Main Symptom that Led to Medical Evaluation and Diagnosis of Vestibular Schwannoma and Patient-Reported Tumor Size: Cross-sectional Study in 1,304 Patients

Abstract

Objectives Although vestibular schwannomas (VS) are known to cause cranial nerve deficits, cerebellar symptoms, and hydrocephalus, the role of these symptoms as the key driver of presentation from the patient's perspective has not been described. Our objective was to survey a large, retrospective VS cohort to document the patient-reported principal initial symptom, and self-reported tumor size, and to study trends in VS patient presentation.

Methods Patients diagnosed with VS at our tertiary referral center and belonging to the Acoustic Neuroma Association (ANA) answered a questionnaire between 2015 and 2017. Demographic data, self-reported tumor size, and symptomatology were analyzed.

Results 1,304 patients completed the questionnaire. Tumors were diagnosed from 1966 to 2017 at a mean 51.8 years (range: 8–86 years); 66% were female, and 1.1% had confirmed neurofibromatosis type 2 (NF2). Tumor size was reported using a 6-point scale: 0 to 1 cm (22.9%), 1 to 2 cm (28.7%), 2 to 3 cm (20.5%), 3 to 4 cm (10%), greater than 4 cm (7.2%), and unknown (10.6%). Hearing loss was the most common symptom that led to diagnosis (51.5%), followed by dizziness (17%), tinnitus (11.2%), and incidental diagnosis (10.2%); a fraction that has increased significantly in the last decade (p = 0.022). Larger tumors and NF2 were significantly associated with young age (p < 0.001).

Conclusion Our large-scale questionnaire-driven review of 1,304 patients confirms that VS presentations are stereotypical, with most individuals recalling hearing loss, dizziness, or tinnitus as their chief complaint. Many tumors were incidentally diagnosed; an expanding population, attributable to increased access to magnetic resonance imaging (MRI). Large tumors were significantly more prevalent among younger patients at diagnosis, excluding NF2 patients, suggesting a more aggressive tumor biology that remains incompletely understood.

Keywords

vestibular schwannoma - hearing loss - dizziness - symptoms at presentation - acoustic neuroma

• References

• 1 Foley RW, Shirazi S, Maweni RM. , et al. Signs and symptoms of acoustic neuroma at initial presentation: an exploratory analysis. Cureus 2017; 9 (11) e1846
• 2 Matthies C, Samii M. Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation. Neurosurgery 1997; 40 (01) 1-9 , discussion 9–10
• 3 Broomfield SJ, O'Donoghue GM. Self-reported symptoms and patient experience: A British Acoustic Neuroma Association survey. Br J Neurosurg 2016; 30 (03) 294-301
• 4 Kleijwegt M, Ho V, Visser O, Godefroy W, van der Mey A. Real incidence of vestibular schwannoma? estimations from a national registry. Otol Neurotol 2016; 37 (09) 1411-1417
• 5 Wong BY, Capper R. Incidence of vestibular schwannoma and incidental findings on the magnetic resonance imaging and computed tomography scans of patients from a direct referral audiology clinic. J Laryngol Otol 2012; 126 (07) 658-662
• 6 Evans DG, Moran A, King A, Saeed S, Gurusinghe N, Ramsden R. 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 2005; 26 (01) 93-97
• 7 Tos M, Stangerup SE, Cayé-Thomasen P, Tos T, Thomsen J. What is the real incidence of vestibular schwannoma?. Arch Otolaryngol Head Neck Surg 2004; 130 (02) 216-220
• 8 Acoustic Neuroma Association (ANA). Available from: https://www.anausa.org/acoustic-neuroma-association/about-ana . Accessed on September 10, 2018
• 9 Stangerup SE, Tos M, Thomsen J, Caye-Thomasen P. True incidence of vestibular schwannoma?. Neurosurgery 2010; 67 (05) 1335-1340 , discussion 1340
• 10 Stangerup SE, Tos M, Caye-Thomasen P, Tos T, Klokker M, Thomsen J. Increasing annual incidence of vestibular schwannoma and age at diagnosis. J Laryngol Otol 2004; 118 (08) 622-627
• 11 Zhang Z, Nguyen Y, De Seta D. , et al. Surgical treatment of sporadic vestibular schwannoma in a series of 1,006 patients. Acta Otorhinolaryngol Ital 2016; 36 (05) 408-414
• 12 Samii M, Matthies C. Management of 1,000 vestibular schwannomas (acoustic neuromas): the facial nerve--preservation and restitution of function. Neurosurgery 1997; 40 (04) 684-694 , discussion 694–695
• 13 Smouha EE, Yoo M, Mohr K, Davis RP. Conservative management of acoustic neuroma: a meta-analysis and proposed treatment algorithm. Laryngoscope 2005; 115 (03) 450-454
• 14 Hunter JB, Francis DO, O'Connell BP. , et al. Single institutional experience with observing 564 vestibular schwannomas: factors associated with tumor growth. Otol Neurotol 2016; 37 (10) 1630-1636
• 15 Anand G, Vasallo G, Spanou M. , et al. Diagnosis of sporadic neurofibromatosis type 2 in the paediatric population. Arch Dis Child 2018; 103 (05) 463-469
• 16 Samii M, Matthies C, Tatagiba M. Management of vestibular schwannomas (acoustic neuromas): auditory and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibromatosis 2. Neurosurgery 1997; 40 (04) 696-705 , discussion 705–706
• 17 Håvik AL, Bruland O, Myrseth E. , et al. Genetic landscape of sporadic vestibular schwannoma. J Neurosurg 2018; 128 (03) 911-922
• 18 Carlson ML, Tveiten OV, Lund-Johansen M, Tombers NM, Lohse CM, Link MJ. Patient motivation and long-term satisfaction with treatment choice in vestibular schwannoma. World Neurosurg 2018; 114: e1245-e1252
• 19 Carlson ML, Tveiten OV, Driscoll CL. , et al. What drives quality of life in patients with sporadic vestibular schwannoma?. Laryngoscope 2015; 125 (07) 1697-1702
• 20 Myrseth E, Møller P, Wentzel-Larsen T, Goplen F, Lund-Johansen M. Untreated vestibular schwannomas: vertigo is a powerful predictor for health-related quality of life. Neurosurgery 2006; 59 (01) 67-76 , discussion 67–76
• 21 Samii M, Gerganov V, Samii A. Improved preservation of hearing and facial nerve function in vestibular schwannoma surgery via the retrosigmoid approach in a series of 200 patients. J Neurosurg 2006; 105 (04) 527-535
• 22 Carlson ML, Marston AP, Glasgow AE. , et al. Racial differences in vestibular schwannoma. Laryngoscope 2016; 126 (09) 2128-2133
• 23 Anderson SH. The acoustic neuroma association. Otolaryngol Clin North Am 1997; 30 (05) 905-912
• 24 Van Gompel JJ, Patel J, Danner C. , et al. Acoustic neuroma observation associated with an increase in symptomatic tinnitus: results of the 2007–2008 Acoustic Neuroma Association survey. J Neurosurg 2013; 119 (04) 864-868
• 25 Moshtaghi O, Goshtasbi K, Sahyouni R, Lin HW, Djalilian HR. Patient decision making in vestibular schwannoma: a survey of the acoustic neuroma association. Otolaryngol Head Neck Surg 2018; 158 (05) 912-916
• 26 Patel J, Vasan R, van Loveren H, Downes K, Agazzi S. The changing face of acoustic neuroma management in the USA: analysis of the 1998 and 2008 patient surveys from the acoustic neuroma association. Br J Neurosurg 2014; 28 (01) 20-24
• 27 Ryzenman JM, Pensak ML, Tew Jr JM. Patient perception of comorbid conditions after acoustic neuroma management: survey results from the acoustic neuroma association. Laryngoscope 2004; 114 (05) 814-820