Usher syndrome has two primary characteristics; inner ear abnormalities and the deterioration of the retina’s light sensing cells. Both conditions result in a loss of hearing and vision, varying in degree from early adolescent and worsening over time as we mature.

The principal cause of Usher syndrome is genetics. The core cause of Usher syndrome is an autosomal recessive disorder, involving two types of genes. One is mutated (recessive) and one is normal (dominant). As an offspring susceptible to Usher syndrome, you inherit one copy of each gene from your parents.

In rare cases, the offspring inherits two copies of the mutated gene, gaining dominance over the normal gene increasing the risks of Usher syndrome. In both cases, one or both parents may not show any symptoms of the disease, but they still carry the gene and pass it on to the next generation. The strength or dilution of the identified mutated gene is responsible for the syndrome’s severity and the rate of hearing and vision deterioration.