Usher Syndrome III

The frequency of Usher syndrome type III is highest in the Finnish population, but it has been noted rarely in a few other ethnic groups.

Mutations in the USH3A gene cause Usher syndrome type III. Usher syndrome type III can be caused by mutations in one of at least two genes, only one of which (USH3A) has been identified. The USH3A gene makes a protein that is important for the development and maintenance of the inner ear and retina. The protein's function in these structures, and its role in hearing and vision loss, have not yet been fully explained.