Fig. 7.

Age-related degeneration of cochlear hair cells in Cdh23^v-ngt/ahl heterozygous mice. SEM micrographs show the stereociliary phenotypes of compound Cdh23^v-ngt/ahl heterozygous (A–G) and hypomorphic Cdh23^ahl/ahl homozygous (H, I) mice. (A–G) Stereociliary morphology of OHCs (A-F) and IHCs (G) in Cdh23^v-ngt/ahl mice at 4, 7 and 10 months of age. Asterisks indicate OHCs with missing bundles (B, D). Highly magnified images of stereocilia in the dotted boxes in B and E are shown in C and F, respectively. The arrowhead (F) indicates an elongated tip link, and the arrow (G) indicates a fused stereocilia bundle. (H, I) Stereociliary morphology of OHCs in Cdh23^ahl/ahl mice at 10 and 15 months of age. Scale bars=5 µm (A, B, D, E, G–I) and 2 µm (C, F).