Usher's syndrome is a genetic condition that is characterized by hearing loss and progressive blindness from retinitis pigmentosa. This article reports on a study of the impairments and demographic characteristics of 60 Louisiana students with Usher's syndrome that were analyzed to identify their interrelationships. The research found that although a substantial majority of the students had a manifested visual impairment and profound hearing loss, some of the students had less-than-profound hearing loss and no manifested visual impairment—a finding that corroborated the findings of previous studies. Moreover, the severity of these two impairments seems to be interrelated.
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References
1.
BoughmanJ.A., VernonM., and ShaverK.A. (1983). Usher's syndrome: Definition and estimate of prevalence from two high-risk populations.Journal of Chronic Diseases, 36, 595–603.
2.
DavenportS.L.H., and OmenG.S. (1977). The heterogeneity of Usher's syndrome. In LittlefieldJ.W., EblingF.J.G., HendersonI.W. (Eds.), Fifth international conference on birth defects (pp. 87–88). Amsterdam, the Netherlands: Excerpta Medica.
3.
DavisH. (1965). Guide for the classification and evaluation of hearing handicap in relation to the international audiometric zero.Transactions of the American Academy of Ophthalmology and Otolaryngology, 69, 740–751.
4.
FishmanG.A., KumarA., JosephM.E., TorokN., and AndersonR.J. (1983). Usher's syndrome: Ophthalmic and neuro-otologic findings suggesting genetic heterogeneity.Archives of Ophthalmology, 101, 1367–74.
5.
ForsiusH., ErikksonA., NuutilaA., Vainio-MattilaB., and KrauseU. (1971). A genetic study of three rare retinal disorders: Dystrophia retinae dyascusis syndrome, X-chromosomal retinoschisis and grouped pigments of the retina.Birth defects original articles series, 7, 83–98.
6.
HallgrenB. (1959). Retinitis pigmentosa combined with congenital deafness; with vestibulo-cerebellar ataxia, a mental abnormality in a proportion of cases: A clinical and genetico-statistical study.Acta Psychiatrica et Neurologica Scandinavica, 34(Suppl. 138), 5–101.
7.
KarchmerM.A. (1985). A demographic perspective. In CherowV., MatkinN.D., TrybusR.J. (Eds.), Hearing impaired children and youth with developmental disabilities: An interdisciplinary foundation for service (pp. 36–56). Washington, D.C.: Gallaudet College Press.
KumarA., FishmanG., and TomokN. (1984). Vestibular and auditory function in Usher's syndrome.Annals of Otology, Rhinology & Laryngology, 93, 600–608.
10.
MerinS., AbrahamF.A., and AuerbachE. (1974). Usher's and Hallgren's syndromes.Acta Geneticae Medicae et Gemellologiae (Roma), 23, 49–55.
11.
NuutilaA. (1970). Dystrophia retinae pigmentosa-dysacusis syndrome (DRD): A study of the Usher or Hallgren syndrome.Journal de Genetique Humaine, 18, 57–88.
12.
RawlingsB.W. (1983). Final report: Louisiana statewide assessment and data management system for bearing impaired, visually impaired and deaf-blind students.Washington, D.C.: Gallaudet Research Institute, Center for Assessment and Demographic Studies, Gallaudet College.
13.
RiesP.W. (1985). The demography of hearing loss. In OrlansH. (Ed.), Adjustment to bearing loss in adulthood (pp. 3–21). San Diego, CA: College Hill Press.
14.
SingerJ.D., TomberlinT.J., SmithJ.M., and SchrierA.J. (1982). Analysis of noiserelated auditory and associated health problems in the U.S. adult population (1971-1975).U.S. Department of Commerce, National Technical Information Service.
15.
VernonM. (1969). Usher's syndrome-deafness and progressive blindness. Clinical cases, prevention, theory and literature survey.Journal of Chronic Diseases, 22, 133–151.
