I've worked extensively with individuals with vision loss. I hold an M.S. degree in rehabilitation counseling from East Carolina University.
Understanding Usher Syndrome
Usher’s syndrome comprises a group of disorders characterized by a combination of sensory impairments affecting the visual and the audio vestibular systems. The dual sensory disorder is the Leading cause of deaf-blindness in humans through an autosomal recessive genetic pattern. In other words, if both parents carry an abnormal gene, there is a 25% chance a child will have the inherited disease with each pregnancy.
Primarily, congenital hearing loss may be profound or mild in people with Usher syndrome. In addition, the progressive eye disease, retinitis pigmentosa (RP), impacts sight. In fact, once RP has been confirmed by an ophthalmologist in a person with hearing loss, then the individual is said to have Usher syndrome.
Although certain genes involved with the disease have been identified and cloned, there is no cure for the estimated twenty-five thousand individuals with Usher syndrome. Moreover, research has demonstrated people born with hearing loss are two to three times as likely to develop visual impairments compared to hearing peers. However, deaf-blindness is rare, accounting for approximately 6% of the total population of individuals who are deaf.
Research reveals about one in every ten pupil at schools for the deaf have Usher syndrome. In the United States, students who are deaf-blind frequently receive services from agencies which assist individuals who have visual impairments or organizations who support people who are deaf. Essentially, finding state supported programs and specialists in educating people who are deaf-blind is difficult in America. Often in other areas of the world, educational assistance for people with deaf-blindness goes ignored.
Important Educational Facts About People who are Deaf-Blind
As a counselor and teaching in the classroom, I’ve worked with people who are deaf-blind. I’ve approached interacting with these individuals with several methods, including tactile sign language and enlarging printed materials. This is because each individual experiencing deaf-blindness may require separate techniques for communicating at different times. In order to achieve positive educational outcomes, a teacher should be aware of various effective techniques when instructing students with dual-sensory impairments.
Coincidentally, the rate of deaf-blindness among people who are deaf is minor. Likewise, the total loss of the use of both senses is uncommon in people with Usher syndrome. Nevertheless, a person with Usher syndrome may need changes in accommodations as a result of hearing and/or gradual vision loss. Therefore, comprehending the three types of Usher syndrome is pivotal for special and general education teachers:
Basic Variations of Usher Syndrome
- Type I (Ush1): A Person already has profound hearing loss from birth. RP starts in adolescence. Balance problems may be present and intensify.
- Type II (Ush2): A person may have varying hearing loss. The individual may use hearing aids without sign language skills. Usually, individuals with Ush2 don’t identify with the general deaf community.
- Type III (Ush3): These individuals experience normal or mild auditory difficulties with progressive vision and hearing loss at puberty. Often, maintaining balance is an issue.
Fourteen Instructional Techniques for People with Usher Syndrome in the Classroom
- An interpreter may be required. Allow the interpreter time to explain a topic by providing instruction at a steady pace.
- Arrange furniture for easy and open movement in the classroom. Provide adequate space between desks. Drawers and doors should be closed.
- Assisted Hearing devices, screen reading software, and Braille displays may be needed. These devices are permitted in the classroom in accordance with the IEP and the individual’s disability.
- Avoid wearing sparkly jewelry. Dress in simple patterns. Flashy colors can impact visual functioning for a student with Usher syndrome.
- Conduct the lesson from an uncluttered background. Eliminate unnecessary movement.
- Consult with the Teacher of the Visually Impaired (TVI) and the Orientation and Mobility specialist (O&M) on ways to increase safe movement in the classroom, including use of human guide techniques.
- Enhance your tactile skills. Allow students with Usher syndrome time to explore visually or through touch.
- Give students who are deaf-blind extra-time to finish tests. Provide exams in the appropriate media. Adjust homework assignments to reduce stress.
- Keep items in permanent locations. For example, if a bookcase is moved frequently, this could create problems for a student with deaf-blindness.
- Talk with the TVI about modifying graphs, charts, and other visual materials. Try to avoid unnecessary handouts.
- Teachers should not be in front of windows during instruction. Make sure windows are behind the student with Usher syndrome.
- Understand assistive technology, such as low vision devices and digital recorders, may become necessary. The progressive nature of RP means a person with Usher syndrome will experience changes in sight. These technologies will improve access to the core curriculum and are permitted in the class if specified on the IEP.
- Use full-spectrum lighting in the classroom. Eliminate glare on whiteboards. Avoid using paper with reflective properties.
- Use high contrasting markers on the whiteboards. Fonts large enough for the student to read should be utilized on smart boards. Keep all instructional displays cleared of unnecessary marks, pictures, and diagrams.
Distinct Needs of Students who are Deaf-Blind
The presence of Usher Syndrome offers unique challenges compared to visual impairments and hearing disabilities alone. For instance, when no secondary disability is present, people with permanent vision loss tend to be sociable in spite of limited access to sight related aspects of the world. Reduced usefulness of the visual environment may emerge in time with individuals who have Usher syndrome because of RP. Eventually the retina alters, causing problems with eyesight in the disorder. This could mean the pace of signing may have to be adjusted as well as the need for Braille as a literacy medium.
Furthermore, if educational interventions are absent, people who are deaf are generally more uncooperative and aggressive than hearing peers. Without instructional services, language skills suffer, from spoken words to reading for people who are deaf. Unless deliberate teaching occurs, these issues may be present with a student who is deaf-blind. Since all of these groups may function below grade level and endure varying degrees of isolation, early involvement of special education professionals is critical for success in the long-run.
Independence and Usher Syndrome
In summary, following recommendations on the Individualized Education Program (IEP) is crucial for teachers and students with sensory disabilities. Findings from the Learning Media Assessment (LMA), Technology Assessment, and O&M evaluation should be implemented as soon as possible. With appropriate instruction, people who have Usher syndrome can attend universities and receive degrees.
Indeed, people with Usher syndrome can become contributing citizens. They are employed throughout the work force, enjoying recreational activities while staying active in their communities. However, parental involvement enhances the likelihood of goal achievement. Connecting with families who have a child who is deaf-blind can increase support and establish more contacts for the future.
This content is accurate and true to the best of the author’s knowledge and is not meant to substitute for formal and individualized advice from a qualified professional.