The association of hearing loss and retinitis pigmentosa has been generally recognized as Usher's Syndrome, although variations in the syndrome have not been clearly delineated. The diagnosis of a progressive visual disease in a person with severe hearing impairment has devastating implications for the individual's future. This article reviews findings of this syndrome and suggests strategies for dealing with some of the clinical problems displayed by Ushers Syndrome patients.
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References
1.
ArdenG. B., and FoxB.Increased incidence of abnormal nasal cilia in patients with retinitis pigmentosa.Nature, 1979, 279, 534–536.
2.
BatemanJ., RiednerE. D., LevinL. S., & MaumeneeI. H.Heterogeneity of retinal degeneration and hearing impairment syndromes.American Journal of Ophthalmology, 1980, 90, 755–767.
3.
BeattyC. W., McDonaldT. J., FeinsteinS., and DuvallA. J.Usher's Syndrome with unusual ontologic manifestations.Proceedings Mayo Clinic, 1979, 54, 543–546.
4.
BetticaL. J., KeaneG. E., BergmanM., EdgecombC. F., LotzJ., and GoldbergH. H.Communication—A key to service for deaf-blind men and women. A manual for professional workers.Brooklyn: The Industrial Home for the Blind, 1959, 1, 35–59.
5.
BoughmanJ. A., ConneallyP. M., and NanceW. E.Population genetic studies of retinitis pigmentosa.American Journal of Human Genetics, 1980, 32, 23–235.
6.
DavenportS. L., O'NuallainS., OmennG. S., and WilkusR. J.Usher syndrome in four hard-of-hearing siblings.Pediatrics, 1978, 63, 578–583.
7.
FishmanG., VasquezV., FishmanM., and BergerD.Visual loss and foveal lesions in Usher's syndrome.British Journal of Ophthalmology, 1979, 7, 484–488.
8.
GarciaC., KretzerF., and RedburyD.Ultrastructure and autoradiography of retinitis pigmentosa in Usher's syndrome.Investigative Ophthalmology and Visual Science, 1981, Supp. 20, 3, 411.
9.
HalgrenB.Retinitis pigmentosa combined with congenital deafness with vestibulo-cerebellar atazia and mental abnormality in a proportion of cases: A clinical and genetico-statistical study.Acta Psychiatrica Scandinavica, 1959, Supp. 34, 138.
10.
KongismarkB. W., and GorlinR. J.Genetic and Metabolic Deafness.Philadelphia: W. B. Saunders Co., 1976.
11.
McLeodA. C., McConnellF. E., SweeneyA., CooperM. C., and NanceI. E.Clinical Variation in Usher's syndrome.Archives of Otolaryngology, 1971, 94, 321–334.
12.
MerinS., AbrahamF.A., & AuerbachE.Usher's and Hallgren's syndromes.Acta Geneticae Medicae et Gemellologiae, 1974, 23, 49–55.
13.
RainerJ. D., and AltshulerK. Z.Comprehensive Mental Health Services for the Deaf.New York: Columbia Press, 1967.
14.
ReissigM., FinklesteinD., KashimaH., MassofR., HillisA., and ProctorD.Nasal cilia in retinitis pigmentosa.Investigative Ophthalmology and Visual Science, 1981, Supp. 20, 3, 41.
15.
UsherC. H.On the inheritance of retinitis pigmentosa, with notes of cases.Royal London Ophthalmology Hospital Report, 1914, 319, 130.
16.
VernonM.Usher's syndrome—deafness and progressive blindness.Journal of Chronic Diseases, 1969, 22, 133–151.
17.
VernonM.Usher's syndrome: Problems and solutions.Hearing and Speech Action, 1976, 1, 6–12.
18.
VernonM., BairR., and LotzS.Psychological evaluation and testing of children who are deaf-blind.School Psychology Digest, 1979, 8, 291–295.
19.
VernonM., and GreenD.A guide to the psychological evaluation of deaf-blind adults.Journal of Visual Impairment & Blindness, 1980, 9, 229–231.