Changing Vision, Changing Hearing: Is It Usher Syndrome?

“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 (see 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: http://www.nidcd.nih.gov/health/hearing/usher.htm

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.

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