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Acoustic Neuroma

Important
It is possible that the main title of the report Acoustic Neuroma is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms

* Acoustic Neurilemoma
* Bilateral Acoustic Neuroma
* Cerebellopontine Angle Tumor
* Fibroblastoma, Perineural
* Neurinoma of the Acoustic Nerve
* Neurofibroma of the Acoustic Nerve
* Schwannoma of the Acoustic Nerve

Disorder Subdivisions

* None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

* Neurofibromatosis Type 2
* Bell's Palsy
* Tinnitus

General Discussion

Acoustic Neuroma is a benign (non-cancerous) growth that begins at the 8th cranial nerve, which runs from the brain to the inner ear. Any disruption of the signals sent along the 8th cranial nerve will interfere with hearing and with the patient’s balance.
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Symptoms

The earliest symptoms of an Acoustic Neuroma almost invariably (about 90% of the cases) include hearing loss in one ear and a ringing in the ear (tinnitus). These symptoms occur due to pressure from the tumor on the 8th cranial nerve. The 8th cranial nerve is double barreled; one part deals with hearing and the other with balance and the body’s sense of position in space. Unsteadiness and balance problems may occur, therefore, as the tumor grows.

An Acoustic Neuroma may press on or squeeze (compress) the facial nerve (7th cranial nerve) resulting in facial muscle weakness. The trigeminal nerve (5th cranial nerve) is responsible for sensation on the skin of the face and the surface of the eye; if the 5th cranial nerve becomes involved it may lead to facial numbness.

An Acoustic Neuroma may also grow in the direction of the brain stem and press on the rear portion of the brain (cerebellum). This may result in an impaired ability to coordinate muscle movement (ataxia) of the arms and legs. Downward expansion of the tumor may produce numbness in the mouth, impaired speech (dysphagia), and/or hoarseness.

The growth of an Acoustic Neuroma may increase pressure within the skull (intracranial pressure) resulting in personality changes and an impaired ability to think and reason. Pressure may increase on the facial nerve resulting in facial twitching and a lack of balance (asymmetry) to the face. Sudden expansion of the tumor may be caused by excessive bleeding (hemorrhage) and/or swelling due to an abnormal accumulation of fluid (edema).
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Causes

The exact cause of an Acoustic Neuroma is not known. Among a small group of people, there appears to be a hereditary predisposition. It is among this small group that Acoustic Neuromas may be found on both sides of the brain.

A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease.

The gene associated with sporadic Acoustic Neuroma has been mapped to chromosome 22 (22q12.2).

Acoustic Neuromas can also occur as a symptom of Neurofibromatosis Type II, which is a hereditary disorder characterized by multiple benign tumors. (See related disorders section of this report.)
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Affected Populations

The ANA suggests that acoustic neuromas may often be overlooked since autopsies show 5 percent of persons who have never been diagnosed do have confirmed signs of the neuroma. About one person in 100,000 has acoustic neuromas large enough to cause hearing loss. Most neuromas are found in patients between the ages of 30 and 60.
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Related Disorders

Symptoms of the following disorders can be similar to those of Acoustic Neuroma. Comparisons may be useful for a differential diagnosis:

Bell's palsy is a nonprogressive facial nerve disorder characterized by sudden onset of facial paralysis. The paralysis results from compression and the excessive accumulation of blood around the 7th cranial (facial) nerve. Early symptoms of Bell's Palsy may include a slight fever, pain behind the ear, a stiff neck, and facial muscle weakness on one side of the face, and/or facial stiffness. Symptoms may begin very suddenly or over the course of several hours and sometimes follows exposure to cold or a draft. Part or all of the face may be affected. (For more information on this disorder, choose "Bell's Palsy" as your search term in the Rare Disease Database.)

Neurofibromatosis Type 2 is a rare inherited disorder characterized by bilateral (both sides) Acoustic Neuromas, the appearance of brown spots (cafe-au-lait macules) on the side and fibrous tumors (neurofibromas) on the skin. This disorder may also be associated with tumors in the brain, spinal cord and other areas of the body. Major symptoms may include a buzzing or ringing sound in the ears (tinnitus) and eventual loss of hearing. (For more information on this disorder, choose "Neurofibromatosis" as your search term in the Rare Disease Database.)

Tinnitus is a person's subjective experience of sound that does not exist in the environment. The sounds of Tinnitus have been described as clicking, buzzing, whistling, ringing and/or roaring. These sounds may always be present, or they may come and go. Tinnitus is frequently associated with a loss of hearing. (For more information on this disorder, choose "Tinnitus" as your search term in the Rare Disease Database.)
Standard Therapies

Three treatment options are available. These are observation, microsurgery, and radiation therapy.

Observation
Especially if the patient is elderly, the more advantageous course of action may be to “watch and wait” and to have periodic MRI procedures to determine how fast the neuroma is growing. If the growth rate is slow enough, alternative treatments may not be worth the effort.

Watchful waiting is also appropriate if a patient with hearing only in one ear is found with an acoustic neuroma in that ear. The patient may choose to live on with the neuroma as long as it is not life-threatening rather than become completely deaf.

Microsurgery
Microsurgery may be performed to affect partial or total tumor removal. Partial tumor removal is undertaken to reduce the risk of unwanted surgical complications. In other words, it may be easier and safer to take out part of the growth rather than the whole. If the tumor is very large or if the patient is very old, partial removal may be more appropriate.

When total tumor removal is indicated, the objective of the procedure is to protect the facial nerve and avoid facial paralysis. In addition, of course, the surgeon tries to preserve hearing as much as possible in the affected ear.

Radiation Therapy
Three dimensional focusing of radiation has become more accurate in recent years so that patients may be treated at one session on an outpatient basis or, alternatively, smaller doses may be delivered over several sessions. The objective is to aim so accurately that the tumor cells are affected and the damage to surrounding cells is minimized.

Post-treatment problems may include: headache, obstruction or leakage of fluid that surrounds the brain and spinal cord (cerebrospinal fluid), inflammation of the membranes that surround the brain (meningitis), and/or decreased mental alertness due to blood clots or obstruction of flow of cerebrospinal fluid.

The facial nerve may be damaged by the Acoustic Neuroma or as a result of surgery. In some cases, it may be necessary for the surgeon to remove portions of the facial nerve, resulting in temporary or permanent facial paralysis. The regrowth of the nerve (regeneration) and restoration of function to the muscles of the face may take up to a year. If the facial paralysis persists, a second surgery may be performed to connect the healthy portion of the facial nerve to the hypoglossal nerve in the neck. This may bring some improvement in function to the muscles of the face. There are also other surgical procedures to aid in reanimating the sagging face. Continued facial paralysis may cause food to "get lost" in the mouth on the affected side, which may eventually cause dental problems.

Eye problems may develop in approximately 50 percent of patients following surgical removal of an Acoustic Neuroma. Double vision (diplopia) may occur if there is pressure on the 6th cranial nerve, and there may be impairment of the muscles of the eyelids. Artificial tears or eye lubricants may be needed.
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Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.

For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

Researchers are studying the association between unilateral vestibular schwannomas (acoustic neuromas) in children and young adults and Neurofibromatosis Type II (NF2). Affected individuals will have been diagnosed before the age of 25 with unilateral vestibular schwannoma and may or may not have developed other symptoms of NF2. For more information, contact:

Mia MacCollin, M.D.
(617) 726-5725
E-mail: maccollin@helix.mgh.harvard.edu

A clinical trial sponsored by the House Ear Institute in Los Angeles, California, is designed for the long term in order to better understand the natural progression (natural history) of acoustic neuromas. The study’s purposes are; (1) to develop expertise in several medical centers to identify and recruit patients for this trial; (2) to collect tissue samples from recruited patients; (3) to measure in vivo growth rates of these tumors; (4) to develop a technique that predicts behavior of the tumor; and (5) to integrate the information collected with a larger database of patients with neurofibromatosis type II.

For information, contact:
Dr. William H. Slattery III, study chair
House Ear Institute
Tel: 213-483-9930
References

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No: 101000; Last Update: 12/18/01.

PUBLICATION FOR PATIENTS
Acoustic Neuroma Association. ACOUSTIC NEUROMA: A Basic Overview. ANA: Cummings, GA. 2001:11pp.

TEXTBOOKS
Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:683, 1447.

Berkow R., ed. The Merck Manual-Home Edition. Whitehouse Station, NJ: Merck Research Laboratories; 1997:384, 1012.

Larson DE, ed. Mayo Clinic Family Health Book. New York, NY: William Morrow and Company, Inc; 1996:584.

REVIEW ARTICLES
Casselman JW. Diagnostic imaging in clinical neuro-otology. Curr Opin Neurol. 2002;15:23-30.

Maeta M, Saito R, Nameki H. False-positive magnetic resonance image in the diagnosis of small acoustic neuroma. J Laryngol Otol. 2001;115:842-44.

Stpkovits EM, Graamans K, Vasbinder GB, et al. Assessment of vestibular schwannoma growth: application of a new measuring protocol to the results of a longitudinal study. Ann Otol Rhinol Laryngol. 2001;110:326-30.

Brophy BP. Acoustic neuroma—surgery or radiosurgery? Stereotact Funct Neurosurg. 2000;74:121-28.

Reed N, Gutmann GH. Tumorigenesis in neurofibromatosis: new insights and potential therapies. Trends Mol Med. 2001;7:157-62.

Brackman DE, Owens RM, Friedman RA, et al. Prognostic factors for hearing preservation in vestibular schwannoma surgery. Am J Otol. 2000;21:417-24.

JOURNAL ARTICLES
Magnan J, Barbieri M, Mora R, et al. Retrosigmoid approach for small and medium-sized acoustic neuromas. Otol Neurotol. 2002;23:141-45.

Greenberg JS, Manolidis S, Stewart MG, et al. Facial nerve monitoring in chronic ear surgery: US practice patterns. Otolaryngol Head Neck Surg. 2002;126:108-14.

Petit JH, Hudes RS, Chen TT, et al. Reduced-dose radiosurgery for vestibular schwannomas. Neurosurgery. 2001;49:1299-306, discussion 1306-07.

Bush DA, McAllister CJ, Loredo LN, et al. Fractionated proton beam radiotherapy for acoustic neuroma. Neurosurgery. 2002:50:270-75.

Browning S, Mohr G, Dufour JJ, et al. Hearing preservation in acoustic neuroma surgery. J Otolaryngol. 2001;30:307-15.

Kalamarides M, Grayeli AB, Bouccara D, et al. Hearuing restoration with auditory brainstem implants after radiosurgery for neurofibromatosis type 2. J Neurosurg. 2001;95:1028-33.

Pothula VB, Lesser T, Mallucci C, et al. Vestibular schwannomas in children. Otol Neurotol. 2001;22:903-07.
Resources

The Children's Tumor Foundation: Ending Neurofibromatosis Through Research
95 Pine Street
16th Floor
New York, NY 10005
Tel: (212)344-6633
Fax: (212)747-0004
Tel: (800)323-7938
TDD: (212)344-6633
Email: info@ctf.org
Internet: http://www.nf.org

American Tinnitus Association
P.O. Box 5
Portland, OR 97207
United States
Tel: 5032489985
Fax: 5032480024
Tel: 8006348978
Email: tinnitus@ata.org
Internet: http://www.ata.org

Deafness Research Foundation
1050 17th Street, NW
Suite 701
Washington, DC 20036
United States
Tel: 2022895805
Fax: 2022931805
Tel: 8008295934
Email: drf@drf.org

Acoustic Neuroma Association
600 Peachtree Parkway
Suite 108
Cumming, GA 30041
USA
Tel: 7702058211
Fax: 7702050239
Email: ANAUSA@AOL.COM
Internet: http://www.ANAUSA.org

Alexander Graham Bell Association for the Deaf, Inc.
3417 Volta Place, NW
Washington, D.C. 20007-2778
United States
Tel: 2023375220
Fax: 2023378314
Tel: 8004327543
Email: agbell2@aol.com
Internet: http://www.agbell.org

Better Hearing Institute
515 King Street, Suite 420
Alexandria, VA 22314
United States
Tel: 7036843391
Fax: 7037509302
Tel: 8884327435
Email: mail@betterhearing.org
Internet: http://www.betterhearing.org

NIH/National Institute on Deafness and Other Communication Disorders (Balance)
National Temporal Bone, Hearing
and Balance Pathology Resource Registry
Massachusetts Eye & Ear Infirmary
243 Charles Street
Boston, MA 02114-3096
Fax: (617)573-3838
Tel: (800)822-1327
TDD: (888)561-3277
Email: TBRegistry@meei.harvard.edu
Internet: http://www.tbregistry.org

Acoustic Neuroma Association of Canada
Box 369
Edmonton
Alberta, T5J 2J6
Canada
Tel: 7804283384
Fax: 7804384837
Tel: 8005612622
TDD: 4034283383
Email: anac@compusmart.ab.ca
Internet: http://www.anac.ca

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