Highlights & Basics
- Vestibular schwannoma is a histologically benign, generally slow-growing tumor that may remain stable for long periods on follow-up magnetic resonance imaging scans after initial detection.
- Progressive unilateral sensorineural hearing loss is the most common presenting symptom, followed by tinnitus and intermittent dizziness; hemifacial numbness or pain may occur less frequently.
- Larger tumors may cause headaches, ataxia, and obstructive hydrocephalus and may rarely be life threatening.
- Diagnosis is confirmed by gadolinium-enhanced magnetic resonance imaging of the head.
- Management includes observation (active surveillance), focused radiation (stereotactic radiosurgery or radiation therapy), or surgery.
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History & Exam
Key Factors
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Definition
Epidemiology
Etiology
Pathophysiology
Citations
Goldbrunner R, Weller M, Regis J, et al. EANO guideline on the diagnosis and treatment of vestibular schwannoma. Neuro Oncol. 2020 Jan 11;22(1):31-45.[Abstract][Full Text]
Ren Y, Chari DA, Vasilijic S, et al. New developments in neurofibromatosis type 2 and vestibular schwannoma. Neurooncol Adv. 2021 Jan-Dec;3(1):vdaa153.[Abstract][Full Text]
Lin VY, Stewart C, Grebenyuk J, et al. Unilateral acoustic neuromas: long-term hearing results in patients managed with fractionated stereotactic radiotherapy, hearing preservation surgery, and expectantly. Laryngoscope. 2005 Feb;115(2):292-6.[Abstract]
Pollock BE. Management of vestibular schwannomas that enlarge after stereotactic radiosurgery: treatment recommendations based on a 15 year experience. Neurosurgery. 2006 Feb;58(2):241-8.[Abstract]
Darrouzet V, Martel J, Enee V, et al. Vestibular schwannoma surgery outcomes: our multidisciplinary experience in 400 cases over 17 years. Laryngoscope. 2004 Apr;114(4):681-8.[Abstract]
Roland JT Jr, Fishman AJ, Golfinos JG, et al. Cranial nerve preservation in surgery for large acoustic neuromas. Skull Base. 2004 May;14(2):85-91.[Abstract][Full Text]
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22. Evans DG, Wallace AJ, Hartley C, et al. Familial unilateral vestibular schwannoma is rarely caused by inherited variants in the NF2 gene. Laryngoscope. 2019 Apr;129(4):967-73.[Abstract][Full Text]
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51. Ren Y, Sethi RKV, Stankovic KM. National trends in surgical resection of vestibular schwannomas. Otolaryngol Head Neck Surg. 2020 Dec;163(6):1244-9.[Abstract]
52. Hunt AA, Cass ND, Coughlin A, et al. Time-based assessment of hearing preservation rates after microsurgical resection of vestibular schwannomas: a systematic review. Otol Neurotol. 2020 Jun;41(5):679-85.[Abstract]
53. Wallerius KP, Macielak RJ, Lawlor SK, et al. Hearing preservation microsurgery in vestibular schwannomas: worth attempting in "larger" tumors? Laryngoscope. 2022 Aug;132(8):1657-64.[Abstract]
54. Carlson ML, Vivas EX, McCracken DJ, et al. Congress of Neurological Surgeons systematic review and evidence-based guidelines on hearing preservation outcomes in patients with sporadic vestibular schwannomas. Neurosurgery. 2018 Feb 1;82(2):E35-9.[Abstract][Full Text]
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56. Nikolopoulos TP, Fortnum H, O'Donoghue G, et al. Acoustic neuroma growth: a systematic review of the evidence. Otol Neurotol. 2010 Apr;31(3):478-85.[Abstract]
57. Macielak RJ, Wallerius KP, Lawlor SK, et al. Defining clinically significant tumor size in vestibular schwannoma to inform timing of microsurgery during wait-and-scan management: moving beyond minimum detectable growth. J Neurosurg. 2022 May;136(5):1289-97.[Abstract][Full Text]
58. Carlson ML, Link MJ, Driscoll CLW, et al. Working toward consensus on sporadic vestibular schwannoma care: a modified Delphi study. Otol Neurotol. 2020 Dec;41(10):e1360-71.[Abstract]
59. Marinelli JP, Lohse CM, Carlson ML. Introducing an evidence-based approach to wait-and-scan management of sporadic vestibular schwannoma: size threshold surveillance. Otolaryngol Clin North Am. 2023 Jun;56(3):445-57.[Abstract]
60. Lin VY, Stewart C, Grebenyuk J, et al. Unilateral acoustic neuromas: long-term hearing results in patients managed with fractionated stereotactic radiotherapy, hearing preservation surgery, and expectantly. Laryngoscope. 2005 Feb;115(2):292-6.[Abstract]
61. Pollock BE. Management of vestibular schwannomas that enlarge after stereotactic radiosurgery: treatment recommendations based on a 15 year experience. Neurosurgery. 2006 Feb;58(2):241-8.[Abstract]
62. Prasad D, Steiner M, Steiner L. Gamma surgery for vestibular schwannoma. J Neurosug. 2000 May;92(5):745-9.[Abstract]
63. Pollock BE, Lunsford LD, Noren G. Vestibular schwannoma management in the next century: a radiosurgical perspective. Neurosurgery. 1998 Sep;43(3):475-83.[Abstract]
64. Chamoun R, MacDonald J, Shelton C, et al. Surgical approaches for resection of vestibular schwannomas: translabyrinthine, retrosigmoid, and middle fossa approaches. Neurosurg Focus. 2012 Sep;33(3):E9.[Abstract][Full Text]
65. Barker FG II, Carter BS, Ojemann RG, et al. Surgical excision of acoustic neuroma: patient outcome and provider caseload. Laryngoscope. 2003 Aug;113(8):1332-43.[Abstract]
66. Darrouzet V, Martel J, Enee V, et al. Vestibular schwannoma surgery outcomes: our multidisciplinary experience in 400 cases over 17 years. Laryngoscope. 2004 Apr;114(4):681-8.[Abstract]
67. Ciric I, Zhao JC, Rosenblatt S, et al. Suboccipital retrosigmoid approach for removal of vestibular schwannomas: facial nerve function and hearing preservation. Neurosurgery. 2005 Mar;56(3):560-70.[Abstract]
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83. Samii M, Metwali H, Gerganov V. Microsurgical management of vestibular schwannoma after failed previous surgery. J Neurosurg. 2016 Nov;125(5):1198-203.[Abstract][Full Text]
84. Akagami R, Di Maio S. Prospective comparison of quality of life before and after observation, radiation, or surgery for vestibular schwannomas. J Neurosurg. 2009 Oct;111(4):855-62.[Abstract]
85. Yong RL, Westerberg BD, Dong C, et al. Length of tumor-cochlear nerve contact and hearing outcome after surgery for vestibular schwannoma. J Neurosurg. 2008 Jan;108(1):105-10.[Abstract]
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87. Passier L, Doherty D, Smith J, et al. Vestibular rehabilitation following the removal of an acoustic neuroma: a systematic review of randomized trials. Head Neck Oncol. 2012 Sep 9;4(2):59.[Abstract]
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