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MeSH Review

Hearing Loss

 
 
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Disease relevance of Hearing Loss

 

Psychiatry related information on Hearing Loss

 

High impact information on Hearing Loss

  • Through a transgenic rescue experiment, we verified that sequence polymorphisms in the neuron-specific microtubule-associated protein 1a gene (Mtap1a) observed in the susceptible strain C57BL/6J (B6) are crucial for the hearing-loss phenotype [1].
  • Urocortin-deficient mice show hearing impairment and increased anxiety-like behavior [11].
  • Beethoven, a mouse model for dominant, progressive hearing loss DFNA36 [12].
  • This indicates that at least some of the observed polymorphisms are functionally important and that the hearing loss in C57BL/6J-tub/tub (B6-tub/tub) mice may be caused by impaired protein interactions involving MTAP1A [1].
  • Dentinogenesis imperfecta 1 with or without progressive hearing loss is associated with distinct mutations in DSPP [13].
 

Chemical compound and disease context of Hearing Loss

 

Biological context of Hearing Loss

 

Anatomical context of Hearing Loss

 

Gene context of Hearing Loss

 

Analytical, diagnostic and therapeutic context of Hearing Loss

References

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  2. Eya1-deficient mice lack ears and kidneys and show abnormal apoptosis of organ primordia. Xu, P.X., Adams, J., Peters, H., Brown, M.C., Heaney, S., Maas, R. Nat. Genet. (1999) [Pubmed]
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  11. Urocortin-deficient mice show hearing impairment and increased anxiety-like behavior. Vetter, D.E., Li, C., Zhao, L., Contarino, A., Liberman, M.C., Smith, G.W., Marchuk, Y., Koob, G.F., Heinemann, S.F., Vale, W., Lee, K.F. Nat. Genet. (2002) [Pubmed]
  12. Beethoven, a mouse model for dominant, progressive hearing loss DFNA36. Vreugde, S., Erven, A., Kros, C.J., Marcotti, W., Fuchs, H., Kurima, K., Wilcox, E.R., Friedman, T.B., Griffith, A.J., Balling, R., Hrabé De Angelis, M., Avraham, K.B., Steel, K.P. Nat. Genet. (2002) [Pubmed]
  13. Dentinogenesis imperfecta 1 with or without progressive hearing loss is associated with distinct mutations in DSPP. Xiao, S., Yu, C., Chou, X., Yuan, W., Wang, Y., Bu, L., Fu, G., Qian, M., Yang, J., Shi, Y., Hu, L., Han, B., Wang, Z., Huang, W., Liu, J., Chen, Z., Zhao, G., Kong, X. Nat. Genet. (2001) [Pubmed]
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  16. Divergent roles for thyroid hormone receptor beta isoforms in the endocrine axis and auditory system. Abel, E.D., Boers, M.E., Pazos-Moura, C., Moura, E., Kaulbach, H., Zakaria, M., Lowell, B., Radovick, S., Liberman, M.C., Wondisford, F. J. Clin. Invest. (1999) [Pubmed]
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  20. Murine autoimmune hearing loss mediated by CD4+ T cells specific for inner ear peptides. Solares, C.A., Edling, A.E., Johnson, J.M., Baek, M.J., Hirose, K., Hughes, G.B., Tuohy, V.K. J. Clin. Invest. (2004) [Pubmed]
  21. Blood viscosity as a factor in sensorineural hearing impairment. Browning, G.G., Gatehouse, S., Lowe, G.D. Lancet (1986) [Pubmed]
  22. A gene for fluctuating, progressive autosomal dominant nonsyndromic hearing loss, DFNA16, maps to chromosome 2q23-24.3. Fukushima, K., Kasai, N., Ueki, Y., Nishizaki, K., Sugata, K., Hirakawa, S., Masuda, A., Gunduz, M., Ninomiya, Y., Masuda, Y., Sato, M., McGuirt, W.T., Coucke, P., Van Camp, G., Smith, R.J. Am. J. Hum. Genet. (1999) [Pubmed]
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  25. Mutations in COL11A2 cause non-syndromic hearing loss (DFNA13). McGuirt, W.T., Prasad, S.D., Griffith, A.J., Kunst, H.P., Green, G.E., Shpargel, K.B., Runge, C., Huybrechts, C., Mueller, R.F., Lynch, E., King, M.C., Brunner, H.G., Cremers, C.W., Takanosu, M., Li, S.W., Arita, M., Mayne, R., Prockop, D.J., Van Camp, G., Smith, R.J. Nat. Genet. (1999) [Pubmed]
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  29. Novel mutations in the connexin 26 gene (GJB2) that cause autosomal recessive (DFNB1) hearing loss. Kelley, P.M., Harris, D.J., Comer, B.C., Askew, J.W., Fowler, T., Smith, S.D., Kimberling, W.J. Am. J. Hum. Genet. (1998) [Pubmed]
  30. Usherin, the defective protein in Usher syndrome type IIA, is likely to be a component of interstereocilia ankle links in the inner ear sensory cells. Adato, A., Lefèvre, G., Delprat, B., Michel, V., Michalski, N., Chardenoux, S., Weil, D., El-Amraoui, A., Petit, C. Hum. Mol. Genet. (2005) [Pubmed]
  31. Mutations in the KCNQ4 gene are responsible for autosomal dominant deafness in four DFNA2 families. Coucke, P.J., Van Hauwe, P., Kelley, P.M., Kunst, H., Schatteman, I., Van Velzen, D., Meyers, J., Ensink, R.J., Verstreken, M., Declau, F., Marres, H., Kastury, K., Bhasin, S., McGuirt, W.T., Smith, R.J., Cremers, C.W., Van de Heyning, P., Willems, P.J., Smith, S.D., Van Camp, G. Hum. Mol. Genet. (1999) [Pubmed]
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  33. Human nonsyndromic hereditary deafness DFNA17 is due to a mutation in nonmuscle myosin MYH9. Lalwani, A.K., Goldstein, J.A., Kelley, M.J., Luxford, W., Castelein, C.M., Mhatre, A.N. Am. J. Hum. Genet. (2000) [Pubmed]
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