Usher Syndromes

Vitamins & supplements   Genetic disorders   Types   Causes   Self help summary   News

Genetic Disorders

The Usher syndromes (USH) comprise genetically-related disorders that bring about simultaneous hearing loss and vision loss (resulting from an eye condition called retinitis pigmentosa). Retinitis pigmentosa is the loss of night and peripheral vision, resulting in tunnel vision. About 3-6% of deaf children have Usher.

Self Help & Tips

  • Usher Syndrome Vitamins Find Vitamins & Supplements to support the retina and photoreceptors.

  • Recommended Homeopathic remedy for maculaHomeopathic Macular Degeneration Pellets Helpful for retinal support.

  • Nutritional support.
  • Early Communication Skills. In the case of type 1 Usher, work with your child's doctor to determine the best communication method as soon as possible. This is important because in very early life your child's brain is very receptive to learning language skills. If the child begins this process well before sight is lost, he/she will have the best chance of living a normal life.

  • Management. To help manage and possibly slow the progress of the condition, these vision support tips are important.

  • See more recommendations.

Types of Usher

There are three types of Usher with similar symptoms. Different genes have been identified for each type.

  • Type 1 Usher. At birth, the child is severely deaf with severe balancing problems. Before age 10 years old, the child may begin to develop vision problems. This genetic disorder has been identified frequently in today's Ashkenazi Jewish population.
  • Type 2 Usher. At birth the child has moderate to serious deafness and normal capacity to balance. These children are assisted by a hearing aids. Retinitis pigmentosa isn't noticed until they are in their teens.
  • Type 3 Usher. These children do not have hearing problems at birth and they have normal or almost normal balance. However hearing and vision deteriorate with time, usually by the teen years. Balance difficulties might develop later on. Hearing aids are usually needed by adulthood, and night blindness may start in early teens. Blind spots can develop and the person is often legally blind by mid-adulthood. This 3rd type is prevalent in Finland.

Causes

All types of retinitis pigmentosa-related disorders are genetically based. The trait is recessive meaning that a gene must come from both parents. If someone has only one of the genes, they have the capacity to pass the trait on to a child but do not themselves exhibit any symptoms of the condition.

Some specific genes have been identified, but there may be more.

  • Type 1 involves the genes: CDH23, MY07A, PCDH15, SANS, USH1C
  • Type 2 involves: USH2A, VLGR1, WHRN
  • Type 3 involves: USH3A

Conventional Treatment

There is no treatment available to date. It is important to diagnose the condition early so that early childhood communication skills may be developed. There is some future possibility of artificial retina implantation, and development of retina cells from stem cells.

Complementary Treatment

Photoreceptor News

Takes a moment to load ...

Usher Syndrome Vitamins Find Vitamins & Supplements to support the retina & photoreceptors.

Research

See research on retinitis pigmentosa.

Related Conditions