School of Medicine

Department of Genetics

Usher Syndrome

Danielle Mercer1, Fern Tsien2, and Barbara Gordon-Wendt1

1 Department of Communication Disorders, LSUHSC School of Allied Health Professions
2 Department of Genetics, LSUHSC School of Medicine


The incidence of Usher syndrome is about 3 in 100,000 people.  Usher syndrome is more common in certain ethnic populations, most notably the Acadian population in Louisiana and those of Ashkenazi Jewish ancestry.  The inheritance pattern is autosomal recessive.  The disorder causes both hearing loss and vision loss.  There are three types of Usher syndrome, with the most severe form causing profound sensorineural deafness at birth and progressive vision loss ultimately leading to blindness.  Balance can also be affected.  About half of all concurrent deafness and blindness in adults is thought to be due to Usher syndrome. 

For a more thorough review of Usher syndrome, please see the following links:


For information on support groups for Usher syndrome, visit the following links: