The Usher's Syndrome Adolescent: Programming Implications for School Administrators, Teachers, and Residential Advisors

The Usher's Syndrome Adolescent: Programming Implications for School Administrators, Teachers, and Residential Advisors Wanda M. Hicks and Doin E. Hicks OVERVIEW Usher's Syndrome, first described in 1858, is a condition of congenital deafness accompanied by progressive loss of vision through retinitis pigmentosa. In childhood, the syndrome is characterized by poor adaptation to darkness (night blindness), progressing to limited peripheral vision that usually becomes increasingly evident by the teenage years. During the middle-aged or later years, there is degeneration of central vision. It is difficult, however, to generalize about the disease since the rate of visual deterioration varies considerably from individual to individual, and there is a wide range of concomitant handicaps. The incidence data on Usher's Syndrome are somewhat sketchy and thus open to conjecture. Available estimates indicate the disease occurs in approximately 3 per 100,000 in the general population (Kloepfer, Laguaite, & McLaurin, 1966). This may seem like a small number, placing the disease almost in the rare category. But when applied to the deaf population, and particularly to those persons who are congenitally deaf, the data are much more meaningful and significant. Regarding those persons who were born deaf, the literature has variously reported the incidence of Usher's Syndrome at 4% (Nance, 1971, 1973) and 3-6% (Vernon, 1973). Bergsma Wanda Hicks is a specialist with the Office of Special Services, Division of Pre-College Programs at Gallaudet College in Washington, D.C. Doin Hicks is the Vice President for Research, Division of Research at Gallaudet College. (1973) reported, however, that the incidence of Usher's Syndrome among the deaf population may be substantially higher than previous estimates since the symptoms of retinitis pigmentosa may not be present or detected until well after the student has started school. Nevertheless , Vernon (1969) estimates that 16,000 persons in the United States suffer from Usher's Syndrome. Recent estimates from the Office of Special Education, Department of Education, support Vernon's estimate by indicating there are 3,000 to 5,000 severely vision and hearingimpaired school-aged individuals in the United States. Although this is a relatively small segment of the total school-aged handicapped population (estimated at 7,000,000), it is a group that requires and deserves extensive assistance in order to achieve its potential. Vernon (1974) puts the situation in perspective by stating, "It is almost unbelievable that a condition which causes 3% to 6% of deaf children to become blind is still relatively ignored by professionals working with deaf adults and children." To dramatize further the significance of Usher's Syndrome, Vernon (1969) reported that half of all deaf-blindness was due to this disease. USHER'S SYNDROME: SYMPTOMATOLOGY AND STAGES When inquiring about the condition of Usher's Syndrome, educators usually ask questions relating to symptoms of the condition, its stages, and, most importantly, programmatic considerations. Doin Hicks (1978) has provided a comprehensive outline and discussion of the 422 A.A.D. !June 1981 The Usher's Syndrome Adolescent characteristics of the condition that should be taken into consideration for programmatic planning. He summarizes the five stages as: (a) Stage 1—"Awareness Stage" (chronological ages of 6-12); (b) Stage!—"General Counseling Stage" (chronological ages of 13-20); (c) Stage 3—"General Planning and Community Resource Identification Stage" (early adult years); (d) Stage 4—"Specific Planning and Adjustment Counseling Stage" (middle adult years; and (e) Stage 5—"Adjustment Stage" (late adult years or sooner). In the same article, Hicks points out that careful , comprehensive, and well-coordinated programming should be undertaken from the point of initial diagnosis through the late adult years. He recommends professionals be forthright and candid with the person having the condition. Even in the first stage, the initial symptoms of the disease should be described to the individual and he or she should be alerted to compensatory measures that should be taken to cope with the night vision problems. Even at this early age, the family should be provided with supportive counseling. Genetic counseling should be included in such a program. As the loss of night vision becomes compounded with loss of peripheral vision during the early adolescent years, career counseling should be implemented. Additional genetic...