Usher Syndrome

People with Ushers do not need people to think, ‘How terrible, this person is deaf and blind; there is nothing to do.’ What we need is for people to say, ‘This person is deaf and blind. What can I do to facilitate his achievement of his full potential?’

Lawrence W. Lee, 43 year old Afro-American with Usher syndrome. First published Summer 1994, taken from


Usher Syndrome is inherited and presents in the individual having difficulties with both hearing and vision. It is a major cause of Deafblindness (see page 144). Symptoms of Usher syndrome include hearing impairment and retinitis pigmentosa (an eye disorder that causes one’s vision to deteriorate over time).

There are three types of Usher syndrome:

Type I: the student is profoundly deaf at birth and will have difficulties with balance. Typically, develops vision problems by the age of ten.

Type II: the student is born with moderate to severe hearing loss and has normal balance characteristics. Most perform well in school and some may benefit from hearing aids. Visual problems progress at a slower rate than Type I and blind spots can begin to appear when the student enters adolescence at which time the student may experience stress and embarrassment.

Type III: the student is born with normal hearing and balance. However, hearing and vision deteriorate over time, at a rate varying between individuals. Typically, students develop notable hearing problems by adolescence. Blind spots appear from late adolescence onwards and by midadulthood the individual may have a total loss of sight.