Top

Journal of the Association for Research in Otolaryngology

Published in:

01-03-2004

Progressive Hearing Loss and Increased Susceptibility to Noise-Induced Hearing Loss in Mice Carrying a Cdh23 but not a Myo7a Mutation

Authors: Ralph H. Holme, Karen P. Steel

Published in: Journal of the Association for Research in Otolaryngology | Issue 1/2004

Abstract

Exposure to intense noise can damage the stereocilia of sensory hair cells in the inner ear. Since stereocilia play a vital role in the transduction of sound from a mechanical stimulus into an electrical one, this pathology is thought to contribute to noise-induced hearing loss. Mice homozygous for null mutations in either the myosin VIIa (Myo7a) or cadherin 23 (Cdh23) genes are deaf and have disorganized stereocilia bundles. We show that mice heterozygous for a presumed null allele of Cdh23 (Cdh23 ^v) have low- and high-frequency hearing loss at 5–6 weeks of age, the high-frequency component of which worsens with increasing age. We also show that noise-induced hearing loss in 11–12-week-old Cdh23 ^v heterozygotes is two times greater than for wild-type littermates. Interestingly, these effects are dependent upon the genetic background on which the Cdh23 ^v mutation is carried. Noise-induced hearing loss in 11–12-week-old mice heterozygous for a null allele of Myo7a (Myo7a ^4626SB) is not significantly different from wild-type littermates. CDH23 is the first gene known to cause deafness in the human population to be linked with predisposition to noise-induced hearing loss.

Boeda, B, El-Amraoui, A, Bahloul, A, Goodyear, R, Daviet, L, Blanchard, S, Perfettini, I, Fath, KR, Shorte, S, Reiners, J, Houdusse, A, Legrain, P, Wolfrum, U, Richardson, G, Petit, C 2002Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle.EMBO J2166896699CrossRefPubMed

Bolz, H, von Brederlow, B, Ramirez, A, Bryda, EC, Kutsche, K, Nothwang, HG, Seeliger, M, del C-Salcedo Cabrera, M, Vila, MC, Molina, OP, Gal, A, Kubisch, C 2001Mutation of CDH23, encoding a new member of the cadherin gene family, causes Usher syndrome type 1D.Nat. Genet27108112CrossRefPubMed

Bork, JM, Peters, LM, Riazuddin, S, Bernstein, SL, Ahmed, ZM, Ness, SL, Polomeno, R, Ramesh, A, Schloss, M, Srisailpathy, CR, Wayne, S, Bellman, S, Desmukh, D, Ahmed, Z, Khan, SN, Kaloustian, VM, Li, XC, Lalwani, A, Riazuddin, S, Bitner–Glindzicz, M, Nance, WE, Liu, XZ, Wistow, G, Smith, RJ, Griffith, AJ, Wilcox, ER, Friedman, TB, Morell, RJ 2001Usher syndrome 1D and nonsyndromic autosomal recessive deafness DFNB12 are caused by allelic mutations of the novel cadherin-like gene CDH23.AM. J. Hum. Genet682637CrossRefPubMed

Bryda, EC, Kim, HJ, Legare, ME, Frankel, WN, Noben–Trauth, K 2001High-resolution genetic and physical mapping of modifier-of-deafwaddler (mdfw) and Waltzer (Cdh23v).Genomics73338342PubMed

Davis, AC 1989The prevalence of hearing impairment and reported hearing disability among adults in Great Britain.Int. J. Epidemiol8911917

Davis, RR, Newlander, JK, Ling, X, Cortopassi, GA, Krieg, EF, Erway, LC 2001Genetic basis for susceptibility to noise-induced hearing loss in mice.Hear. Res1558290CrossRefPubMed

Di Palma, F, Holme, RH, Bryda, EC, Belyantseva, IA, Pellegrino, R, Kachar, B, Steel, KP, Noben–Trauth, K 2001Mutations in Cdh23, encoding a new type of cadherin, cause stereocilia disorganization in waltzer, the mouse model for Usher syndrome type 1D.Nat. Genet27103107CrossRefPubMed

Ehret, G 1975Masked auditory thresholds, critical ratios, and scales of the basilar membrane of the housemouse (Mus musculus).J. Comp. Physiol103329341

Erway, LC, Shiau, YW, Davis, RR, Krieg, EF 1996Genetics of age-related hearing loss in mice. III. Susceptibility of inbred and F1 hybrid strains to noise-induced hearing loss.Hear. Res93181187CrossRefPubMed

10.

Gibson, F, Walsh, J, Mburu, P, Varela, A, Brown, KA, Antonio, M, Beisel, KW, Steel, KP, Brown, SD 1995A type VII myosin encoded by the mouse deafness gene shaker-1.Nature3746264CrossRefPubMed

11.

Hakuba, N, Koga, K, Gyo, K, Usami, SI, Tanaka, K 2000Exacerbation of noise-induced hearing loss in mice lacking the glutamate transporter GLAST.J. Neurosci2087508753PubMed

12.

Hasson, T, Gillespie, PG, Garcia, JA, MacDonald, RB, Zhao, Y, Yee, AG, Mooseker, MS, Corey, DP 1997aUnconventional myosins in inner-ear sensory epithelia.J. Cell. Biol13712871307

13.

Hasson, T, Walsh, J, Cable, J, Mooseker, MS, Brown, SD, Steel, KP 1997bEffects of shaker-1 mutations on myosin-VIIa protein and mRNA expression.Cell Motil. Cytoskeleton37127138

14.

Hequembourg, S, Liberman, MC 2001Spiral ligament pathology: a major aspect of age-related cochlear degeneration in C57BL/6 mice.J. Assoc. Res. Otolaryngol2118129PubMed

15.

Holme, RH, Steel, KP 2002Stereocilia defects in waltzer (Cdh23), shaker1 (Myo7a) and double waltzer/shaker1 mutant mice.Hear. Res1691323CrossRefPubMed

16.

Hudspeth, AJ 1989How the ear’s works work.Nature341397404PubMed

17.

Hunter–Duvar, IM 1978A technique for preparation of cochlear specimens for assessment with the scanning electron microscope.Acta Otolaryngol. Suppl351323PubMed

18.

Johnson, KR, Erway, LC, Cook, SA, Willott, JF, Zheng, QY 1997A major gene affecting age-related hearing loss in C57BL/6J mice.Hear. Res1148392CrossRefPubMed

19.

Johnson, KR, Zheng, QY, Bykhovskaya, Y, Spirina, O, Fischel–Ghodsian, N 2001A nuclear–mitochondrial DNA interaction affecting hearing impairment in mice.Nat. Genet27191194CrossRefPubMed

20.

Johnson, KR, Zheng, QY, Erway, LC 2000A major gene affecting age-related hearing loss is common to at least ten inbred strains of mice.Genomics70171180

21.

Keithley, EM, Ma, CL, Ryan, AF, Louis, JC, Magal, E 1998GDNF protects the cochlea against noise damage.Neuroreport921832187PubMed

22.

Kozel, PJ, Davis, RR, Krieg, EF, Shull, GE, Erway, LC 2002Deficiency in plasma membrane calcium ATPase isoform 2 increases susceptibility to noise-induced hearing loss in mice.Hear. Res164231239CrossRefPubMed

23.

Kros, CJ, Marcotti, W, van Netten, SM, Self, TJ, Libby, RT, Brown, SD, Richardson, GP, Steel, KP 2002Reduced climbing and increased slipping adaptation in cochlear hair cells of mice with Myo7a mutations.Nat. Neurosci54147CrossRefPubMed

24.

Leckband, D, Sivasankar, S 2000Mechanism of homophilic cadherin adhesion.Curr. Opin. Cell Biol12587592CrossRefPubMed

25.

Liberman, MC 1987Chronic ultrastructural changes in acoustic trauma: serial-section reconstruction of stereocilia and cuticular plates.Hear. Res266588CrossRefPubMed

26.

Liu, XZ, Walsh, J, Mburu, P, Kendrick–Jones, J, Cope, MJ, Steel, KP, Brown, SD 1997aMutations in the myosin VIIA gene cause non-syndromic recessive deafness.Nat. Genet16188190

27.

Liu, XZ, Walsh, J, Tamagawa, Y, Kitamura, K, Nishizawa, M, Steel, KP, Brown, SD 1997bAutosomal dominant non-syndromic deafness caused by a mutation in the myosin VIIA gene.Nat. Genet17268269

28.

Maison, SF, Luebke, AE, Liberman, MC, Zuo, J 2002Efferent protection from acoustic injury is mediated via alpha9 nicotinic acetylcholine receptors on outer hair cells.J. Neurosci221083810846PubMed

29.

Mburu, P, Liu, XZ, Walsh, J, Saw, D Jr, Cope, MJ, Gibson, F, Kendrick–Jones, J, Steel, KP, Brown, SD 1997Mutation analysis of the mouse myosin VIIA deafness gene.Genes Funct1191203PubMed

30.

Noben–Trauth, K, Zheng, QY, Johnson, KR, Nishina, PM 1997mdfw: a deafness susceptibility locus that interacts with deaf waddler (dfw).Genomics44266272PubMed

31.

Ohlemiller, KK, McFadden, SL, Ding, DL, Flood, DG, Reaume, AG, Hoffman, EK, Scott, RW, Wright, JS, Putcha, GV, Salvi, RJ 1999Targeted deletion of the cytosolic Cu/Zn-superoxide dismutase gene (Sod1) increases susceptibility to noise-induced hearing loss.Audiol. Neurootol4237246CrossRefPubMed

32.

Ohlemiller, KK, McFadden, SL, Ding, DL, Lear, PM, Ho, YS 2000Targeted mutation of the gene for cellular glutathione peroxidase (Gpx1) increases noise-induced hearing loss in mice.J. Assoc. Res. Otolaryngol1243254PubMed

33.

Prezant, TR, Agapian, JV, Bohlman, MC, Bu, X, Oztas, S, Qiu, WQ, Arnos, KS, Cortopassi, GA, Jaber, L, Rotter, JI, Shohat, M, Fischel–Ghodsian, N 1993Mitochondrial ribosomal RNA mutation associated with both antibiotic-induced and non-syndromic deafness.Nat. Genet4289294PubMed

34.

Richardson, GP, Russell, IJ 1991Cochlear cultures as a model system for studying aminoglycoside induced ototoxicity.Hear. Res53293311CrossRefPubMed

35.

Rinchik, EM, Carpenter, DA, Selby, PB 1990A strategy for fine-structure functional analysis of a 6- to 11-centimorgan region of mouse chromosome 7 by high-efficiency mutagenesis.Proc. Natl. Acad. Sci. USA87896900PubMed

36.

Robertson, D 1982Effects of acoustic trauma on stereocilia structure and spiral ganglion cell tuning properties in the guinea pig cochlea.Hear. Res75574CrossRefPubMed

37.

Saunders, JC, Dear, SP, Schneider, ME 1985The anatomical consequences of acoustic injury; A review and tutorial.J. Acoust. Soc. Am78833860PubMed

38.

Self, T, Mahony, M, Fleming, J, Walsh, J, Brown, SD, Steel, KP 1998Shaker-1 mutations reveal roles for myosin VIIA in both development and function of cochlear hair cells.Development125557566PubMed

39.

Shoji, F, Miller, AL, Mitchell, A, Yamasoba, T, Altschuler, RA, Miller, JM 2000Differential protective effects of neurotrophins in the attenuation of noise-induced hair cell loss.Hear. Res146134142CrossRefPubMed

40.

Siemens, J, Kazmierczak, P, Reynolds, A, Sticker, M, Littlewood–Evans, A, Muller, U 2002The Usher syndrome proteins cadherin 23 and harmonin form a complex by means of PDZ-domain interactions.Proc. Natl. Acad. Sci. USA991494614951CrossRefPubMed

41.

Smith, RJ, Berlin, CI, Hejtmancik, JF, Keats, BJ, Kimberling, WJ, Lewis, RA, Moller, CG, Pelias, MZ, Tranebjaerg, L 1994Clinical diagnosis of the Usher syndromes. Usher Syndrome Consortium.Am. J. Med. Genet503238PubMed

42.

Steel, KP, Kros, CJ 2001A genetic approach to understanding auditory function.Nat. Genet27143149CrossRefPubMed

43.

Street, VA, McKee–Johnson, JW, Fonseca, RC, Tempel, BL, Noben–Trauth, K 1998Mutations in a plasma membrane Ca²⁺-ATPase gene cause deafness in deafwaddler mice.Nat. Genet19390394CrossRefPubMed

44.

Wang, Y, Hirose, K, Liberman, MC 2002Dynamics of noise-induced cellular injury and repair in the mouse cochlea.J. Assoc. Res. Otolaryngol3248268CrossRefPubMed

45.

Weil, D, Blanchard, S, Kaplan, J, Guilford, P, Gibson, F, Walsh, J, Mburu, P, Varela, A, Levilliers, J, Weston, MD, Kelly, PM, Kimberling, WJ, Wagenaar, M, Levi–Acobas, F, Larget–Piet, D, Munnich, A, Steel, KP, Brown, SDM, Petit, C 1995Defective myosin VIIA gene responsible for Usher syndrome type 1B.Nature3746061PubMed

46.

Weil, D, Kussel, P, Blanchard, S, Levy, G, Levi–Acobas, F, Drira, M, Ayadi, H, Petit, C 1997The autosomal recessive isolated deafness, DFNB2, and the Usher 1B syndrome are allelic defects of the myosin-VIIA gene.Nat. Genet16191193PubMed

47.

Wilson, SM, Householder, DB, Coppola, V, Tessarollo, L, Fritzsch, B, Lee, EC, Goss, D, Carlson, GA, Copeland, NG, Jenkins, NA 2001Mutations in Cdh23 cause nonsyndromic hearing loss in waltzer mice.Genomics74228233PubMed

48.

Yamoah, EN, Lumpkin, EA, Dumont, RA, Smith, PJ, Hudspeth, AJ, Gillespie, PG 1998Plasma membrane Ca²⁺-ATPase extrudes Ca²⁺ from hair cell stereocilia.J. Neurosci18610624PubMed

49.

Yoshida, N, Hequembourg, SJ, Atencio, CA, Rosowski, JJ, Liberman, MC 2000Acoustic injury in mice: 129/SvEv is exceptionally resistant to noise-induced hearing loss.Hear. Res14197106CrossRefPubMed

50.

Zhai, SQ, Cheng, JC, Wang, JL, Yang, WY, Gu, R, Jiang, SC 2002Protective effect of basic fibroblast growth factor on auditory hair cells after noise exposure.Acta Otolaryngol122370373CrossRefPubMed

51.

Zheng, QY, Johnson, KR 2001Hearing loss associated with the modifier of deaf waddler (mdfw) locus corresponds with age-related hearing loss in 12 inbred strains of mice.Hear. Res1544553CrossRefPubMed

Title: Progressive Hearing Loss and Increased Susceptibility to Noise-Induced Hearing Loss in Mice Carrying a Cdh23 but not a Myo7a Mutation
Authors: Ralph H. Holme
Karen P. Steel
Publication date: 01-03-2004
Publisher: Springer-Verlag
Published in: Journal of the Association for Research in Otolaryngology / Issue 1/2004
Print ISSN: 1525-3961
Electronic ISSN: 1438-7573
DOI: https://doi.org/10.1007/s10162-003-4021-2

2024 ASCO Annual Meeting

Springer Medicine

Progressive Hearing Loss and Increased Susceptibility to Noise-Induced Hearing Loss in Mice Carrying a Cdh23 but not a Myo7a Mutation

Abstract

2024 ASCO Annual Meeting

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 1/2004

Neck Muscle Vibration Alters Visually Perceived Roll in Normals

Across-Site Variation in Detection Thresholds and Maximum Comfortable Loudness Levels for Cochlear Implants

Acrylonitrile Potentiates Noise-Induced Hearing Loss in Rat

Cortical Responses to Cochlear Implant Stimulation: Channel Interactions

Purinergic Modulation of Cochlear Partition Resistance and Its Effect on the Endocochlear Potential in the Guinea Pig

Age-Dependent Changes of Gap Detection in the Mongolian Gerbil (Meriones unguiculatus)