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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://www.dblink.org/lib/topics/usher.htm
Copies are available in our Resource Libarary.
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