Usher syndrome is a hereditary cause of deafness, blindness, and (sometimes) disequilibrium, with symptoms often beginning in childhood. There are several types of Usher syndrome, each with somewhat different features. The diagnosis may be suspected in someone with young and/or progressive bilateral visual impairment and bilateral hearing loss. Testing of hearing, vision, and balance may further raise suspicion for the diagnosis. Confirmation of the diagnosis through genetic testing is usually offered. Depending on the type of Usher syndrome, the hearing loss may be treated with a hearing aid or cochlear implantation. Patients with disequilibrium may consider a trial of vestibular rehabilitation therapy.