Named after Scottish ophthalmologist Charles Usher, Usher Syndrome causes deafblindedness. This is a rare genetically acquired disease that not only causes hearing loss but also balance problems and blindness. In the United States of America, 4 in every 100,000 children are born with this disorder.
Usher syndrome is an inherited disease which means that it is passed on to a child by its parents. Normally, parents with normal vision and hearing abilities are not aware that they are carrying mutated or altered genes which might cause their child to suffer from usher syndrome. There is a one in four chance that child of parents with mutated genes will suffer from usher syndrome. Scientists are working on finding out if it is possible to determine if a person is carrying mutated genes. Until now there is no way to determine that.
Types of Usher Syndrome-
There are 3 types of Usher Syndrome with different levels of severity.
Usher Syndrome Type 1- Infants born with Type 1 disorder are profoundly deaf at the time birth. They face severe balance problems. They find it difficult to sit on their own until the age of 1.5 years of age. They cannot walk independently either. They are also more likely to develop vision problems until the age of 10. Early intervention can help them get benefited from intervention strategies before their vision is completely lost.
Usher Syndrome Type 2- The second type of usher syndrome is different from the first one. Infants born with this hearing loss can take advantage of hearing devices. Vision problems develop slower than type one. The severity of hearing loss varies from moderate to severe. Children with this disorder do not suffer from balance problems like usher syndrome type 1. Hearing aids can help in reducing the effects of hearing loss.
Usher Syndrome Type 3- Infants born with usher syndrome type 3 have normal hearing ability at birth. There are no early signs of reduced hearing or vision with this disorder, but start losing hearing by teenage. Night blindedness strikes at the age of puberty and the person loses his sight by mid adulthood.
Diagnosis of Usher Syndrome-
Early diagnosis of Usher syndrome is very important as educational programs can help the child to handle the loss of hearing and vision.
Is there any Cure for Usher Syndrome-
Unfortunately, there is no cure for Usher syndrome at present, but as advised above, educational programs help in managing the loss.
Adnan Hafiz runs Global Hearing, a hearing clinic in melbourne that offers hearing loss treatment. We also provides free hearing test to patients in Melbourne.