Usher Syndrome Team
Usher syndrome is a genetic disorder that involves hearing loss, from mild to profound, and the progressive loss of vision due to retinitis pigmentosa (RP), a degeneration of the retina of the eyes. In addition, depending on the type of Usher syndrome, it may affect a person’s sense of balance.
The main purpose of the Usher Syndrome Team is to screen children and youth for visual acuity difficulties who are hearing impaired. Screening for Usher syndrome is important for several reasons:
• Early identification impacts medical decisions, educational decisions/services and social implications for the student as well as the family members.
• According to research, there is an under-identification of children with Usher syndrome both in the state of Nebraska and in the nation.
• New research is being developed daily for the treatment of Usher syndrome, especially in the area of Vitamin A supplements.
Students with Usher syndrome typically exhibit behaviors, which may be misinterpreted as clumsiness, slowness or uncooperativeness. Identification of Usher syndrome in a student prevents this type of misinterpretation.
• A diagnosis of Usher syndrome allows parents and children to consider genetic counseling and testing for other children in the family.
• The student can receive counseling and support to prepare him or her for the future.
Among children who are born profoundly deaf, three to six percent can be expected to have Usher syndrome, Type 1. Among children born hard of hearing, Usher syndrome, Type 2, probably occurs at the same rate. Vision screenings for retinitis pigmentosa are not routinely performed in the school setting, resulting in an under-identification of students with Usher syndrome.
To learn more about Usher Syndrome, please visit the page on K12 Academics.
For additional information, please contact one of the team members listed above or Teresa Coonts, Nebraska Project for Children and Youth With Deaf-Blindness, at (402) 595-1810.