Prognosis

Children with type I Usher syndrome usually obtain little or no benefit from hearing aids. Unless fitted with a cochlear implant, they do not typically develop speech. Due to the progressive visual loss, communication by sign language and lip reading becomes increasingly difficult over time, thus tactile signing remains a communication option for these patients. Most of the children with type 2 Usher syndrome can benefit from hearing aids. They often communicate through speech assisted by the use of lip-reading and hearing. Individuals with Usher syndrome 3 usually require hearing aids by mid- to late adulthood.

Generally, progressive and permanent loss of visual acuity and visual field became substantial between the second and third decades of life in patients with Usher syndrome, though total vision loss before the age of 60 years is uncommon. This is why visual prognosis is an important issue when counseling Usher syndrome patients and their families.