Acoustic Neuroma Association
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What is Acoustic Neuroma?

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Important Points


An acoustic neuroma, also called a vestibular schwannoma, is a rare, non-cancerous tumor.

 • They do not spread (metastasize) to other parts of the body. The brain is not invaded by the acoustic tumor, but the tumor pushes on the brain as it enlarges.

 • Acoustic tumors constitute 6%-10% of all brain tumors and are found in roughly one of every 100,000 people per year in the United States. This translates to about 2,500-3,000 newly diagnosed acoustic tumors per year.

 • In most cases, these tumors grow slowly over a period of years, but sometimes the rate of growth is more rapid.

 • Symptoms can be mild or severe, and multiple symptoms might develop rather rapidly.

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 • The first signs or symptoms usually are related to ear function and include tinnitus (ear noise/ringing in the ear) and often disturbances in hearing on one side.

 • When large tumors cause severe pressure on the brainstem and cerebellum of the brain, vital functions that sustain life can be threatened.

 • The most common method of diagnosing an acoustic tumor is by a detailed MRI of the head.

 • The treatment options are observation (watchful waiting), surgical removal or radiation therapy. The choice of treatment may be based on tumor size, hearing in the ear at time of diagnosis, patient age and health, and patient preference.

 (Printed with permission of House Clinic – www.houseclinic.com)

Anatomy of an Acoustic Neuroma

  • An acoustic neuroma, known as a vestibular schwannoma, is a benign (non-cancerous) growth that arises on the eighth cranial nerve leading from the brain to the inner ear. This nerve has two distinct parts, one part associated with transmitting sound and the other with sending balance information to the brain from the inner ear.

  • The eighth nerve, along with the facial or seventh cranial nerve, lie adjacent to each other as they pass through a bony canal called the internal auditory canal. This canal is approximately 2 cm (0.8 inches) long and it is generally here that acoustic neuromas originate from the sheath surrounding the eighth nerve. The seventh or facial nerve provides motion to the muscles of facial expression.

  • Acoustic neuromas usually grow slowly over a period of years. They expand in size at their site of origin and when large can displace normal brain tissue. The brain is not invaded by the tumor, but the tumor pushes the brain as it enlarges. The slowly enlarging tumor protrudes from the internal auditory canal into an area behind the temporal bone called the cerebellopontine angle. The tumor assumes a pear shape with the small end in the internal auditory canal.

  • Larger tumors can press on another nerve in the area (the trigeminal nerve) which is the nerve of facial sensation. Vital functions to sustain life can be threatened when large tumors cause severe pressure on the brainstem and cerebellum. 

  • Tumors are typically described as small (less than 2 cm), medium (2-4 cm) or large (more than 4 cm).

 

Anatomical Diagrams

Fig1Anatomy600

Figure 1. The normal anatomy of the ear. The outer ear funnels sound to the eardrum, which vibrates three tiny bones called ossicles (malleus, incus and stapes). The spiral-shaped cochlea is filled with liquid, which moves in response to vibrations. As the fluid moves, thousands of hair cells are stimulated, sending signals along the cochlear nerve (responsible for hearing) to the brain. Attached to the cochlea are three semicircular canals positioned at right angles to each other. The three canals are able to sense head position and posture. Electrical signals from the semicircular canals are carried to the brain by the superior and inferior vestibular nerves (responsible for balance). The cochlear and vestibular nerves form a bundle inside the bony internal auditory canal. Inside the canal, the vestibulocochlear nerve lies next to the facial nerve (responsible for facial movement). (Printed with permission of the Mayfield Clinic – www.mayfieldclinic.com)

Fig2Anatomy600

Figure 2. An acoustic neuroma expands out of the internal auditory canal displacing the cochlear, facial and trigeminal nerves located in the cerebellopontine angle. Eventually the tumor can compress the brainstem. (Printed with permission of the Mayfield Clinic – www.mayfieldclinic.com)

Fig3Anatomy600

Figure 3. Acoustic neuromas are classified according to their size. MRI scans and correlative illustrations of small (intracanalicular), medium and large acoustic neuromas. (Printed with permission of the Mayfield Clinic – www.mayfieldclinic.com)

Educational Video

 

 

Types of Acoustic Neuroma/ Rate of Occurrence

Unilateral (one side) occurs spontaneously without any evidence of family history, accounts for 95% of acoustic neuromas

Bilateral (both sides) is most likely caused by a genetic condition called Neurofibromatosis Type 2 (NF2) which affects approximately 1 in every 40,000 people. NF2 will be suspected if a parent/sibling/child of the patient has NF2 and acoustic neuroma occurs before age 30 or if the patient has had a meningioma, glioma, or cataract.

Most recent publications suggest that the incidence of acoustic neuromas is rising. This is because of advances in MRI scanning both on incidental scans and for patients experiencing symptoms.  It is estimated that the instances of acoustic neuroma are between 1 to 3.5* in every 100,000.

*In your research you may find differing statistics on acoustic neuroma occurrence.  ANA provides a range that is inclusive of data from medical center websites and research studies.

What Causes  Acoustic Neuroma?

The cause of acoustic neuroma is not well understood. For most acoustic neuromas, the cause at the level of the cellular machinery is the failure of a “governor” gene to exert its effect in suppressing the growth of Schwann cells—those cells responsible for coating nerve fibers with insulation. The result is “wart-like” growth of these cells to produce the neuroma.

The only environmental exposure that has been definitively associated with an increased incidence is high-dose, therapeutic radiation exposure to the head. While studying the association between cell phone use and the development of these tumors is difficult, the scientific community does not generally accept that any relationship has been proven.

For the most part it is not an inherited disease; however, 5% of cases are associated with a genetic disorder called neurofibromatosis type 2. These individuals demonstrate two-sided vestibular tumors often associated with other tumors around the brain and/or in the spine. The vast majority of acoustic neuromas are sporadic (nonhereditary).

(Printed with permission of the USC Acoustic Neuroma Center at Keck Medicine of USC www.acousticneuroma.keckmedicine.org)

What Determines the Symptoms?

Symptoms can be associated with the size of the tumor but this is not always consistent. Many patients experience few symptoms despite the presence of a large tumor. Small tumors are defined at 2 centimeters or less, medium tumors are between 2 and 4 centimeters, and large tumors are over 4 centimeters. Smaller tumors can often be associated with few symptoms whereas large compressive tumors can be life threatening.

(Printed with permission of the USC Acoustic Neuroma Center at Keck Medicine of USC www.acousticneuroma.keckmedicine.org)

Neurofibromatosis (NF2)

NF2, a genetic disorder, occurs with a frequency of 1 in 30,000 to 1 in 50,000 births. The hallmark of this disorder is bilateral acoustic neuromas (an acoustic neuroma on both sides). This creates the perplexing problem of the possibility of complete deafness if the tumors are left to grow unchecked. Preventing or treating the complete deafness that may befall individuals with NF2 requires complex decision making. The trend at most academic U.S. medical centers is to recommend treatment for the smallest tumor which has the best chance of preserving hearing. If this goal is successful, then treatment can also be offered for the remaining tumor. If hearing is not preserved at the initial treatment, then usually the second tumor, in the only-hearing ear, is just observed. If it shows continued growth and becomes life-threatening, or if the hearing is lost over time as the tumor grows, then treatment is undertaken. This strategy has the highest chance of preserving hearing for the longest time possible.

There are now several options to try to rehabilitate deafness in NF2 patients. Implanting the hearing part of the brainstem (Auditory Brainstem Implant) can help restore some sound perception to these patients. Also cochlear implants can be used if the cochlear nerve is preserved following surgery. Radiosurgery may be an option although stereotactic radiosurgery may not have the effect on the NF2 patient as in patients with unilateral sporadic tumors.There are some centers using radiation therapy for NF2 with mixed results. The risk of malignant transformation after radiation is higher in this group. Recent studies have shown that these individuals may have more tumors that are resistant to radiation, due to the cell type. These cases should be handled in centers with very experienced skull base teams.

 

 

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