Changing Vision, Changing Hearing: Is It Usher Syndrome?
“She wears hearing aids, but her hearing seems to be getting worse. Lately, she gets headaches when she reads. She doesn’t like to go out when it’s dark. Are these things related?”
“He’s deaf, but does that explain his balance problems? He runs into doorways, and kids tease him because he can’t catch a ball.”
Often, before there is a diagnosis, there are signs of change. Approximately 25,000 people in the United States have some form of Usher syndrome (click on the “More Info” tab to read more about Types I, II and III).
Usher Syndrome is a genetic disorder which includes a hearing loss at birth and a gradually developing vision impairment. The vision impairment is known as retinitis pigmentosa (RP), and it can eventually lead to the loss of vision. Some individuals also have balance issues.
RP causes tunnel vision or reduced field of vision.
RP is different for each person. No one can predict how fast a person’s vision will change.
Diagnosis: https://www.nidcd.nih.gov/health/usher-syndrome
Emotional adjustment is individual. Usher syndrome can be overwhelming; you may think you have the best way to help, but the person with Usher needs to learn, accept and adjust in his own way. Learning what works (e.g. a brighter lamp, large print and/or braille lessons, dark glasses outside, an FM system, an interpreter who knows tactile sign) is a highly individualized, coordinated process with the child, family and educational team. Learning to recognize the need for a new tool and/or strategy and learning to use these will happen gradually.
What can you do? Learn more about people who grew up with Usher syndrome. Meet adults with Usher and introduce them to the child or young adult. Keep an open mind and respect for the child as a child (who may not want to use a cane any more than you do.) Introduce gently, kindly and in different settings. Be willing to try it yourself. It takes a team, including adults with Usher, an education specialist who knows about the combination of hearing and vision loss (deaf-blindness), and educators who recognize that communication/language needs to be a primary focus.
Connecting through the Usher Syndrome Coalition:
- Join a private online forum, the USH Blue Book, where you can share questions, experiences and support with USH “neighbors” worldwide;
- Find out about USH Ambassadors, volunteers who are a connection to a local USH community. They support Facebook groups, like Awesome Moms with USH, and Young Adults Living with Usher Syndrome.
Marissa
Interpreter or Intervener
Audrey
More Cautious
Why Lighting Matters
Hearing Issues
Learning Braille and Tools for Vision
Using a Cane at School
- One Deafblind Woman’s Approach to Life by Amelia WesterfieldThis is an article in the Oregon Deafblind Project newsletter (pp.5-7) that describes one young woman’s journey with Usher II. www.oregondb.org/news/Winter-Spring2010.pdf
- Counseling Students Who Have Usher Syndrome: Teacher Tipsheet Compiled by Patricia Lago-Avery, retired counselor/professor from the National Technical Institute for the Deaf, RIT, Rochester, New York.http://www.netac.rit.edu/publication/tipsheet/ushers1.html
- Fact Sheet: Usher syndrome. Who is affected? What causes it? How is it diagnosed? Latest research findings and treatments, genetic testing.U.S. Dept of Health and Human Services, National Institutes of Health, National Institutes on Deafness and Other Communication Disorders, Feb. 2008 View Article (PDF)
- Educational Accommodations to Consider for Children, Teens, and College Students with Usher Syndrome Tips and strategies about accommodations in school settings. A “Learning Media Assessment” and other specialized assessments can help determine accommodations for an individual student. Ask the student what he or she thinks would be helpful and acceptable, and involve family members to get their input. View Article
Children with Type I Usher syndrome are born profoundly deaf and have balance issues that can have an impact on the child’s development. Communication and language are of primary importance, and parents can gather information from a variety of sources including: early support specialists who work with children who are deaf/hard of hearing, parents and “Guide by Your Side” programs, adults who are deaf/hard of hearing, and hearing health professionals. Early support services should be unbiased and consider the individual child and family’s needs, so that the child can take advantage of the unique window of time during which the brain is most receptive to learning a first language, whether spoken, signed or both.
If a child is diagnosed with Type I Usher syndrome early on, before he loses peripheral vision, the child is more likely to benefit from the full spectrum of support that can help him have a more active life.
Because of the balance problems associated with Type I Usher syndrome, children may take longer to learn to sit without support and may not walk before they are 18 months old. These children usually begin to develop vision problems in early childhood, almost always by the time they reach age ten. Vision problems most often begin with difficulty seeing at night, and progress at varied rates until the person has tunnel vision. (Adapted from “Fact Sheet on Usher Syndrome,” NIDCD article, listed in links.)
There are people with Usher Type I who are fluent in two or more languages, are leaders in their communities and live active lives. According to Patricia Lago-Avery, retired counselor from NTID*, people with Usher syndrome can be found working as: author, researcher, professor, chef/owner of restaurant, lawyer, certified public accountant, information technology specialist, computer programmer, public health manager, counselor, minister, librarian, veterinarian, health planner, clerk, medical transcriptionist, mental health counselor, and high school teacher.
Parents and professionals can help children with Usher syndrome connect with adults who have Usher syndrome in a variety of settings. There are things only another person who is living with vision changes can understand… about adjusting, and the tools and services that can increase a person�s independence. All children are children first, and developing self-awareness takes time. Connections between children and mentors can have a very positive impact, if the relationships are nurtured over time, at gatherings that include age-appropriate activities for kids and families to interact with adults who have Usher (e.g. shared meals, tandem bikes, cane races for blindfolded parents, chat time in small groups, skilled interpreters).
*NTID: National Technical Institute for the Deaf
See videos of Marissa
See video of Patrick Cave, Deaf-Blind Etiquette
Children with Type II Usher syndrome are born with moderate to severe hearing loss and typical balance. Although the severity of hearing loss varies, many of these children tend to use hearing aids and communicate orally. The vision problems in Type II Usher syndrome tend to progress more slowly than those in Type I, with the onset of RP often not apparent until the teens. (Excerpt from “Fact Sheet on Usher Syndrome,” U.S. Dept. of Health and Human Services, National Institute of Health, NIDCD)
“My name is Audrey and I’ve had a moderate-to-severe hearing loss since birth. I found out I had a vision impairment when I was seventeen. I’ve had hearing aids since I was two and half and used a FM system throughout elementary school and part of middle school. I wear my hearing aids everyday and all the time; it doesn’t matter what I’m doing.
“I drifted away from using the FM system in middle school because I found that I had to instruct each teacher how to use it, and it was hard to carry my school supplies and the system… and a little bit had to deal with not liking to use it. In high school I did not use it at all. I just made sure I was in the front of the classroom and asked teachers to try their best to not talk with their backs to me. Background noise is just hard.”
(Chairs scraping, people laughing, machinery, radio or TV nearby, all interfere with understanding speech.)
“In my senior year I was playing sofball and I started noticing that I couldn’t find the ball. I had my eyes tested and that’s when I got the diagnosis. I had to quit driving, and quit playing ball.”
“Looking back on those days, I think maybe trying harder at finding a way to make the FM system work in different settings would have been more beneficial. I know that I missed a lot of what was being said and what the students read or asked. I made it through high school and college not using it, but it wouldn’t have hurt to use the FM during those years.”
“When you’re young, you don’t know what normal eye sight is. I think my peripheral vision was gone sooner than I thought, but I didn’t realize it. I thought it was normal eyesight. You don’t really register. As far as parents helping their kids, what they need is going to change probably every year that a child has RP. In the classroom I needed to sit close to hear, but later I had to sit farther back because of the RP. And at the movies, I have to sit farther back. So each time your vision changes, you adjust.”
Audrey enjoys hiking and surfing.
| Type 1 | Type 2 | Type 3 | |
|---|---|---|---|
| Hearing | Profound deafness in both ears from birth | Moderate to severe hearing loss from birth | Normal at birth; progressive loss in childhood or early teens |
| Vision | Decreased night vision before age 10 | Decreased night vision begins in late childhood or teens | Varies in severity; night vision problems often begin in teens |
| Vestibular function (balance) | Balance problems from birth | Normal | Normal to near-normal, chance of later problems |
¿Es esto Síndrome de Usher?
“Ella usa audífonos, pero su audición parece empeorar. Últimamente ella padece dolor de cabeza cuando lee. A ella no le gusta salir cuando está oscuro. ¿Tiene esto alguna relación?
“Él es una persona sorda, ¿explica esto sus problemas de equilibrio? El prefiere no salir y los niños se burlan porque no puede atrapar la pelota.
Con frecuencia, antes de que haya un diagnóstico, se presentan algunos cambios. Aproximadamente 25.000 personas en Estados Unidos, tienen algún tipo de Síndrome de Usher (Tipos I, II y III).
El Síndrome de Usher es un desorden genético que incluye, pérdida de audición al nacer y el desarrollo gradual de un impedimento visual. Este impedimento visual se conoce como Retinitis Pigmentosa (RP) y eventualmente puede conducir a la pérdida de la visión. Algunas personas también presentan problemas de equilibrio.
La RP produce Visión de Túnel o campo reducido de visión.
La RP se presenta de forma diferente en cada persona. Nadie puede predecir la rapidez con la que puede cambiar la visión de una persona.
La adaptación emocional es individual. El Síndrome de Usher puede ser abrumador; usted puede pensar que conoce la mejor manera de ayudar pero la persona con Usher necesita aprender, aceptar y ajustarse a su manera. Aprender qué es lo que funciona (Ej. una lámpara más brillante, letra más grande y/o lecciones de Braille, lentes oscuros al salir, un sistema FM, un intérprete que conozca las señas táctiles) es algo totalmente personalizado, es un proceso coordinado con el niño, la familia y el equipo educativo. Aprender a reconocer la necesidad de una nueva herramienta y/o estrategia y aprender a utilizarlos es algo que sucede gradualmente.
¿Qué puede usted hacer? Aprender más sobre las personas que crecen con Síndrome de Usher. Conocer adultos con Usher y presentárselo a los niños o jóvenes. Tener una mente abierta y respeto por los niños como personas (que, al igual que usted, no quieren tener que usar un bastón). Presentarse de forma gentil, amable y en diferentes entornos. Estar dispuesto probarlo usted mismo. Se necesita un equipo en el que haya adultos con Usher, especialistas en educación que conozcan sobre la pérdida de visión y audición combinada (sordo-ceguera) y educadores que comprendan que la comunicación y el lenguaje deben ser el objetivo principal.