| First Author | British Society of Audiology short papers meeting on experimental studies of hearing and deafness. Cambridge, UK, 22-23 September 1996. Self TJ | Year | 1997 |
| Journal | Br J Audiol | Volume | 31 |
| Issue | 2 | Pages | 73-132 (82 Abstr.) |
| Mgi Jnum | J:39921 | Mgi Id | MGI:87263 |
| Citation | British Society of Audiology short papers meeting on experimental studies of hearing and deafness. Cambridge, UK, 22-23 September 1996. Self TJ, et al. (1997) British Society of Audiology short papers meeting on experimental studies of hearing and deafness. CambridgA scanning electron microscope study of the development of the mouse mutant Snell's waltzer. Br J Audiol 31(2):73-132 (82 Abstr.) |
| abstractText | Full text of Abstract. A scanning electron microscope study of the development of the mouse mutant Snell's waltzer. T.J. Self1, K. Avraham2 and K.P. Steel1, 1MRC Institute of Hearing Research, University Park, Nottingham, NG7 2RD, and 2Sackler School of Medicine, Tel Aviv University, Israel. The Snell's waltzer (sv) mouse has an autosomal recessive mutation on chromosome 9, characterized by deafness, hyperactivity, jerking movements of the head and circling. The histology was first described at the light microscope level by Deol and Green (1966). They observed a degeneration and eventual loss of hair cells in the organ of Corti. The mutation has recently been identified and encodes an unconventional myosin heavy chain, myosin VI (Avraham et al., 1995). The sv gene is expressed within sensory hair cells of the inner ear and is thought to be required for maintaining their structural integrity. In our study we have examined the surface morphology of the organ of Corti, using scanning electron microscopy of 6 mutants and 7 heterozygous littermate controls between the ages of 12 and 20 days after birth (DAB). The outer and inner hair cell stereocilia at 12 DAB were found to be disrupted with many udergoing fusion and elongation to produce super-stereocilia. At 20 DAB the disruption to stereocilia had increased. Also some hair cells appeared to have degenerated completely. The super-stereocilia had developed large bulges at their tips at this stage. The outer and inner hair cell stereocilia were found to be undergoing progressive degenerative disruption and fusion, resulting in a very abnormal surface arrangement. All littermate controls were unaffected. References Avraham KB, Hasson T, Steel KP, Kingsley DM, Russell LB, Mooseker MS, Copeland NG, Jenkins NA. Nature Genetics, 1995; 11:369-75. Deol MS, Green MC. Genet Res, 1966; 8:339-45. |
