By Susan Chow, PhD, ELS
Usher syndrome is characterized by loss of hearing and vision, as well as problems relating to balance and movement. There are three types of the syndrome, according to when the symptoms present and the severity of the symptoms.
People with type 1 Usher syndrome are born with significant symptoms that inhibit their function and learning from infancy.
They are almost completely deaf at birth and have stark balance problems that affect their mobility. They usually learn to sit without the need for support at a later age, commonly older than 1 year old. Likewise, the first steps and walking are often delayed to approximately 2 years old.
Loss of vision tends to present in early childhood and uniformly before the age of 10 years old. The initial signs are problems seeing in the nighttime and with peripheral vision, which soon progresses to total blindness.
Type 2 Usher syndrome also displays signs from birth, although the severity of the symptoms is usually not as profound as those affected by type 1.
Hearing loss may be moderate to severe at birth, but ability to maintain balance is usually normal. Abnormalities in vision begin to develop in teen years but typically progress more slowly than in individuals with type 1, such that most individuals do not ever completely lose their sight.
A baby with type 3 Usher syndrome does not usually exhibit major signs of the condition at birth. They have relatively normal hearing, vision and balance abilities, such that the condition may not be recognized until a later date.
Over time, there is a gradual loss of vision and hearing but the rate at which this occurs differs considerably between different children. However, a loss of hearing is usually evident when the child reaches their teen years and night vision also begins to present as an issue.
The decline in hearing and vision ability continues in life and most individuals are clinically blind when they reach middle age. Problems in balance may also arise, varying greatly in severity for each individual.
It is important that babies with Usher syndrome are identified and diagnosed as soon as possible, to enable them to begin learning techniques that will help them to cope with the changes associated with the loss of senses.
Children with type 1 Usher syndrome are almost completely deaf at birth and, with currently available medical treatments, will never regain their ability to hear. As such, it is beneficial for them and their families to begin learning sign language to help them communicate.
Whilst hearing aids do not offer a benefit to individuals with type 1, as they are completely deaf, children with type 2 or type 3 can improve their quality of life greatly with a hearing aid. This is because they have not completely lost their sense of sound so the amplifying effect of hearing aids can help.
Specialist health professionals trained in the learning development of children with Usher syndrome can aid in the support of parents and children affected by the syndrome. It can be a steep learning curve for everyone involved and it is essential that adequate support be given as symptoms develop or become more severe over time.
Last Updated: Jul 9, 2015