Hearing Loss
Diagnosis and Audiological Evaluation
Americans with Disabilities Act (ADA)
Diagnosis
Before your hearing loss can be treated, your hearing must be tested by an audiologist. He or she will determine the degree and type of hearing loss you have. Here is a list of some of the tests an audiologist may administer. Not everyone needs all these tests. Your physician or the audiologist may recommend the ones you need, based on your family history and the symptoms of your hearing loss.
Audiological Evaluation
1. Otoscopy
Otoscopy is a physical examination of your outer ear, ear canal, and ear drum. An audiologist will use an otoscope to look for things such as holes in your ear drum, redness of the ear drum, ear wax build-up, or any signs of infection.
2. Tympanometry
Tympanometry is a measure of the stiffness of the eardrum and tells us how the bones in your middle ear are functioning. This test helps detect fluid in the middle ear, broken or dislocated middle ear bones, a hole in your eardrum , and a middle ear disease called otosclerosis. The audiologist will put a soft probe into your ear canal and a machine releases a small amount of pressure. The instrument measures movement of your eardrum in responses to the pressure changes and records the outcome on a graph.
3. Pure Tone Testing 
Pure tone testing is the most common type of hearing evaluation. The audiologist may use headphones or insert earphones, which are like ear plugs, for the air conduction portion. During the testing, you will hear tones at different pitches and volumes, and raise your hand when you hear the sound. The audiologist records the thresholds for each pitch on a graph called an audiogram. A threshold is the lowest level of loudness that you can hear the tone. Pure tone testing also includes bone conduction testing. During this type of testing, you will wear a headband on your head while the tones are presented. You can hear the sounds as they are conducted through your skull. An audiologist will then compare your pure tone results of the air conduction to the bone conduction and determine which part of the hearing mechanism is responsible for any hearing loss.
4. Otoacoustic Emission (OAE) Testing
The cochlea is the part of your ear that receives information from incoming sounds and sends the sounds to your brain. The cochlea contains many nerve endings called “hair cells”. When these nerve ending work properly, sounds are accurately sent to your brain, and your brain interprets these sounds as music, speech, or noise. Normal cochleas have special hair cells that produce sound. OAE testing records the sounds that your ear produces and helps the audiologist determine if your inner ear is transmitting sound to your brain.
5. Auditory Brainstem Response (ABR)
ABR testing is a noninvasive diagnostic tool that measures your brain waves in response to an auditory stimulus. It measures the activity of the auditory pathway that is located in your brainstem. For further information on ABR testing, go to these websites:
The tests described above are just a few of the possible ways that audiologists determine if your hearing is normal. To learn more about the diagnosis of hearing loss, go to the following web sites:
American Speech and Hearing Association
Treatment
The options for treating WS vary depending on your particular WS characteristics. Part of hearing loss treatment is referral to a genetic counselor. Treatment may include one or a combination of the following options. Remember that the treatment(s) chosen by one person with WS, may not be appropriate for you or your child.
1. Genetic Testing & Counseling
2. Options for treating hearing loss include:
- Hearing Aids or Amplification
- Cochlear Implants
- Sign Language
- Speech Therapy
You or your child can choose from a variety of schools for hearing impaired individuals. Consider your child's specific needs and degree of hearing loss when you choose a school. Many schools specialize in education for individuals with hearing impairments. Some schools rely completely on American Sign Language, some include a combination of oral speech and sign language, and others use only oral speech. In addition to the specialized schools, you may choose to send your child to a public school within your community. The United States' Individuals with Disabilities Education Act (IDEA) states that “every child has the right to a free and appropriate education in the least restrictive environment.” In other words, your child should be provided with the appropriate resources within a school near you.
Links that may be helpful:
Laurent Clerc National Deaf Education Center
A list of elementary, middle, and high schools for deaf students in the United States, complete with links to each one.
National Center on Deafness
This website offers a list of residential schools for the deaf in the United States.
Help is Hear!
This site explains different educational choices and includes a list of a few instutitions in the US.
Oral Deaf Educational Insutitions
This is a list of some of the private Oral Deaf Education Schools in North America, Australia, and the United Kingdom.
Royal Association for Deaf People
This site lists mainstream schools in UK with resources for hearing impaired.
National Deaf Children’s Society
This UK website explains choices for deaf education in England and Wales. The site also offers valuable resources for parents and caregivers.
Aussie Deaf Kids
This site includes a complete list of schools in Australia that may provide educational services to children with hearing impairments. The schools are organized by state/territory.
Americans With Disabilities Act
The Americans With Disabilities Act (ADA) of 1990 is designed to prohibit any form of prejudice or discrimination toward persons who have disabilities, including hearing loss and deafness. Many people in the United States prefer to distance themselves from individuals who have disabilities. This attitude can lead to discrimination on the part of public institutions, employers, landlords, and health care facilities. The ADA was created to help people with disabilities receive equal and fair treatment by employment agencies, schools and universities, and health and public service agencies. The Act guarantees that the Federal Government will help implement the standards made on behalf of persons with disabilities.
For more information on the ADA, visit the Americans with Disabilities home page.
Literature & Multimedia
The following is a list of resources that may be helpful in understanding genetics and/or hearing loss.
Kahn, A. (2007). Waardenburg Syndrome. San Diego, CA: Plural Publishing, Inc. (IBSN# 1-59756-021-9)
Milunsky, A. (2001). Your genetic destiny: Know your genes, secure your health, save your life. Cambridge, MA: Perseus Publishing.
(IBSN# 0738203777)Schwartz, S. (1996). Choices in deafness: A parent's guide to communication options (2nd ed.). Bethesda, MD: Woodbine House, Inc.(IBSN#0-933149-85-9)
Van Cleve, J.V. (2004). Genetics, disability, and deafness (2nd ed.). Washington, DC: Gallaudet University Press. (IBSN# 1563683075)
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