Usher's Syndrome: Electrophysiological Tests of the Visual and Auditory Systems

Overview

Journal Doc Ophthalmol

Specialty Ophthalmology

Date 1977 Dec 30

PMID 598276

Citations 2

Authors

F A Abraham

D Cohen

H Sohmer

Affiliations

Soon will be listed here.

Abstract

The symptoms of Usher's syndrome - congenital hearing impairment and tapetoretinal dystrophy - are difficult to detect in young children. The electroretinogram (ERG), visual evoked potential (VEP), auditory nerve and brain-stem responses as well as the cochlear microphonic potentials were used to evaluate the defects of the visual and auditory systems in 20 patients, 3-45 years of age. This study demonstrates the usefulness, reliability and convenience of the electrophysiological tests for early diagnosis and functional evaluation of Usher's syndrome.

Citing Articles

Anisocoria in unilateral ophthalmic disease.

Pearson R, Rose G Br J Ophthalmol. 1991; 75(1):31-3.

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Visual loss and foveal lesions in Usher's syndrome.

Fishman G, Vasquez V, Fishman M, Berger D Br J Ophthalmol. 1979; 63(7):484-8.

PMID: 465425 PMC: 1043521. DOI: 10.1136/bjo.63.7.484.

References

Sohmer H, Feinmesser M . Electrocochleography in clinical-audiological diagnosis. Arch Otorhinolaryngol. 1974; 206(2):91-102. DOI: 10.1007/BF00460400. View

STRAUB W, Schmidt B . [The diagnosis of tapeto-retinal degenerescence in children]. Bull Mem Soc Fr Ophtalmol. 1969; 82:5-11. View

McGOVERN F . The association of nerve deafness and retinitis pigmentosa. Interval report. Ann Otol Rhinol Laryngol. 1960; 69:1044-53. DOI: 10.1177/000348946006900411. View

Sohmer H, Pratt H . Recording of the cochlear microphonic potential with surface electrodes. Electroencephalogr Clin Neurophysiol. 1976; 40(3):253-60. DOI: 10.1016/0013-4694(76)90149-8. View

Goodman G, GUNKEL R . Familial electroretinographic and adaptometric studies in retinitis pigmentosa. Am J Ophthalmol. 1958; 46(3 Part 2):142-72. DOI: 10.1016/0002-9394(58)90062-x. View

Holland M, Cambie E, KLOEPFER W . An evaluation of genetic carriers of Usher's syndrome. Am J Ophthalmol. 1972; 74(5):940-7. DOI: 10.1016/0002-9394(72)91215-9. View

ARMINGTON J . The electroretinogram, the visual evoked potential, and the area-luminance relation. Vision Res. 1968; 8(3):263-76. DOI: 10.1016/0042-6989(68)90014-x. View

Yoshie N, Yamaura K . Cochlear microphonic responses to pure tones in man recorded by a non-surgical method. Acta Otolaryngol Suppl. 1969; 252:37-69. DOI: 10.3109/00016486909120513. View

SUNGA R, SLOAN L . Pigmentary degeneration of the retina: early diagnosis and natural history. Invest Ophthalmol. 1967; 6(3):309-25. View

10.

Belal Jr A . Usher's syndrome. (Retinitis pigmentosa and deafness). A temporal bone report. J Laryngol Otol. 1975; 89(2):175-81. DOI: 10.1017/s0022215100080233. View

11.

AMMANN F, Klein D, Franceschetti A . Genetic and epidemiological investigations on pigmentary degeneration of the retina and allied disorders in Switzerland. J Neurol Sci. 1965; 2(2):183-96. DOI: 10.1016/0022-510x(65)90079-1. View

12.

Jacobson J, Hirose T, SUZUKI T . Simultaneous ERG and VER in lesions of the optic pathway. Invest Ophthalmol. 1968; 7(3):279-92. View

13.

DeVoe R, Ripps H, Vaughan Jr H . Cortical responses to stimulation of the human fovea. Vision Res. 1968; 8(2):135-47. DOI: 10.1016/0042-6989(68)90002-3. View

14.

Auerbach E . The human electroretinogram in the light and during dark adaptation. Doc Ophthalmol. 1967; 22:1-71. DOI: 10.1007/BF00144160. View

15.

COATS A, DICKEY J . Nonsurgical recording of human auditory nerve action potentials and cochlear microphonics. Ann Otol Rhinol Laryngol. 1970; 79(4):844-52. DOI: 10.1177/000348947007900417. View

16.

Regan D, HERON J . Clinical investigation of lesions of the visual pathway: a new objective technique. J Neurol Neurosurg Psychiatry. 1969; 32(5):479-83. PMC: 496563. DOI: 10.1136/jnnp.32.5.479. View

17.

HALLGREN B . Retinitis pigmentosa combined with congenital deafness; with vestibulo-cerebellar ataxia and mental abnormality in a proportion of cases: A clinical and genetico-statistical study. Acta Psychiatr Scand Suppl. 1959; 34(138):1-101. View

18.

Sohmer H, Feinmesser M, Szabo G . Sources of electrocochleographic responses as studied in patients with brain damage. Electroencephalogr Clin Neurophysiol. 1974; 37(6):663-9. DOI: 10.1016/0013-4694(74)90081-9. View

19.

Cherry P . Usher's syndrome. Ann Ophthalmol. 1973; 5(7):743-52. View

20.

Kosel K . [Electroretinographic studies on the exclusion of tapetoretinal degeneration in children]. Klin Monbl Augenheilkd. 1971; 159(5):645-9. View