Usher Syndrome Essay

950 Words4 Pages
Usher Syndrome The ability to see and hear is amazing but often taken for granted. According to the National Institute of Health, 3-6% of children who are deaf and 3-6% of children who are hard of hearing are affected with Usher Syndrome. It is estimated that 4 babies in every 100,000 births have Usher Syndrome. Usher syndrome (USH) is responsible for 50% of all monogenic deaf-blindness cases. (Bonnet et al). USH is an autosomal recessive disorder, with 2 major symptoms: sensorineural hearing impairment and Retinitis pigmentosa (R.P). R.P is an eye disorder that causes loss of peripheral vision and night blindness because the retina degenerates over time (Vache, C., et al). Types of Usher Syndrome: There are 3 types of Usher syndrome, type 1, type 2, and type 3. Type 1 is the most severe form. Children with type 1 Usher syndrome are born deaf at birth and have major balance problems. Children with type 1 Usher syndrome usually lose their vision by age 10. Type 2 Usher syndrome characteristics include moderate to severe hearing loss at birth but normal balance. Depending on the severity of hearing loss most children with type 2 can benefit from hearing aids and communicate verbally. The onset of retinitis pigmentosa often appears in the teenage years. The National Health Institute reports that Type 1 and 2 are the most common in the United States. In Type 3 Usher Syndrome the children are born with normal hearing and as the children reach puberty hearing loss begins and night vision problems increase. Children with type 3 Usher Syndrome have normal or close to normal balance but chance of balance problems increases with age. why its important: As with any disability the earlier the syndrome is diagnosed the more beneficial a treatment plan can be for the patient. It is critical to be able to diagnosis infants with Usher syndrome as early as

More about Usher Syndrome Essay

Open Document