Usher’s Syndrome - A guide to Hearing Loss

This is a genetic condition which affects hearing, balance and sight which can result in permanent deafness and blindness.

Hearing and vision decline over time but it is unusual for someone to experience both of these from birth. An early diagnosis of this condition is usually correct.

Loss of vision occurs as a result of retinitis pigmentosa which is a degenerative disease of the retina, which affects its ability to transmit images to the brain and leads to sight loss.

There are three types of this syndrome which are:

• Type 1: the child is completely deaf from birth and has problems with balance. Vision starts to be affected by around the age of 10 or so and results in total blindness before adolescence. The only way the child can communicate is by sign language.
• Type 2: the child has impaired hearing (moderate to sever hearing loss) but their balance is normal. Vision doesn’t become affected until later on, usually at the end of adolescence. Sight loss is slower compared to Type 1. Lip reading, speech and hearing aids can help.
• Type 3: the child is born with normal hearing and vision but these become affected during adolescence. People with type 3 are blind and deaf by the time they reach middle age.

Types 1 and 2 are the most common forms of Usher’s syndrome.

Causes of Usher’s syndrome

This is an inherited condition which is passed down the family. A child will only have Usher’s syndrome if both parents carry this gene. Basically it is caused by ‘faulty’ genes: the genes which are responsible for the function of the retina and the workings of the cochlea have been found to cause the problem.

Treatment for Usher’s syndrome

Unfortunately there is no cure for this condition at present. It is therefore a case of managing the condition and learning to live as normal a life as possible.

Children with this condition need to be told as soon as possible so that they can be helped to prepare for the outcome of this condition and how to deal with it. This may seem harsh but the earlier the child is told the sooner the parents can help the child to prepare for the future. This means learning new skills and new ways to communicate.

Hearing aids, sign language, lip-reading and cochlear implants are all good ways of helping the child to communicate with others and navigate in today’s world. There are also a wide range of assistive technologies which also help with communication, for example, screen readers and voice activated software.

Children tend to adapt better to adversity than adults and cope with this condition quite well.