First Author | Watanabe M | Year | 2005 |
Journal | In Vivo | Volume | 19 |
Issue | 4 | Pages | 675-81 |
PubMed ID | 15999533 | Mgi Jnum | J:148676 |
Mgi Id | MGI:3846050 | Citation | Watanabe M, et al. (2005) Progressive degeneration of stereocilia in cochlear hair cells in hearing-impaired kuru2 mice. In Vivo 19(4):675-81 |
abstractText | BACKGROUND: We previously isolated a mouse strain, kuru2, which exhibits abnormal behavior and hearing impairment. To investigate the etiology of this impairment, the ultrastructure of the inner ear was examined. MATERIALS AND METHODS: The morphologies of the cochlea and the vestibule of control (Jcl:ICR) and mutant mice were analyzed by electron microscopy. In some experiments, the mice were cross-mated and their offspring examined. RESULTS: The mutant mice displayed progressive degeneration of the stereocilia in the cochlea. The stereocilia started to degenerate on post-natal day 10 and, subsequently, the hair bundles continued to degenerate. On day 18, degeneration of the stereocilia was complete. In contrast, the vestibule was intact. DISCUSSION: Many mutant mice display hearing impairment These mice demonstrate a characteristic morphology of the inner ear and, since correlations may be made with corresponding human diseases, the current results could contribute to the further understanding of hearing impairment mechanisms. |