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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


  1. Microtubule-associated protein 1A is a modifier of tubby hearing (moth1). Ikeda, A., Zheng, Q.Y., Zuberi, A.R., Johnson, K.R., Naggert, J.K., Nishina, P.M. Nat. Genet. (2002) [Pubmed]
  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]
  3. Epistatic relationship between Waardenburg syndrome genes MITF and PAX3. Watanabe, A., Takeda, K., Ploplis, B., Tachibana, M. Nat. Genet. (1998) [Pubmed]
  4. Defective myosin VIIA gene responsible for Usher syndrome type 1B. Weil, D., Blanchard, S., Kaplan, J., Guilford, P., Gibson, F., Walsh, J., Mburu, P., Varela, A., Levilliers, J., Weston, M.D. Nature (1995) [Pubmed]
  5. Dexamethasone as adjunctive therapy in bacterial meningitis. A meta-analysis of randomized clinical trials since 1988. McIntyre, P.B., Berkey, C.S., King, S.M., Schaad, U.B., Kilpi, T., Kanra, G.Y., Perez, C.M. JAMA (1997) [Pubmed]
  6. Spectrum of mutations in alpha-mannosidosis. Berg, T., Riise, H.M., Hansen, G.M., Malm, D., Tranebjaerg, L., Tollersrud, O.K., Nilssen, O. Am. J. Hum. Genet. (1999) [Pubmed]
  7. Mode of action: neurotoxicity induced by thyroid hormone disruption during development--hearing loss resulting from exposure to PHAHs. Crofton, K.M., Zoeller, R.T. Crit. Rev. Toxicol. (2005) [Pubmed]
  8. Autosomal dominant syndrome with strokelike episodes and leukoencephalopathy. Tournier-Lasserve, E., Iba-Zizen, M.T., Romero, N., Bousser, M.G. Stroke (1991) [Pubmed]
  9. Prevalence of epilepsy and epileptic seizures in 10-year-old children: results from the Metropolitan Atlanta Developmental Disabilities Study. Murphy, C.C., Trevathan, E., Yeargin-Allsopp, M. Epilepsia (1995) [Pubmed]
  10. Language and speech perception outcomes in hearing-impaired children with and without connexin 26 mutations. Dahl, H.H., Wake, M., Sarant, J., Poulakis, Z., Siemering, K., Blamey, P. Audiol. Neurootol. (2003) [Pubmed]
  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]
  14. A comparison of ceftriaxone and cefuroxime for the treatment of bacterial meningitis in children. Schaad, U.B., Suter, S., Gianella-Borradori, A., Pfenninger, J., Auckenthaler, R., Bernath, O., Cheseaux, J.J., Wedgwood, J. N. Engl. J. Med. (1990) [Pubmed]
  15. N-methyl-D-aspartate antagonists limit aminoglycoside antibiotic-induced hearing loss. Basile, A.S., Huang, J.M., Xie, C., Webster, D., Berlin, C., Skolnick, P. Nat. Med. (1996) [Pubmed]
  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]
  17. Ifosfamide and exacerbation of cisplatin-induced hearing loss. Meyer, W.H., Ayers, D., McHaney, V.A., Roberson, P., Pratt, C.B. Lancet (1993) [Pubmed]
  18. Erythromycin-induced hearing loss. Boyd, I. Lancet (1991) [Pubmed]
  19. Soundbites. Patel, P. Nat. Genet. (2001) [Pubmed]
  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]
  23. A new nonsyndromic X-linked sensorineural hearing impairment linked to Xp21.2. Lalwani, A.K., Brister, J.R., Fex, J., Grundfast, K.M., Pikus, A.T., Ploplis, B., San Agustin, T., Skarka, H., Wilcox, E.R. Am. J. Hum. Genet. (1994) [Pubmed]
  24. Mutations in the gene encoding gap junction protein beta-3 associated with autosomal dominant hearing impairment. Xia, J.H., Liu, C.Y., Tang, B.S., Pan, Q., Huang, L., Dai, H.P., Zhang, B.R., Xie, W., Hu, D.X., Zheng, D., Shi, X.L., Wang, D.A., Xia, K., Yu, K.P., Liao, X.D., Feng, Y., Yang, Y.F., Xiao, J.Y., Xie, D.H., Huang, J.Z. Nat. Genet. (1998) [Pubmed]
  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]
  26. GJB2 mutations and degree of hearing loss: a multicenter study. Snoeckx, R.L., Huygen, P.L., Feldmann, D., Marlin, S., Denoyelle, F., Waligora, J., Mueller-Malesinska, M., Pollak, A., Ploski, R., Murgia, A., Orzan, E., Castorina, P., Ambrosetti, U., Nowakowska-Szyrwinska, E., Bal, J., Wiszniewski, W., Janecke, A.R., Nekahm-Heis, D., Seeman, P., Bendova, O., Kenna, M.A., Frangulov, A., Rehm, H.L., Tekin, M., Incesulu, A., Dahl, H.H., du Sart, D., Jenkins, L., Lucas, D., Bitner-Glindzicz, M., Avraham, K.B., Brownstein, Z., del Castillo, I., Moreno, F., Blin, N., Pfister, M., Sziklai, I., Toth, T., Kelley, P.M., Cohn, E.S., Van Maldergem, L., Hilbert, P., Roux, A.F., Mondain, M., Hoefsloot, L.H., Cremers, C.W., Löppönen, T., Löppönen, H., Parving, A., Gronskov, K., Schrijver, I., Roberson, J., Gualandi, F., Martini, A., Lina-Granade, G., Pallares-Ruiz, N., Correia, C., Fialho, G., Cryns, K., Hilgert, N., Van de Heyning, P., Nishimura, C.J., Smith, R.J., Van Camp, G. Am. J. Hum. Genet. (2005) [Pubmed]
  27. Predictors of sudden cardiac death. Friedman, G.D., Klatsky, A.L., Siegelaub, A.B. Circulation (1975) [Pubmed]
  28. Epidemiology of A3243G, the mutation for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes: prevalence of the mutation in an adult population. Majamaa, K., Moilanen, J.S., Uimonen, S., Remes, A.M., Salmela, P.I., Kärppä, M., Majamaa-Voltti, K.A., Rusanen, H., Sorri, M., Peuhkurinen, K.J., Hassinen, I.E. Am. J. Hum. Genet. (1998) [Pubmed]
  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]
  32. Visual and auditory neurotoxicity in patients receiving subcutaneous deferoxamine infusions. Olivieri, N.F., Buncic, J.R., Chew, E., Gallant, T., Harrison, R.V., Keenan, N., Logan, W., Mitchell, D., Ricci, G., Skarf, B. N. Engl. J. Med. (1986) [Pubmed]
  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]
  34. Clinically significant hearing loss in renal allograft recipients treated with intravenous erythromycin. Vasquez, E.M., Maddux, M.S., Sanchez, J., Pollak, R. Arch. Intern. Med. (1993) [Pubmed]
  35. Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma. Paulino, A.C., Simon, J.H., Zhen, W., Wen, B.C. Int. J. Radiat. Oncol. Biol. Phys. (2000) [Pubmed]
  36. Intensity-modulated radiation therapy for pediatric medulloblastoma: early report on the reduction of ototoxicity. Huang, E., Teh, B.S., Strother, D.R., Davis, Q.G., Chiu, J.K., Lu, H.H., Carpenter, L.S., Mai, W.Y., Chintagumpala, M.M., South, M., Grant, W.H., Butler, E.B., Woo, S.Y. Int. J. Radiat. Oncol. Biol. Phys. (2002) [Pubmed]
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