Per the NIH: Usher syndrome type 2A is a genetic condition characterized by hearing loss from birth and progressive vision loss that begins in adolescence or adulthood. Vision loss is due to retinitis pigmentosa. Night vision loss begins first, followed by blind spots that develop in the side (peripheral) vision. Over time, these blind spots enlarge and merge to produce tunnel vision. (See nih.gov for more). .
Congential syndrome. It is a syndrome commonly associated with sensorineural hearing loss and retinitis pigementosa. It can account for up to 50% of patients with both deafness and blindness. There are 3 types of usher syndromes (1b, 2a, 3) and each has a specific but related genetic mutation. Usher type 2a (ush2a) is the most common mutation.
Vision/hearing loss. Ushers syndrome is an autosomal recessive inherited disease that involves various degrees of vision loss in the form of retinitis pigmentosa & varying degrees of hearing/balance loss. Type 1 has profound hearing/balance loss at birth, type 2 has moderate to severe hearing/balance loss at birth,& type 3 normal hearing/balance at birth but progresses in later childhood/teens. . Read more...