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Visual Function in the Syndrome: Diabetes Mellitus, Diabetes Insipidus, Optic Atrophy, and Hearing Loss*

Published online by Cambridge University Press:  01 August 2014

Jean-Real Brunette*
Affiliation:
Hôpital Maisonneuve-Rosemont, Université de Montréal
John M. Little
Affiliation:
Montreal Children's Hospital, Mc Gill University, Montreal, Canada
*
Hôpital Maisonneuve-Rosemont, Université de Montréal, 5689 Boul. Rosemont, Montréal 410, P.Q., Canada

Abstract

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Since the late thirties a syndrome has been progressively completing its description. It consists essentially in juvenile diabetes mellitus, diabetes insipidus, neurosensory hearing loss and optic atrophy. Neurosensory hearing loss, neurogenic bladder, autonomic dysfunction and hyperalanineuria have been added. The syndrome is generally familial.

Electrophysiologic and psychophysiological retinal studies have been done in these cases. Profound electrophysiological disturbance has been described. Cases under actual evaluation are presented. Physiopathologic processes remain a problem. The nature of the retinal affection is also open for discussion.

Type
5. Free Contributions: First Group
Copyright
Copyright © The International Society for Twin Studies 1974

Footnotes

*

Travail du Laboratoire de Neurophysiologie Visuelle, subvention No. MA-2593 du Conseil de la Recherche Médicale du Canada.

References

BIBLIOGRAPHIE

Albutt, T.C. 1871. On the Use of the Ophthalmoscope of the Nervous System and of the Kidneys, Also in Certain Other General Disorders. London.Google Scholar
Alström, C.H., Hallgren, B., Nilsson, C.B., Asandar, H. 1959. Retinal degeneration combined with obesity, diabetes mellitus and neurogenous deafness. Acta Psychiatr. Scand. [Suppl.]: 129: 135.Google ScholarPubMed
Bretz, G.W., Baghdassarian, A. 1970. Co-existence of diabetes mellitus and diabetes insipidus and optic atrophy in two male sibs. Am. J. Med., 48: 398403.Google Scholar
Brunette, J.R. 1972. Oscillatory potentials relationships using computer averaging techniques. Can. J. Ophthalmol., 7: 223229.Google Scholar
Brunette, J.R. 1973. A standardizable method for separating rod and cone responses in clinical electroretinography. Am. J. Opthalmol., 75: 833845.Google Scholar
Ikkos, O.G., Fraser, G.R., Matsouki-Gavra, E., Petrochilos, A. 1970. The association of juvenile diabetes mellitus, primary optic atrophy and perceptive hearing loss in three sibs with additional ideopathic diabetes mellitus insipidus in one case. Acta Endocrinol., 65: 95102.Google Scholar
Moore, J.R. 1971. Juvenile diabetes mellitus, diabetes insipidus and neurological abnormalities. Proc. R. Soc. Med., 64: 730.Google Scholar
Najjar, S., Mahmud, J. 1968. Diabetes insipidus and diabetes mellitus in a six year old girl. J. Pediatr., 73: 251253.Google Scholar
Niemeyer, G., Marquardt, J.L. 1972. Retinal function in an unique syndrome of optic atrophy, diabetes mellitus, diabetes insipidus with hearing loss and hyperalaninuria. Invest. Ophthalmol., 11: 617624.Google Scholar
Rorsman, G., Soderstrom, N. 1967. Optic atrophy and juvenile diabetes mellitus with familial occurrence. Acta Med. Scand., 182: 419425.CrossRefGoogle ScholarPubMed
Rose, F.C., Fraser, G.R., Freidmann, A.I., Kohner, E.M. 1966. The association of juvenile diabetes mellitus and optic atrophy: clinical and genetic aspects. Q.J. Med., New Series XXV, 139: 385407.Google Scholar
Shaw, D.A., Duncan, L.J.P. 1958. Optic atrophy and nerve deafness in diabetes mellitus. J. Neurol. Neurosurg. Psychiatr., 21: 4749.Google Scholar
Sunder, J.M., Danowski, T.S., Kenny, F.M. 1972. Pedigrees with diabetes insipidus, diabetes mellitus and atrophy. J. Med. Genet., 9: 408412.Google Scholar
Waite, J.H., Beetham, W.P. 1935. The visual mechanism of diabetes mellitus. N. Engl. J. Med., 212, 367, 429.Google Scholar
Wolfram, .Google Scholar