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USHER SYNDROME EDUCATION

Usher Syndrome is a genetic condition in which children are born deaf (USH1) or hard of hearing (USH2) and become DeafBlind due to RP (retinitis pigmentosa). The impact on education of is both subtle and profound.

Students with USH1 are found in programs for the deaf and have inner ear balance problems (vestibular loss) and show signs of RP in early to mid childhood.  Students with USH2 are hard of hearing,are usually in mainstream education or in programs for the hearing impaired.  They do not have vestibular loss and lose their peripheral vision somewhat later than those with USH1.  Therefore, they may graduate before the diagnosis is made.

Intelligence: The majority of people with Usher Syndrome have average or above-average intelligence. Infants and toddlers with USH1 present with delayed language because of deafness, which is often not diagnosed early because they are usually the first children in the family with a hearing loss.  They also have delayed motor skills so they do not crawl and walk on time.  Because they are not speaking and moving the way most infants do, they are labeled developmentally delayed or even mentally retarded. Psychologists, neurologists, and pediatricians often are not aware of the effects of vestibular loss on development.  For more information about vestibular loss, click on vestibular loss. Even in teenage years, psychological testing may greatly underestimate intelligence.  If the psychologist does not understand what the student can and cannot see and does not take the time to make sure the student sees all the instructions, the test results can be very misleading.

Poor classroom performance: Frequently students in both groups do not do well in school despite having normal to superior intelligence because they have problems following classroom discussions.  Students function fairly well in deaf or hard of hearing settings until their vision deteriorates due to RP. Then they miss a lot of information that comes from the peripheral vision and will have a very hard time being aware of the teacher's instructions, following a classroom discussion, or following any signed or spoken narrative when overheads, filmstrips or videos are being shown.  For more information about the impact of vision loss click on RP.

Social and emotional consequences of being deaf with RP can be profound. Please review the manual Usher Syndrome in the School Setting written by a social worker and O&M instructor who are both fluent in ASL
and have worked extensively with adults who have Usher Syndrome.
http://nationaldb.org/ISSelectedTopics.php?topicID=1257&topicCutID=13Open in new window. Link to a different web site.

Copies are available in our Resource Libarary.

Helpful web site - DB-LINKOpen in new window. Link to a different web site.

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