Significant hearing loss has been reported in more than 50% of individuals with osteogenesis imperfecta (OI). For individuals with Type I OI (the largest population experiencing severe hearing loss), this condition usually begins around 20 to 30 years of age.

Types of Hearing Loss

There are three main types of hearing loss:

Conductive: This usually results from a physical problem in the external or middle ear. It may occur as a result of ear infection, blockage of the middle ear, or from fixation or fracture of the stapes, one of three tiny bones in the middle ear that transmits sound through the middle ear to the fluid in the inner ear.

Sensorineural: This type of hearing loss occurs when the inner ear is not transmitting sounds normally to the brain.

Mixed: Hearing loss that occurs when both the inner and middle ear are involved.

Hearing losses are also classified according to the degree of severity (mild, moderate, severe, or profound) and the frequency affected (low, high, or all frequencies).

Symptoms

Some of the signs of hearing loss are:

- Difficulty understanding certain words or parts of words

- Frequently asking others to repeat themselves.

- Difficulty understanding others on the telephone.

- Turning up the sound on the television or radio to a level that is too loud for others in the room.

-Difficulty hearing in noisy surroundings.

As it progresses, a hearing loss can interfere with communication, performance at work social activities, and personal relationships. Left unassisted, a hearing loss may even result in isolation and depression.

Diagnosis

Any child with OI who demonstrates articulation problems, speech delays, or recurrent ear infections should have a formal audiologic assessment. Young adults should have baseline assessments for later comparison. Adults experiencing tinnitus (ringing in the ear) or symptoms of hearing loss should also have an audiologic assessment.

Surgery

Individuals with a conductive hearing loss that is severe and progressive may helped with a surgical procedure known as a stapedectomy. In this procedure, the fixed foot plate of the stapes is replaced by a prosthesis that allows for the normal transmission of sound waves to the inner ear. It should be noted, however, that this operation should not be considered routine in OI because of tissue fragility. Use of a laser to do the bony work improves accuracy and visibility during the surgery. There are also many other pre- and post-operative issues that need to be assessed, discussed, and clarified before any individual with OI may be considered a "good candidate" for surgery. As a general rule, patients should seek treatment centers where the otologists (physicians who specialize in ear disorders) have considerable experience with stapes surgery.

Hearing Aids

While hearing aids can't cure a hearing loss or duplicate natural hearing, they can provide amplification that can help all age groups. They come in a number of shapes and have different amplification abilities. The style that is right for you depends on the severity and range of the hearing loss.

When choosing a hearing aid dealer, you should ask questions about the warranty and the service that will be offered after your purchase. You should also make sure the individual is licensed in your state and is board certified through the National Hearing Aid Society as a Hearing Instrument Specialist or Audiologist.

Don't Be Afraid to Seek Help

If you or a family member is experiencing hearing loss, don't resign yourself to a life of frustration and suffering. There are many assistive listening devices to help you, including doorbells that light to alarm clocks that vibrate in your pillow. There are also organizations that can educate you about the devices and services that are available.

Additional Resources

Your local telephone service provides a listing of communication devices.

American Association of Retired Persons
601 E Street, NW
Washington, DC 20049
(202) 434-2477

AARP has a variety of resources about hearing loss and product reports that describe how to choose the most appropriate equipment.

American Speech-Language-Hearing Assoc.
10801 Rockville Pike
Rockville, MD 20852
(800) 638-8255

The American Speech-Language-Hearing Association (ASHA) is a national professional, scientific, and credentialing association for speech-language pathologists and audiologists. Part of its mission is to ensure that individuals with hearing disorders have access to high quality services to help them communicate effectively. A toll-free HELPLINE is available for those seeking information.

Capcom, Inc.
6707 Old Dominion Drive, Suite 210
McLean, VA 22101
(800) 241-2ADA

Capcom, Inc., is a nonprofit organization that provides free message relay services. It also provides information referrals regarding telecommunication devices.

Hear Now
9745 E. Hampden Ave., Suite 300
Denver, CO 80231
(800) 648-4327

Hear Now coordinates a national hearing aid bank and provides hearing aids for individuals with limited financial resources.

Hearing Health
P.O. Box Drawer V
Ingleside, TX 78362
(512) 776-7240

Hearing Health publishes a bimonthly, independent consumer magazine that focuses on hearing loss and hearing issues.

Miracle Ear Children's Foundation
P.O. Box 59261
Minneapolis, MN 55459-0261

Miracle Ear Children's Foundation provides free hearing aids for qualifying children in low income families.

This information is brought to you by the
NIH Osteoporosis and Related Bone Diseases~National Resource Center (ORBD~NRC)
and the Osteogenesis Imperfecta Foundation

National Institutes of Health
Osteoporosis and Related Bone Diseases
National Resource Center
1232 22nd St., NW
Washington, DC 20037-1292
Tel: 800/624-BONE or 202/223-0344
Fax: 202/293-2356, TYY: 202/466-4315
http://www.osteo.org
E-mail: orbdnrc@nof.org

The National Resource Center is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases with contributions from the National Institute of Child Health and Human Development, National Institute of Dental and Craniofacial Research, National Institute of Environmental Health Sciences, NIH Office of Research on Women's Health, Office of Women's Health, PHS, and the National Institute on Aging. The Resource Center is operated by the National Osteoporosis Foundation, in collaboration with the Paget Foundation and the Osteogenesis Imperfecta Foundation.