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.