Children with Usher syndrome: mental and behavioral disorders

This article investigates the prevalence and characteristics of mental and behavioral disorders among 26 children, 3-17 years of age, with Usher syndrome.Six of the 26 children were diagnosed with a mental or behavioral disorder (1 with schizophrenia and mild mental retardation, 1 with atypical autism and severe mental retardation, 1 with atypical autism and mild mental retardation, 1 with mild mental retardation, and 2 with conduct disorder). Another 3 children had had a mental or behavioral disorder previously in their childhood.Even though vision impairment first manifests in late childhood, some children with Usher syndrome seem to develop mental and behavioral disorders during childhood. The aetiology and treatment of mental and behavioral disorders among children with Usher syndrome are discussed. Children with Usher syndrome and their parents may need clinical support during early childhood to prevent development of mental and behavioral disorders.Usher syndrome is characterized by deafness and a gradual loss of vision. The hearing loss is sensorineural, whereas the vision loss is associated with retinitis pigmentosa (RP), a degeneration of the retinal cells. Three subtypes of Usher syndrome have been found [1]. People with Usher I are congenitally deaf, and start to lose vision early in life. They also face balance difficulties due to vestibular system problems. Individuals with Usher II also experience hearing loss but are not profoundly deaf. They have no noticeable problems with their balance. Individuals with Usher syndrome III are not congenitally deaf, but gradually lose their sense of hearing and vision. Some individuals with Usher III experience balance difficulties while others do not [2,3]. Because of the balance difficulties many children with Usher take longer to learn to walk. Several genes have been associated with Usher syndrome. To date, seven genetic loci for Usher I (USH1B-H) have been mapped on chromosomes 14q32, 11q13.5, 11p15.1, 10q22.1,