurgery for acoustic neuroma depends on initial hearing and time. Radiother Oncol 2011; 101:420.
- Mousavi SH, Niranjan A, Akpinar B, et al. Hearing subclassification may predict long-term auditory outcomes after radiosurgery for vestibular schwannoma patients with good hearing. J Neurosurg 2016; 125:845.
- Yomo S, Carron R, Thomassin JM, et al. Longitudinal analysis of hearing before and after radiosurgery for vestibular schwannoma. J Neurosurg 2012; 117:877.
- Kano H, Kondziolka D, Khan A, et al. Predictors of hearing preservation after stereotactic radiosurgery for acoustic neuroma: clinical article. J Neurosurg 2013; 119 Suppl:863.
- Jacob JT, Carlson ML, Schiefer TK, et al. Significance of cochlear dose in the radiosurgical treatment of vestibular schwannoma: controversies and unanswered questions. Neurosurgery 2014; 74:466.
- Pollock BE. Management of vestibular schwannomas that enlarge after stereotactic radiosurgery: treatment recommendations based on a 15 year experience. Neurosurgery 2006; 58:241.
- Niu NN, Niemierko A, Larvie M, et al. Pretreatment growth rate predicts radiation response in vestibular schwannomas. Int J Radiat Oncol Biol Phys 2014; 89:113.
- 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.
- Shin M, Ueki K, Kurita H, Kirino T. Malignant transformation of a vestibular schwannoma after gamma knife radiosurgery. Lancet 2002; 360:309.
- Tanbouzi Husseini S, Piccirillo E, Taibah A, et al. Malignancy in vestibular schwannoma after stereotactic radiotherapy: a case report and review of the literature. Laryngoscope 2011; 121:923.
- Roche PH, Régis J, Devèze A, et al. [Surgical removal of unilateral vestibular schwannomas after failed Gamma Knife radiosurgery]. Neurochirurgie 2004; 50:383.
- 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.
- Williams JA. Fractionated stereotactic radiotherapy for acoustic neuromas. Int J Radiat Oncol Biol Phys 2002; 54:500.
- 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.
- 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.
- 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.
- Combs SE, Engelhard C, Kopp C, et al. Long-term outcome after highly advanced single-dose or fractionated radiotherapy in patients with vestibular schwannomas - pooled results from 3 large German centers. Radiother Oncol 2015; 114:378.
- Weber DC, Chan AW, Bussiere MR, et al. Proton beam radiosurgery for vestibular schwannoma: tumor control and cranial nerve toxicity. Neurosurgery 2003; 53:577.
- 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.
- Smouha EE, Yoo M, Mohr K, Davis RP. Conservative management of acoustic neuroma: a meta-analysis and proposed treatment algorithm. Laryngoscope 2005; 115:450.
- Pulec JL. Acoustic neuroma surgery in geriatric patients. Ear Nose Throat J 1999; 78:429.
- Samii M, Tatagiba M, Matthies C. Acoustic neurinoma in the elderly: factors predictive of postoperative outcome. Neurosurgery 1992; 31:615.
- Akpinar B, Mousavi SH, McDowell MM, et al. Early Radiosurgery Improves Hearing Preservation in Vestibular Schwannoma Patients With Normal Hearing at the Time of Diagnosis. Int J Radiat Oncol Biol Phys 2016; 95:729.
- 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.
- Sughrue ME, Kane AJ, Kaur R, et al. A prospective study of hearing preservation in untreated vestibular schwannomas. J Neurosurg 2011; 114:381.
- PATHOGENESIS AND RISK FACTORS
- CLINICAL PRESENTATION
- Physical examination
- Vestibular testing
- - Surgical techniques
- - Outcomes
- - Other complications
- Radiation therapy
- - Stereotactic radiosurgery
- - Stereotactic radiotherapy
- - Proton beam therapy
- POSTTREATMENT FOLLOW-UP
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS