Überlegungen Zur Pathogenese Des Cochleo-Renalen Syndroms: Considerations on the Pathogenesis of the Cochleo-renal Syndrome

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Abstract
The histological findings in 2 cases of Alport''s syndrome are presented. Damage within the inner ear occurs mainly in the vascular stria region and apparently concur with the renal alterations. Glomerular basement thickening and splitting occur 1st with confluence of the epithelial feet. Increase in the epithelial cell volume and tremendous enlargement of the glomerular basement membranes later on leads to an obliteration glomerular capillaries. In the 1st case a subtotal loss of stria vascularis is apparently the cause degeneration of the inner and outer hair cells and some spiral ganglion cells. The 2nd case revealed only a slight edema of the stria vascularis and apical protrusions of cytoplasm, sometimes vascuolated. In the basal turn most of the inner hair cells had disappeared and in the upper turns a slight degeneration of the inner hair cell occurred but the outer hair cell system remained intact. Inner ear damage in the cochleo-renal syndrome apparently secondary to the kidney disease.