Specific pathology of the stria vascularis in postnatal progressive genetic inner ear disorder.
After reaching normal morphological maturation the stria vascularis of the Shaker-2 (Sh-2) mouse postnatally underwent a progressive degeneration, especially of the intermediate cell layer. Considerable changes were present on the 3rd week after birth. The marginal cells became atrophic and showed a reduction in the number and extent of the cellular extensions towards the intermediate cell layer. The intermediate cells disintegrated but were present 9 weeks after birth. The basal cells showed a normal morphology. Specific inclusion bodies were identified in the marginal cells from the 3rd week on. Occasionally such inclusions occurred in marginal cells. In the Shaker-1 (Sh-1) a flattening of intermediate cells occurred but otherwise no pathological changes were found 6--9 weeks after birth.
D000367:Age Factors; D000818:Animals; D001284:Atrophy; D003051:Cochlea / Q000648:ultrastructure*; D004195:Disease Models, Animal; D007758:Ear, Inner / Q000473:pathology / Q000648:ultrastructure*; D007759:Labyrinth Diseases / Q000235:genetics* / Q000473:pathology; D051379:Mice; D008821:Mice, Quaking; D013316:Stria Vascularis / Q000473:pathology / Q000648:ultrastructure*; D014722:Vestibule, Labyrinth / Q000473:pathology