| First Author | British Society of Audiology short papers meeting on experimental studies of hearing and deafness. Cambridge, United Kingdom, 22-23 September 1996. Bussoli T | Year | 1997 |
| Journal | Br J Audiol | Volume | 31 |
| Issue | 2 | Pages | 73-132 (83 Abstr.) |
| Mgi Jnum | J:39922 | Mgi Id | MGI:87264 |
| Citation | British Society of Audiology short papers meeting on experimental studies of hearing and deafness. Cambridge, United Kingdom, 22-23 September 1996. Bussoli T, et al. (1997) Localization of the bronx waltzer mouse mutation and its effect on organ of Corti development. Br J Audiol 31(2):73-132 (83 Abstr.) |
| abstractText | Full text of Abstract. Localization of the bronx waltzer mouse mutation and its effect on organ of Corti development. T. Bussoli, A. Kelly and K.P. Steel, MRC Institute of Hearing Research, Nottingham NG7 2RD The bronx waltzer (bv) mutation is an autosomal recessive mutation that is manifested as head tossing, circling and hearing impairment in the mouse. The mutation affects the inner hair cells (IHCs) and pillar cells in the organ of Corti, and the maculae and cristae of the vestibular part of the inner ear (Deol and Gluecksohn-Waelsch, 1979). Abnormalities are seen in the IHCs as early as gestational (G) day 17 (Whitlon et al., 1995) and only 25% of IHCs are present in the adult organ of Corti. Our study of the bronx waltzer organ of Corti confirmed that IHCs begin to degenerate in the basal/middle coils between G17andG18 by a controlled process of cell death followed 2 days later by the same process in the apical coil. At birth degenerating IHCs are not arranged in a regular row as normal, and they are seen being resorbed into the sensory epithelium as supporting cells creep over the IHC surface. Secondary effects of IHC degeneration include a breakdown in the regular arrangement of pillar cells and outer hair cells. As very few genes causing deafness have been cloned, the identification of this gene will prove very useful in forwarding our knowledge of how the mammalian organ of Corti both develops and functions. As a first step towards identification we have analysed a total of 20 loci in 118 progeny from an intraspecific with the mouse strain 101/H: [(bv/bv x 101/ H)Fl x bv/bv].This gave the gene order; centromere - D5Mit1 - D5Mit73 - D5Mit55 - [D5Mit12, D5Nds4 (Afp)] - D5Mit87 Ã [D5Mit205, 20, 88, 208, 93] - D5Mit338 - D5Mit25 [D5Mit209, bv] Ã D5Mit188 - D5Mit367- D5Mit95- D5Mit43 - D5Mit102. We have further analysed the markers D5Mit(93, 338, 25, 209, 188, 367, 95) and by covering a genetic distance of 11.56 cM in over 450 mice, to give a more detailed localization of bv. We have shown that the bv gene localizes to the central region of chromosome 5 and that this mutation not only affects IHC development, but that secondary effects include OHC and pillar cell disorganization. Acknowledgement Supported by the MRC and Hearing Research Trust. References Deol MS, Gluecksohn-Waelsch S. Nature, 1979; 278:250-2. Whitlon DS, Gabel C, Zhang X. J Comp Neurol, 1995; 364:515-22. |
