Other hearing diseases

Acoustic neuroma (vestibular schwannoma)

Acoustic neuromas are small cranial tumors that affect roughly one to two people per 100,000 annually. While benign, these tumors can cause symptoms that affect quality of life and often require surgery or radiation to reduce or stop growth. Learn what an acoustic neuroma is, plus causes, symptoms and treatment options.

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Dr. Thomas Tedeschi, Au.D.

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What is an acoustic neuroma?

Symptoms

Causes

Treatment options

Diagnosis

Prevention

Key takeaways

An acoustic neuroma is a benign tumor that grows in the inner ear.
Acoustic neuromas may occur on one side (unilateral) or both sides (bilateral).
Acoustic neuromas affect roughly 1 to 2 people per 100,000 each year.
Bilateral acoustic neuromas are often caused by an inherited disorder called acoustic neuroma neurofibromatosis type 2 (NF2).
Symptoms of an acoustic neuroma include hearing loss, tinnitus, dizziness, facial numbness and balance issues. These symptoms may come and go.
The cause of most acoustic neuromas is unknown, but some potential factors include genetic predisposition, age and exposure to loud noises or radiation.
An acoustic neuroma can be diagnosed with a series of tests, including hearing and balance tests and MRIs.
Acoustic neuroma treatment options include watching and waiting, surgery or radiation. While uncommon, it is possible for a neuroma to return after surgery.

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What is an acoustic neuroma?

An acoustic neuroma, also known as a vestibular schwannoma, is a tumor that grows in the inner ear. While they are generally benign, acoustic neuroma tumors can become dangerous if left untreated, as they could affect hearing and balance nerves.

Acoustic neuromas can occur on one or both sides. When a tumor is only present on one side, it is considered unilateral. On the other hand, bilateral acoustic neuromas occur on both sides. This type of neuroma is often associated with a condition called acoustic neuroma neurofibromatosis type 2 (NF2), a genetic disorder that may be passed down from family or acquired through mutation¹.

Location of an acoustic neuroma tumor

Acoustic neuromas form in the inner ear—specifically on the nerve leading from the inner ear into the brain. If left to grow, it can also lead to subsequent damage to the nerves that control balance and face sensation.

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Symptoms of an acoustic neuroma

Experiences vary by person and can change from day to day, but common acoustic neuroma symptoms include:

Hearing loss
Tinnitus (ringing in the ear)
Loss of balance or dizziness
Numbness around the face
Muscle weakness or loss of movement

The acoustic neuroma hearing loss pattern is another common indicator, as patients tend to lose the ability to hear high frequencies first². This pattern of gradual hearing loss occurs over months and years, and may eventually affect the ability to understand speech and communicate with others.

Do acoustic neuroma symptoms come and go?

Every case is different, but it is not uncommon for symptoms to come and go. As the tumor grows and changes shape, it compresses inner ear nerves in different ways, leading to changes in the intensity and frequency of symptoms.

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Causes of an acoustic neuroma

Acoustic neuromas occur when the body overproduces Schwann cells, which support and insulate the nerves in your inner ear. Some scientists believe this is caused by the loss of function of a specific gene that controls the growth of Schwann cells.¹ This may be the case in patients with NF2. However, in many cases, the cause of overgrowth is unknown.

Common acoustic neuroma causes include:

Genetic predisposition or mutations in the Schwann cells of your inner ear
Age
Exposure to radiation
Exposure to loud noises

Treatment options for an acoustic neuroma

Acoustic neuroma treatment plans vary from person to person based on the size and location of the tumor and the severity of symptoms. Here is an overview of common acoustic neuroma treatment options.

Also known as “watch and wait,” the watchful waiting strategy is exactly what it sounds like. It includes monitoring your condition over time before starting treatment, and it’s most commonly recommended if your tumor is small and not causing major symptoms. Your doctor may monitor your condition with regular acoustic neuroma MRIs.

In cases where symptoms are more pronounced or the tumor is growing, your doctor may recommend acoustic neuroma surgery. This involves entering your inner ear to remove the tumor and requires several weeks of recovery with regular follow-up appointments.

Stereotactic radiosurgery for acoustic neuroma is an emerging alternative treatment to traditional surgery. This type of radiation therapy for acoustic neuromas is sometimes recommended to limit or reduce tumor growth for patients who are aging, in poor health or are affected in both ears. It has a high success rate for treating early-stage neuromas³.

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Before and after an acoustic neuroma surgery

Before acoustic neuroma surgery, symptoms may include hearing loss, dizziness or numbness. These should subside if the tumor can be removed fully. However, surgery comes with the risk of complications, including:

Hearing loss
Facial numbness
Tinnitus
Headache
Fluid leakage
Balance issues

If you experience any of these after acoustic neuroma surgery, your doctor will work with you to determine the best path forward.

Diagnosis of an acoustic neuroma

Acoustic neuroma is most commonly diagnosed between ages 40 and 60⁴. There are several tests for acoustic neuroma diagnosis, including:

Audiogram: Tests hearing with headphones and a series of tones
AEP test: Auditory Evoked Potentials measures brain wave activity in response to sounds
Balance tests: Evaluates balance through eye movement patterns
MRI: Collects images of the tumor with contrast dye
CT scan: Captures images of the tumor if an MRI is not possible

How common is an acoustic neuroma?

Unilateral (one-sided) acoustic neuromas account for around 8% of all intracranial tumors, while bilateral (two-sided) acoustic neuromas are even more rare, accounting for less than 5% of all cases².

Affecting roughly one to two people per 100,000 each year, acoustic neuromas are more prevalent than you might think⁴. Some recognizable figures diagnosed with acoustic neuroma include Mark Ruffalo, Kelly Stafford and Tionne Watkins. Each has undergone successful surgery to remove the tumor.

Acoustic neuroma prevention

Because the causes of acoustic neuroma are generally difficult to determine, acoustic neuroma prevention is not an exact science. In general, following these tips may reduce your risk of developing an acoustic neuroma or help detect one early.

Limit exposure to radiation. Some research has shown that radiation may increase the risk of developing an acoustic neuroma².
Limit exposure to loud noise. Extended or frequent exposure to loud noise is a cited risk factor for acoustic neuroma, but research is inconclusive⁵.
Get tested regularly. Regular check-ups and hearing tests can help identify possible acoustic neuroma symptoms early and reduce your risk of developing severe symptoms.

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Living with an acoustic neuroma

Having an acoustic neuroma doesn’t just affect hearing, balance and the ability to communicate. These symptoms can also affect quality of life, especially mental health. Previous research has shown that acoustic neuroma and depression (as well as anxiety) are closely linked⁶. Patients may also experience fatigue due to compression on the brainstem.

FAQs about acoustic neuroma

Learn about these commonly asked questions about acoustic neuroma symptoms, diagnosis and treatment.

An acoustic neuroma cannot directly cause sinus problems. However, it can be difficult to tell the difference between the signs of acoustic neuroma and sinus issues because they share some common symptoms, including:

Facial numbness
Sinus pressure
Headaches

If you are experiencing any of these symptoms, consult a medical professional to get a clear diagnosis.

No, acoustic neuromas are not cancerous. They are considered benign and do not spread to other areas of the body. However, left untreated, these tumors can lead to symptoms like balance issues and hearing loss that negatively affect quality of life. This is why radiation treatment or surgery is sometimes necessary.

While uncommon, it is possible for an acoustic neuroma to return after surgery, especially if the tumor was only partially removed. Most recurrences happen three to five years after surgery, and in some cases, the tumor can regrow after up to 10 years⁷. This is why regular follow-up appointments are necessary after acoustic neuroma surgery.

Although radiosurgery treatment has a high success rate, recurrence is also possible with this method. The good news: A 2021 study showed that repeat radiosurgery after a failed attempt is associated with high tumor control rates⁸.

It is generally recommended to avoid flying for up to three months after acoustic neuroma surgery to limit fluid buildup as well as stress and pressure on the tumor site. If you have travel plans, consult with your doctor before flying.

The size of an acoustic neuroma varies from person to person, ranging from a few millimeters to several inches in diameter. However, acoustic neuromas are slow-growing and increase in size at an average rate of two millimeters per year, and at least 10% of all acoustic neuromas show no signs of growth once identified.

In most cases, an acoustic neuroma is not hereditary and occurs sporadically (i.e., without any genetic factors). Acoustic neuromas that are associated with NF2 are considered hereditary and are often bilateral; these account for fewer than 5% of all acoustic neuromas².

Sources

1. National Institute on Deafness and Other Communication Disorders. “Vestibular Schwannoma (Acoustic Neuroma) and Neurofibromatosis.” NIDCD, 18 Aug. 2015, www.nidcd.nih.gov/health/vestibular-schwannoma-acoustic-neuroma-and-neurofibromatosis.

2. Greene, Joshua, and Mohammed A Al-Dhahir. “Acoustic Neuroma.” Nih.gov, StatPearls Publishing, 17 Aug. 2023, www.ncbi.nlm.nih.gov/sites/books/NBK470177/.

3. G. Dupic, et al. “Stereotactic Radiosurgery for Vestibular Schwannomas: Reducing Toxicity with 11 Gy as the Marginal Prescribed Dose.” Frontiers in Oncology, vol. 10, 29 Oct. 2020, https://doi.org/10.3389/fonc.2020.598841.

4. Concheri, Stefano, et al. “Prognostic Factors for Hearing Preservation Surgery in Small Vestibular Schwannoma.” Audiology Research, vol. 13, no. 4, 3 July 2023, pp. 473–483, https://doi.org/10.3390/audiolres13040042.

5. Deltour, Isabelle, et al. “Exposure to Loud Noise and Risk of Vestibular Schwannoma: Results from the INTERPHONE International Case‒Control Study.” Scandinavian Journal of Work, Environment & Health, vol. 45, no. 2, 5 Nov. 2018, pp. 183–193, https://doi.org/10.5271/sjweh.3781.

6. Younes, Samira, et al. “Psychiatric Disorders in the Acoustic Neuroma: About a Case.” The Pan African Medical Journal, vol. 33, 4 June 2019, p. 80, www.ncbi.nlm.nih.gov/pmc/articles/PMC6689850/, https://doi.org/10.11604/pamj.2019.33.80.18398.

7. Scheer, Maximilian, et al. “Recurrences and Progression Following Microsurgery of Vestibular Schwannoma.” Frontiers in Surgery, vol. 10, 21 June 2023, pmc.ncbi.nlm.nih.gov/articles/PMC10322218/, https://doi.org/10.3389/fsurg.2023.1216093.

8. Balossier, Anne, et al. “Repeat Stereotactic Radiosurgery for Progressive Vestibular Schwannomas after Previous Radiosurgery: A Systematic Review and Meta-Analysis.” Neurosurgical Review, vol. 44, no. 6, 13 Apr. 2021, pp. 3177–3188, https://doi.org/10.1007/s10143-021-01528-y.