[Argyll-Robertson syndrome during diabetes mellitus; diabetic ocular neuropathies].
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In France, the combination of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (DIDMOAD) is designated as Wolfram syndrome. An analysis of 14 personal cases and previous reports showed that the syndrome develops gradually and specified the most common order of occurrence of the various components as well as the other abnormalities (e.g., of the urinary tract) which may be found. Wolfram syndrome is an inherited condition (recessive autosomal transmission). The lack of association with HLA antigens seems to have been established (in the few cases where HLA typing was performed). The prognosis of Wolfram syndrome is grim, with the occurrence of each additional component adding to the severity of the disease.
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We observed the course of diabetes in 23 genetically diabetic mice (C 57 BL/KSJ mdb) and in 25 controls (C 57 BL/KSJ). Diabetes was not treated. Histological changes of pancreas, aorta, kidneys, and inner ear vessels were noted. Auditory function was tested by means of the pinnal reflex of Preyer for a frequency range between 4000 and 20000 cps. The diabetic mice developed partly a mild diabetes associated with weight gain, and partly a severe diabetes with a loss of weight. Histologically, the diabetic mice showed (dependent on the duration and degree of severity of the diabetes) progressive mesangial sclerosis and thickening of basal laminas of the glomerula, as well as progressive slight to moderate thickening of the vascular walls of all inner ear vessels. The hearing ability of diabetic mice and of controls diminished with age to a comparable degree. The diabetic mice showed a slightly higher hearing loss for high frequencies, which did not correlate with the extent of thickening of inner ear vessel walls.
Diabetic ketoacidosis (DKA) and hyperglycemic hyperosmolar state (HHS) potentially are fatal but largely preventable acute metabolic conditions of uncontrolled diabetes, the incidence of which continues to increase. Mortality from DKA has declined remarkably over the years because of better understanding of its pathophysiology and treatment. The mortality rate of HHS remains alarmingly high, however, owing to older age and mode of presentation of patients and associated comorbid conditions. DKA and HHS also are economically burdensome; therefore, any resources invested in their prevention would be rewarding.
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