Usher's Syndrome

Key words: Deaf Education Information/Other Disabilities

Submitted by: Laurie Hellings

Topic: Usher's Syndrome

Task: To provide information about Usher's Syndrome, its educational implications, and resources to learn more about the syndrome.

Resources:

Bess, F.H., PhD. (Ed.) (1988). Hearing impairment in children. New York: Maple Press.

Fritsch, M.H., MD., & Sommer, A., MD. (1991). Handbook of congenital and early onset hearing losses. New York: Igaku-Shoin Medical Publishers.

Galloway, M. (1996). RE: Usher's Syndrome. Edudeaf (Online), Available: mwgalloway@gallau.gallaudet.edu .

Goeke, T. (1996). RE: Usher's Syndrome. Edudeaf (Online), Available: tgoeke@whitecap.psesd.wednet.edu .

Newby, H.A. & Popelka, G.R. (1992). Audiology. (6th ed.). Englewood Cliffs: Prentice Hall.

Pappas, D.G. (1985). Diagnosis and treatment of hearing impairment in children: A clinical manual. San Diego: College-Hill Press.

Programs for deaf-blind children and adults. (1995). American Annals of the Deaf, 140, 202-206, 224-225.

Stiefel, D.H. (1991). The madness of Usher's: Coping with vision and hearing loss (Usher syndrome type II). Corpus Christi: The Business of Living Publications.

Information:

What is Usher's Syndrome?

Usher's Syndrome is a recessive disorder which causes both visual and hearing impairment. Usher's only occurs in individuals who received the recessive gene for it from both of their parents, and consequently its incidence is very low (3 per 1000,000 births). However, this syndrome is the cause of approximately 10% of all hereditary deafness. People with Usher's are born with a congenital profound sensorineural hearing loss. Generally a child with Usher's will develop visual difficulties in childhood which result in night-blindness, usually by the age of ten. Visual acuity and visual fields will continue to decrease as the child grows older. This progressive loss of vision is called Retinitus Pigmentosa (RP). Usually RP will result in complete blindness between the ages of 20 and 30. Currently there is nothing that can be done to cure Usher's Syndrome. However, if a family is aware that they carry the gene for Usher's, genetic counseling is recommended.

Educational Implications/ Suggestions:

1. Children diagnosed with Usher's are sometimes given cochlear implants. The implants allow the child to make maximum use of what hearing he/she does have. There is little research to support this due to the newness of the implants. However, the idea is that the child may be able to learn speech and listening skills before the loss of vision occurs.

2. The child should have regular opthomologic exams to correct the child's vision as long as this is possible, and to provide any tools, such as a magnifying glass, which may be needed by the student while doing visual activities such as reading.

3. The educator, or parent, should be sure that there is adequate lighting provided for any visual activities.

4. When signing, wearing a black shirt aides sign reception. For the child with tunnel vision, it is best to sign small around the face. The child should be permitted to adjust their distance to the signer.

5. Lastly, the child may benefit from a hearing ear dog before vision loss. After the loss of vision occurs, the child's dog could be both a hearing ear dog and seeing eye dog. Many dogs can be trained to provide hearing and visual services to people requiring both.

Resources for more Information or Services:

Art Roehrig at Gallaudet University has Usher's and works with students and other interested in the syndrome. Art can be reached at the Disabled Services and Program number: (202) 651-5256.

The Cochlear Implant Club International (CICI) provides support services and promotes cochlear implants through both research and technology.
Cochlear Implant Club International
P.O. Box 464
Buffalo, NY 14223-0464

The Deaf Blind Seattle Community is comprised mostly of people with Usher's. The deaf blind community center is a place which can be contacted to provide national resources. The number that the Deaf Blind Seattle Community can be reached at is: (206) 323-9178. The e-mail address is dbcsceawa@aol.com .

The Helen Keller Centers for Deaf-Blind Youth and Adults is a national center with 10 regional offices which provide extensive evaluative and rehabilitative services to people who are deaf and blind. Region five includes the states of Illinois, Indian, Missouri, Minnesota, Wisconsin, and Ohio.
Call:
(312) 726-2090 (voice)
(312) 726-2810 (TTY)

Write to :
205 W. Wacker Dr.
Suite 919
Chicago, IL 60606

National Headquarters
Call:
(516) 944-8900 (voice)
(516)944-8637 (TTY)

Write to:
111 Middle Neck Rd.
Sands Point, NY 11050

A regional/local program which can also be contacted for more information is the Comprehensive Program for the Deaf (CPD)
Call:
(614)263-5151 (voice)
(614)263-2299

Write to:
Ohio Deaf-Blind Outreach Program and L.I.F.E. Transition Program
4110 N. High St.
Columbus, OH 43214

Insights: As teachers of the hearing impaired, it is important for us to be well prepared. This means that not only do we need to know about hearing impairment, we also need to know about other syndromes that cause other disabilities in addition to hearing impairment. Usher's Syndrome is one such impairment. As teachers we need to educate ourselves on as many causes of hearing loss, and the educational implications of those causes, as we can.

Questions:

What are some other ways that teachers can help children with Usher's to enable the child to see better?

What are some additional resources that can provide assistance both to the people with Usher's and their families?

What other syndromes cause hearing loss and an additional disability? What can I do educationally for children with other syndromes?

Uploaded by: Melissa Close/ Kent State University/Deaf Education Major