Usher syndrome is named after Charles Usher, a British ophthalmologist who described the nature of the disease in 1914. This article is aimed at increasing audiologists' understanding of the audiologic and visual presentation, diagnostic criteria, and intervention strategies involved with Usher syndrome. Therefore, audiologists are in a position to improve differential diagnosis outcomes for children with Usher syndrome. Because of newborn hearing screening, audiologists are often the family's primary contact. So, while parents learn of their child's hearing loss relatively early, without a differential diagnosis they proceed with critical decisions related to intervention, communication, and educational options unaware that their child will eventually be blind. Newborn hearing screening has reduced the age of identification of children with hearing loss from 12–18 months to 6 months or less (Harrison & Rousch, 1996), but the diagnosis of Usher syndrome, with its devastating vision loss, typically lags 5–10 years behind the identification of the hearing loss (Kimberling & Lindenmuth, 2007). Although considered a rare disease, it is the most frequent cause of deaf-blindness in humans.
Usher syndrome is a genetic condition involving sensorineural hearing loss and retinitis pigmentosa (RP).
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