Usher syndrome—the most common reason for deaf-blindness

Abstract

Deaf-blindness is a serious disability, which affects all aspects of life. The most common condition combining deafness and blindness is the Usher syndrome (USH), which is traditionally divided into three major clinical subtypes, including Usher syndrome type 1, Usher syndrome type 2, and Usher syndrome type 3. The frequency of USH occurrence is often undervalued. To date, five genes for USH1 have been identified, three genes are involved in USH2, and one gene in USH3. In summary, USH can be a multisensory impairment associated with hearing impairment, progressive retinal degeneration, and vestibular dysfunction. Management of these patients requires interdisciplinary approach and collaboration.