hereditary disease that affects hearing and vision and sometimes balance.
Usher syndrome is an inherited disorder that is characterized by moderate to profound hearing impairment, which is present at birth or shortly thereafter, and progressive vision loss due to retinitis pigmentosa (RP), a degeneration of the retina. It is the major cause of deaf blindness. About 10-15,000 people in the United States have Usher syndrome. Read More
A genetic syndrome with congenital nonprogressive scnsorineural (involving the inner ear or the auditory nerve) hearing loss of variable severity, retinitis pigmentosa (night blindness and progressively restricted visual field), and loss of olfactory (sense of smell) and vestibular (balance) functions. Neuropsychiatric difficulties occur in 25% of cases. Inheritance is autosomal recessive (rarely, X-linked) with an incidence of 3 in 100,000. The syndrome accounts for approximately 5% of children with severe hearing loss and half of all adults with deafness and blindness. Named for the Scottish ophthalmologist Charles Usher (1865-1942).
Inherited disease that causes severe hearing and vision impairments.
Inherited recessive genetic disorder characterized by sensorineural hearing impairment, present at birth or shortly thereafter, and a progressive loss of field due to retinitis pigmentosa. It accounts for more than 50% of all cases of deafblindness in the
Usher syndrome (sometimes referred to as "Usher's syndrome") is a genetic disease causing deaf-blindness. It is essentially progressive retinitis pigmentosa combined with congenital hearing impairment. It is inherited in an autosomal recessive pattern.