Vestibular Schwannoma (Acoustic Neuroma)

Entitlement Eligibility Guideline (EEG)

Date reviewed: 22 January 2025

Date created: February 2005

ICD-11 code: 2A02.3

VAC medical code: 38850 Vestibular schwannoma (Acoustic neuroma)

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Definition

Vestibular schwannoma is a benign tumor formed from Schwann cells of the vestibular division (or rarely on the cochlear division), of the vestibulocochlear (Cranial nerve VIII)/acoustic nerve.

For the purpose of this entitlement eligibility guideline (EEG), equivalent diagnoses for vestibular schwannoma include:

  • acoustic neuroma
  • acoustic schwannoma
  • acoustic neurinoma
  • acoustic neurilemmoma
  • vestibular neurilemmoma.

For Veterans Affairs Canada (VAC) purposes, vertigo, hearing loss (HL), and/or tinnitus may present as part of the symptom complex of a diagnosed medical condition, or they may present as a primary stand-alone diagnosed medical condition. In those presenting with symptoms of vertigo, HL, and/or tinnitus, but with a known diagnosed cause (e.g. Meniere’s disease), these symptoms are included in entitlement and assessment of the medical condition. Prior to adjudicating the entitlement and assessment of vertigo, HL, and/or tinnitus, or a diagnosed medical condition that may cause these symptoms, a close review of previously entitled medical conditions with potentially overlapping symptoms is required.

Diagnostic standard

A diagnosis from a qualified medical practitioner (ear, nose and throat specialist [ENT]/otolaryngologist, neuro-surgeon, neurologist, family physician) is required.

The diagnosis is based on magnetic resonance imaging (MRI) findings. Additional investigations may include the following:

  • audiogram
  • computed tomography (CT) scan.

Anatomy and physiology

Vestibular schwannoma is a benign slow growing tumor arising from the myelin- forming Schwann cells which form a sheath or coating over the vestibulocochlear nerve fibers.

A vestibular schwannoma can grow and expand into the cerebellopontine angle.

Clinical features

Early symptomatology may be subtle and easily escape detection. The growth rate is generally slow. The history often reveals symptoms going back many years. Signs and symptoms develop primarily because of pressure effects that result from increasing tumour size.

Clinical features may include:

  1. Unilateral hearing loss is a primary indicator of vestibular schwannoma. Any pattern of hearing loss may develop, including sudden hearing loss. Routine audiometry may show a hearing loss of which the person is unaware.
  2. Tinnitus in the affected ear is a common complaint and may occur prior to onset of hearing loss.
  3. Vertigo is an uncommon complaint. Minor disturbances of balance are common, with episodes of rotary vertigo being less common. These episodes may last several seconds or minutes to hours, with associated marked visceral autonomic symptoms.
  4. Other than the neurologic deficit associated with the vestibulocochlear nerve (Cranial Nerve VIII), the most common neurologic deficits are changes in sensation within the distribution of either or both of the following:
    • trigeminal nerve (Cranial Nerve V): facial numbness (paresthesia), pain
    • facial nerve (Cranial Nerve VII): facial paresis and, less often, taste disturbances, dry eyes.

When comparing males and females, there are no differences in rates of occurrence, presentation or symptoms of vestibular schwannoma.

Entitlement considerations

In this section

Section A: Causes and/or aggravation

Section B: Medical conditions which are to be included in entitlement/assessment

Section C: Common medical conditions which may result, in whole or in part, from vestibular schwannoma and/or its treatment

Section A: Causes and/or aggravation

For VAC entitlement purposes, the following factors are accepted to cause or aggravate the conditions included in the Definition section of this EEG, and may be considered along with the evidence to assist in establishing a relationship to service. The factors have been determined based on a review of up- to-date scientific and medical literature, as well as evidence-based medical best practices. Factors other than those listed may be considered, however consultation with a disability consultant or medical advisor is recommended.

The timelines cited below are for guidance purposes. Each case should be adjudicated on the evidence provided and its own merits.

Factors

  1. Therapeutic radiation to the head at least 10 years before clinical onset or aggravation of vestibular schwannoma. The latency time for the development of vestibular schwannoma following therapeutic radiation to the head has been recognized to be at least 10 years, and up to 30 years.
  2. Neurofibromatosis prior to clinical onset or aggravation of vestibular schwannoma. Neurofibromatosis (Von Recklinghausen’s Disease) is an autosomal dominant genetic disorder with distinctive features. The features may be present at birth, as in café au lait lesions, or may develop decades later. One of these delayed features is vestibular schwannoma. Bilateral vestibular schwannoma is considered characteristic of neurofibromatosis.
  3. Inability to obtain appropriate clinical management of vestibular schwannoma.

Note: At the time of publication of this EEG, the medical literature indicates the following:

  • Head trauma does not cause or permanently aggravate vestibular schwannoma.
  • Occupational noise exposure does not cause or permanently aggravate vestibular schwannoma.
  • Mobile/cellular phone use does not cause or permanently aggravate vestibular schwannoma.

Section B: Medical conditions which are to be included in entitlement/assessment

Section B provides a list of diagnosed medical conditions which are considered for VAC purposes to be included in the entitlement and assessment of vestibular schwannoma.

Section C: Common medical conditions which may result, in whole or in part, from vestibular schwannoma and/or its treatment

Section C is a list of conditions which can be caused or aggravated by vestibular schwannoma and/or its treatment. Conditions listed in Section C are not included in the entitlement and assessment of vestibular schwannoma. A consequential entitlement decision may be considered where the individual merits and the medical evidence of the case support a consequential relationship. Conditions other than those listed in Section C may be considered; consultation with a disability consultant or medical advisor is recommended.

  • Trigeminal nerve injury
  • Facial nerve injury

Related VAC guidance and policy:

References as of 22 January 2025

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Australian Government, Repatriation Medical Authority. (2019). Statement of principles concerning acoustic neuroma, reasonable hypothesis, No 96 of 2019. SOPs - Repatriation Medical Authority

Australian Government, Repatriation Medical Authority. (2019). Statement of principles concerning acoustic neuroma, balance of probabilities, No 97 of 2019. SOPs - Repatriation Medical Authority

Biswas, R., Genitsaridi, E., Trpchevska, N., Lugo, A., Schlee, W., Cederroth, C. R., Gallus, S., & Hall, D. A. (2022). Low evidence for tinnitus risk factors: A systematic review and Meta-analysis. Journal of the Association for Research in Otolaryngology, 24(1), 81–94. https://doi.org/10.1007/s10162-022-00874-y

Blettner, M., Schlehofer, B., Samkange-Zeeb, F., Berg, G., Schlaefer, K., & Schüz, J. (2007). Medical exposure to ionising radiation and the risk of brain tumours: Interphone study group, Germany. European Journal of Cancer, 43(13), 1990– 1998. https://doi.org/10.1016/j.ejca.2007.06.020

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Chen, M., Fan, Z., Zheng, X., Cao, F., & Wang, L. (2016). Risk Factors of Acoustic Neuroma: Systematic Review and Meta-Analysis. Yonsei Medical Journal, 57(3), 776. https://doi.org/10.3349/ymj.2016.57.3.776

Clinical Key. (2022). Vestibular Schwannoma Clinical Overview. Elsevier.

Durham, A. R., Tooker, E. L., Patel, N. S., & Gurgel, R. K. (2023). Epidemiology and Risk Factors for Development of Sporadic Vestibular Schwannoma. Otolaryngologic Clinics of North America, 56(3), 413–420. https://doi.org/10.1016/j.otc.2023.02.003

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McCormack, A., Edmondson-Jones, M., Somerset, S., & Hall, D. (2016). A systematic review of the reporting of tinnitus prevalence and severity. Hearing Research, 337, 70–79. https://doi.org/10.1016/j.heares.2016.05.009

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Schlehofer, B., Schlaefer, K., Blettner, M., Berg, G., Böhler, E., Hettinger, I., Kunna-Grass, K., Wahrendorf, J., & Schüz, J. (2007). Environmental risk factors for sporadic acoustic neuroma (Interphone Study Group, Germany). European Journal of Cancer, 43(11), 1741–1747. https://doi.org/10.1016/j.ejca.2007.05.008

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