Skip to main content Accessibility help
×
Home

Auditory and vestibular hair cell stereocilia: relationship between functionality and inner ear disease

  • R R Ciuman (a1)

Abstract

The stereocilia of the inner ear are unique cellular structures which correlate anatomically with distinct cochlear functions, including mechanoelectrical transduction, cochlear amplification, adaptation, frequency selectivity and tuning. Their function is impaired by inner ear stressors, by various types of hereditary deafness, syndromic hearing loss and inner ear disease (e.g. Ménière's disease). The anatomical and physiological characteristics of stereocilia are discussed in relation to inner ear malfunctions.

Copyright

Corresponding author

Address for correspondence: Dr Raphael R Ciuman, Uranusbogen 15, 45478 Mülheim, Germany E-mail: ciuman.raphael@cityweb.de

References

Hide All
2Wright, A. Dimensions of the cochlear stereocilia in man and the guinea pig. Hear Res 1984;13:8998
3Rzadzinska, AK, Schneider, ME, Davies, C, Riordan, GP, Kachar, B. An actin molecular treadmill and myosins maintain stereocilia functional architecture and self-renewal. J Cell Biol 2004;164:887–97
4Schneider, ME, Beyantseva, IA, Azevedo, RB, Kachar, B. Rapid renewal of auditory hair bundles. Nature 2002;418:837–8
5Pack, AK, Slepecky, NB. Cytoskeletal and calcium-binding proteins in the mammalian organ of Corti: cell type-specific proteins displaying longitudinal and radial gradients. Hear Res 1995;91:119–35
6DeRosier, DJ, Tilney, LG. The structure of the cuticular plate, an in vivo actin gel. J Cell Biol 1989;109:2853–67
7Slepecky, N, Chamberlain, SC. Immunoelectron microscopic and immunofluorescent localization of cytoskeletal and muscle-like contractile proteins in inner ear sensory hair cells. Hear Res 1985;20:245–60
8Hu, BH, Henderson, D. Changes in F-actin labelling in the outer hair cell and the Deiters cell in the chinchilla cochlea following noise exposure. Hear Res 1997;110:209–18
9Lapeyre, P, Guilhaume, A, Cazals, Y. Differences in hair bundles associated with type I and type II hair cells of the guinea pig saccule. Acta Otolaryngol 1992;112:635–42
10Morita, I, Komatsuzaki, A, Tatsuoka, H. The morphological differences of stereocilia and cuticular plates between type I and type II hair cells of human vestibular sensory epithelia. ORL J Otorhinolaryngol Relat Spec 1997;59:193–7
11Rowe, MH, Peterson, EH. Quantitative analysis of stereociliary arrays on vestibular hair cells. Hear Res 2004;190:1024
12Bagger-Sjoback, D, Takumida, M. Geometrical array of the vestibular sensory hair bundle. Acta Otolaryngol 1988;106:393403
13Ernston, S, Smith, CA. Stereo-kinociliar bonds in mammalian vestibular organs. Acta Otolaryngol 1986;101:395402
14Ross, D, Komorowski, TE, Rogers, CM, Pote, KG, Donovan, KM. Macular suprastructure, stereociliary bonding and kinociliary/stereociliary coupling in rat utricular macula. Acta Otolaryngol 1987;104:5665
15Takumida, M. Functional morphology of the crista ampullaris: with special interests in sensory hairs and cupula: a review. Biol Sci Space 2001;15:356–8
16Raphael, Y, Athey, BD, Wang, Y, Lee, MK, Altschuler, RA. F-actin, tubulin and spectrin in the organ of Corti: comparative distribution in different cell types and mammalian species. Hear Res 1994;76:173–87
17Steyger, PS, Furness, DN, Hackney, CM, Richardson, GP. Tubulin and microtubules in cochlear hair cells: comparative immunocytochemistry and ultrastructure. Hear Res 1989;42:116
18Sobkowicz, HM, Slapnick, SM, August, BK. The kinocilium of auditory hair cells and evidence for its morphogenetic role during the regeneration of stereocilia and cuticular plates. J Neurocytol 1995;24:633–53
19Flock, A, Flock, B, Murray, E. Studies on the sensory hairs of receptor cells in the inner ear. Acta Otolaryngol 1977;83:8591
20Vater, M, Lenoir, M, Pujol, R. Development of the organ of Corti in horseshoe bats: scanning and transmission electron microscopy. J Comp Neurol 1997;377:520–34
21Raphael, Y, Lenoir, R, Wroblewski, R, Pujol, R. The sensory epithelium and its innervation in the mole rat cochlea. J Comp Neurol 1991;314:367–82
22Wright, A. Scanning electron microscopy of the human cochlea – the organ of Corti. Arch Otorhinolaryngol 1981;230:1119
23Lim, DJ. Cochlear anatomy related to cochlear micromechanics. A review. J Acoust Soc Am 1980;67:1686–95
24Gu, ZP, Goodwen, J. Observation on Corti's organ of entire cochlea in the guinea pig by scanning electron microscopy. Chin Med J 1989;102:251–6
25Santi, PA, Anderson, CB. A newly identified surface coat on cochlear hair cells. Hear Res 1987;27:4765
26Valk, WL, Oei, ML, Segenhout, JM, Dijk, F, Stokroos, I, Albers, FW. The glycocalyx and stereociliary interconnections of the vestibular sensory epithelia of the guinea pig. A freeze-fracture, low-voltage cryo-SEM, SEM and TEM study. ORL J Otorhinolaryngol Relat Spec 2002;64:242–6
27Takumida, M, Wersall, J, Bagger-Sjoback, D. Stereociliary glycocalyx and interconnections in the guinea pig vestibular organs. Acta Otolaryngol 1988;106:130–9
28Takumida, M, Wersäll, J, Bagger-Sjöbäck, D, Harada, Y. Observation of the glycocalyx of the organ of Corti: an investigation by electron microscopy in the normal and gentamicin treated guinea pig. J Laryngol Otol 1989;103:133–6
29Takumida, M, Harada, Y, Wersäll, J, Bagger-Sjöbäck, D. The glycocalyx of inner ear sensory and supporting cells. Acta Otolaryngol Suppl 1988;458:84–9
30Prieto, JJ, Merchan, JA. Regional specialization of the cell coat in the hair cells of the organ of Corti. Hear Res 1987;31:223–7
31Prieto, JJ, Merchan, JA. Tannic acid staining of the cell coat of the organ of Corti. Hear Res 1986;24:237–41
32Gil-Loyzaga, P, Bueno, AM, Broto, JP, Pérez, AM. Effects of perinatal hypothyroidism in the carbohydrate composition of cochlear tectorial membrane. Hear Res 1990;45:151–5
33Nayak, GN, Ratnayaka, HSK, Goodyear, RJ, Richardson, GP. Development of the hair bundle and mechanotransduction. Int J Dev Biol 2007;51:597608
34Goodyear, R, Richardson, G. Distribution of the 275 kD hair cell antigen and cell surface specialisations on auditory and vestibular hair bundles in the chicken inner ear. J Comp Neurol 1992;325:243–56
35Goodyear, RJ, Richardson, GP. A novel antigen sensitive to calcium chelation that is associated with the tip links and kinocilial links of sensory hair bundles. J Neurosci 2003;23:4878–87
36Goodyear, RJ, Legan, PK, Wright, MB, Marcotti, W, Oganesian, A, Coats, SA. A receptor-like inositol lipid phosphatase is required for the maturation of developing cochlear hair bundles. J Neurosci 2003;23:9208–19
37Goodyear, RJ, Marcotti, W, Kros, CJ, Richardson, GP. Development and properties of stereociliary link types in hair cells of the mouse cochlea. J Comp Neurol 2005;485:7585
38Osborne, MP, Comis, SD. High resolution scanning electron microscopy of stereocilia in the cochlea of normal, postmortem, and drug-treated guinea pigs. J Electron Microsc Tech 1990;15:245–60
39Ahmed, ZM, Goodyear, R, Riazuddin, S, Lagziel, A, Legan, PK, Behra, M et al. The tip-link, a protein associated with the transduction complex of sensory hair cells, is protocadherin-15. J Neurosci 2006;26:7022–34
40Siemens, J, Lillo, C, Dumont, RA, Reynolds, A, Williams, DS, Gillespie, PG et al. Cadherin 23 is a component of the tip link in hair-cell stereocilia. Nature 2004;428:950–5
41Watson, GM, Mire, P. A comparison of hair bundle mechanoreceptors in sea anemones and vertebrate systems. Curr Top Dev Biol 1999;43:5184
42Osborne, MP, Comis, SD. Action of elastase, collagenase and other enzymes upon linkages between stereocilia in the guinea-pig cochlea. Acta Otolaryngol 1990;110:3745
43Katori, Y, Hackney, CM, Furness, DN. Immunoreactivity of sensory hair bundles of the guinea-pig cochlea to antibodies against elastin and keratan sulphate. Cell Tissue Res 1996;284:473–9
44Pickles, JO, Comis, SD, Osborne, MP. The effect of chronic application of kanamycin on stereocilia and their tip links in hair cells of the guinea pig cochlea. Hear Res 1987;28:237–44
45Tsuprun, V, Schachern, PA, Cureoglu, S, Paparella, M. Structure of the stereocilia side links and morphology of auditory hair bundle in relation to noise in the chinchilla. J Neurocytol 2003;32:1117–28
46Pickles, JO, Osborne, MP, Comis, SD. Vulnerability of tip links between stereocilia to acoustic trauma in the guinea pig. Hear Res 1987;25:173–83
47Tsuprun, V, Santi, P. Structure of outer hair cell stereocilia side and attachment links in the chinchilla cochlea. J Histochem Cytochem 2002;50:493502
48Mermall, V, Post, PL, Mooseker, MS. Unconventional myosins in cell movement, membrane traffic, and signal transduction. Science 1998;279:527–33
49Gillespie, PG, Cyr, JL. Myosin-1c, the hair cell's adaptation motor. Ann Rev Physiol 2004;66:521–45
50Cyr, JL, Dumont, RA, Gillespie, PG. Myosin-1c interacts with hair-cell receptors through its calmodulin-binding IQ domains. J Neurosci 2002;22:2487–95
51Gillespie, PG. Myosin I and adaptation of mechanical transduction by the inner ear. Philos Trans R Soc Lond B Biol Sci 2004;359:1945–51
52Metcalf, AB. Immunolocalization of myosin I beta in the hair cell's hair bundle. Cell Motil Cytoskeleton 1998;39:159–65
53Self, T, Sobe, T, Copeland, NG, Jenkins, NA, Avraham, KB, Steel, KP. Role of myosin VI in the differentiation of cochlear hair cells. Dev Biol 1999;214:331–41
54Brown, SD, Hardisty-Hughes, RE, Mburu, P. Quiet as a mouse: dissecting the molecular and genetic basis of hearing. Nat Rev Genet 2008;9:277–90
55Kussel-Andermann, P, El-Amraoui, A, Safieddine, S, Nouaille, S, Perfettini, I, Lecuit, M et al. Vezatin, a novel transmembrane protein, bridges myosin VIIa to the cadherin-catenins complex. Embo J 2000;19:6020–9
56Kros, CJ, Marcotti, W, Van, Netten, Self, TJ, Libby, RT, Brown, SD et al. Reduced climbing and increased slipping adaptation in cochlear hair cells of mice with Myo 7a mutations. Nat Neurosci 2002;5:34
57Redowicz, JA. Myosins and pathology: genetics and biology. Acta Biochim Pol 2002;49:789804
58Salles, FT, Merritt, RC Jr, Manor, U, Dougherty, GW, Sousa, AD, Moore, JE et al. Myosin IIIa boosts elongation of stereocilia by transporting espin 1 to the plus ends of actin filaments. Nat Cell Biol 2009;11:443–50
59Anderson, DW, Probst, FJ, Belyantseva, IA, Fridell, RA, Beyer, L, Martin, DM et al. The motor and tail regions of myosin XV are critical for normal structure and function of auditory and vestibular hair cells. Hum Mol Genet 2000;9:1729–38
60Lin, HW, Schneider, ME, Kachar, B. When size matters: the dynamic regulation of stereocilia lengths. Curr Opin Cell Biol 2005;17:5561
61Belyantseva, IA, Boger, ET, Friedman, TB. Myosin XVa localizes to the tips of inner ear sensory cell stereocilia and is essential for staircase formation of the hair bundle. Proc Natl Acad Sci U S A 2003;100:13958–63
62Belyantseva, IA, Boger, ET, Naz, S, Frolenkov, GI, Sellers, JR, Ahmed, ZM et al. Myosin-XVa is required for tip localization of whirlin and differential elongation of hair-cell stereocilia. Nat Cell Biol 2005;7:148–56
63Siemens, J, Kazmierczak, P, Reynolds, A, Sticker, M, Littlewood-Evans, A, Muller, U. The Usher syndrome proteins cadherin 23 and harmonin form a complex by means of PDZ-domain interactions. Proc Natl Acad Sci U S A 2002;99:14946–51
64Boeda, B, El-Amraoui, A, Bahloul, A, Goodyear, R, Daviet, L, Blanchard, S et al. Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle. EMBO J 2002;21:6689–99
65Weil, D, El-Amraoui, A, Masmoudi, S, Mustapha, M, Kikkawa, Y, Laine, S et al. Usher syndrome type IG (USHIG) is caused by mutations in the gene encoding SANS, a protein that associates with the USHIC protein harmonin. Hum Mol Genet 2003;12:463–71
66Kikkawa, Y, Mburu, P, Morse, S, Kominami, R, Townsend, S, Brown, SD. Mutant analysis reveals whirlin as a dynamic organizer in the growing hair cell stereocilium. Hum Mol Genet 2005;14:391400
67Belyantseva, IA, Labay, Y, Boger, ET, Griffith, AJ, Friedman, TB. Stereocilia: the long and the short of it. Trends Mol Med 2003;9:458–61
68Delprat, B, Michel, V, Goodyear, R, Yamasaki, Y, Michalski, N, El-Amraoui, A et al. Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia and interact directly. Hum Mol Genet 2005;14:401–10
69Kitajiri, S, Fukumoto, K, Hata, M, Sasaki, H, Katsuno, T, Nakagawa, T et al. Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia. J Cell Biol 2004;166:559–70
70Riazuddin, S, Khan, SN, Ahmed, ZM, Ghosh, M, Caution, K, Nazli, S et al. Mutations in TRIOBP, which encodes a putative cytoskeletal-organizing protein, are associated with nonsyndromic recessive deafness. Am J Hum Genet 2006;78:137–43
71Shahin, H, Walsh, T, Sobe, T, AbuSaed, J, AbuRayan, A, Lynch, ED et al. Mutations in a novel isoform of TRIOBP that encodes a filamentous-actin binding protein are responsible for DFNB28 recessive nonsyndromic hearing loss. Am J Hum Genet 2006;78:144–52
72Kitajiri, S, Sakamato, T, Belyantseva, IA, Goodyear, RJ, Stepanyan, R, Fujiwara, I et al. Actin-bundling protein TRIOBP forms resilient rootlets of hair cell stereocilia essential for hearing. Cell 2010;141:786–98
73Hudspeth, AJ. How the ear's works work. Nature 1989;341:397404
74Beurg, M, Fettiplace, R, Nam, JH, Ricci, AJ. Localization of inner hair cell mechanotransducer channels using high speed calcium imaging. Nat Neurosci 2009;12:553–8
75Lumpkin, EA, Hudspeth, AJ. Detection of Ca2+ entry through mechanosensitive channels localizes the site of mechanoelectrical transduction in hair cells. Proc Natl Acad Sci U S A 1995;92:10297–301
76Geleoc, GS, Lennan, GW, Richardson, GP, Kros, CJ. A quantitative comparison of mechanoelectrical transduction in vestibular and auditory hair cells of neonatal mice. Proc Biol Sci 1997;261:611–21
77Howard, J, Roberts, WM, Hudspeth, AJ. Mechanoelectrical transduction by hair cells. Annu Rev Biophys Chem 1988;17:99124
78Martin, P, Mehta, AD, Hudspeth, AJ. Negative hair-bundle stiffness betrays a mechanism for mechanical amplification by the hair cell. Proc Natl Acad Sci U S A 2000;97:12026–31
79Ricci, AJ, Crawford, AC, Fettiplace, R. Mechanisms of active hair bundle motion in auditory hair cells. J Neurosci 2002;22:4452
80Kachar, B, Parakkal, M, Kurc, M, Zhao, Y, Gillepsie, PG. High-resolution structure of hair-cell tip links. Proc Natl Acad Sci U S A 2000;97:13336–41
81Sotomayor, M, Corey, DP, Schulten, K. In search of the hair-cell gating spring elastic properties of ankyrin and cadherin repeats. Structure 2005;13:669–82
82Fettiplace, R, Ricci, AJ, Hackney, CM. Clues to the cochlear amplifier from the turtle ear. Trends Neurosci 2001;24:169–75
83Sotomayor, M, Weihofen, WA, Gaudet, R, Corey, DP. Structural determinants of cadherin-23 function in hearing and deafness. Neuron 2010;66:85100
84Brownell, WE, Bader, CR, Bertrand, D, deRibaupierre, Y. Evoked mechanical responses of isolated outer hair cells. Science 1985;227:641–54
85Bekesy, G. Experiments in Hearing. New York: McGraw Hill, 1960
86Shatz, LF. The effect of hair bundle shape on hair bundle hydrodynamics of inner hair cells at low and high frequencies. Hear Res 2000;141:3950
87Ehret, G. Stiffness gradient along the basilar membrane as a basis for spatial frequency analysis within the cochlea. J Acoust Soc Am 1978;64:1723–6
88Flock, A, Strelioff, D. Graded and nonlinear mechanical properties of sensory hairs in the mammalian hearing organ. Nature 1984;10:597–9
89Robles, L, Ruggero, MA. Mechanics of the mammalian cochlea. Phys Rev 2001;81:1305–52
90Ruggero, MA, Rich, NC, Recio, A, Narayan, SS, Robles, L. Basilar-membrane responses to tones at the base of the chinchilla cochlea. J Acoust Soc Am 1997;101:2151–63
91Cooper, NP, Rhode, WS. Mechanical responses to two-tone distortion products in the apical and basal turns of the mammalian cochlea. J Neurophysiol 1997;78:261–70
92Johnstone, BM, Patuzzi, R, Yates, G. Basilar membrane measurements and the travelling wave. Hear Res 1986;22:147–54
93Camalet, S, Duke, T, Julicher, F, Prost, J. Auditory sensitivity provided by self-tuned critical oscillations of hair cells. Proc Natl Acad Sci U S A 2000;97:3183–8
94Ricci, AJ, Fettiplace, R. Calcium permeation of the turtle hair cell mechanotransducer channel and its relation to the composition of endolymph. J Physiol 1998;506:159–73
95Vilfan, A, Duke, T. Two adaptation processes in auditory hair cells together can provide an active amplifier. Biophys J 2003;85:191203
96Chan, DK, Hudspeth, AJ. Ca2+ current-driven nonlinear amplification by the mammalian cochlea in vitro. Nat Neurosci 2005;8:149–55
97Kennedy, HJ, Crawford, AC, Fettiplace, R. Force generation by mammalian hair bundles supports a role in cochlear amplification. Nature 2005;433:880–3
98Fettiplace, R, Ricci, AJ. Adaptation in auditory hair cells. Curr Opin Neurobiol 2003;13:446–51
99Martin, P, Hudspeth, AJ. Compressive nonlinearity in the hair bundle's active response to mechanical stimulation. Proc Natl Acad Sci U S A 2001;98:14386–91
100Howard, J, Hudspeth, AJ. Compliance of the hair bundle associated with gating of mechanoelectrical transduction by the bullfrog's saccular hair cell. Proc Natl Acad Sci U S A 1987;84:3064–8
101Eatock, RA. Adaptation in hair cells. Annu Rev Neurosci 2000;23:285314
102Wu, YC, Ricci, AJ, Fettiplace, R. Two components of transducer adaptation in auditory hair cells. J Neurophysiol 1999;82:2171–81
103Assad, JA, Corey, DP. An active motor model for adaptation by vertebrate hair cells. J Neurosci 1992;12:3291–309
104Gillespie, PG, Corey, DP. Myosin and adaptation by hair cells. Neuron 1997;19:955–8
105Assad, JA, Hacohen, N, Corey, DP. Voltage dependence of adaptation and active bundle movement in bullfrog saccular hair cells. Proc Natl Acad Sci U S A 1989;86:2918–22
106Eatock, RA, Corey, DP, Hudspeth, AJ. Adaptation of mechanoelectrical transduction in hair cells of the bullfrog's sacculus. J Neurosci 1987;7:2821–36
107Kros, CJ, Lennan, GWT, Richardson, GP. Transducer currents and bundle movements in outer hair cells of neonatal mice. In: Flock, AO, Ulfendahl, M, eds. Active Hearing. Oxford: Elsevier, 1995;113–25
108Holt, JR, Gillespie, SK, Provance, DW, Shah, K, Shokat, KM, Corey, DP et al. A chemical-genetic strategy implicates myosin-1c in adaptation by hair cells. Cell 2002;108:371–81
109Holt, JR, Corey, DP, Eatock, RA. Mechanoelectrical transduction and adaptation in hair cells of the mouse utricle, a low-frequency vestibular organ. J Neurosci 1997;17:8739–48
110Crawford, AC, Evans, MG, Fettiplace, R. Activation and adaptation of transducer currents in turtle hair cells. J Physiol 1989;419:405–34
111Ricci, AJ, Wu, YC, Fettiplace, R. The endogenous calcium buffer and the time course of transducer adaptation in auditory hair cells. J Neurosci 1998;18:8261–77
112Lumpkin, EA, Hudspeth, AJ. Regulation of free Ca2+ concentration in hair-cell stereocilia. J Neurosci 1998;18:6300–18
113Slepecky, NB, Ulfendahl, M. Evidence for calcium-binding and calcium-dependent regulatory proteins in sensory cells of the organ of Corti. Hear Res 1993;70:7384
114Jurado, LA, Chockalingam, PS, Jarrett, HW. Apocalmodulin. Physiol Rev 1999;79:661–82
115Caride, AJ, Filoteo, AG, Penheiter, AR, Paszty, K, Enyedi, A, Penniston, JT. Delayed activation of the plasma membrane calcium pump by a sudden increase in Ca2+: fast pumps reside in fast cells. Cell Calcium 2001;30:4957
116Wood, JD, Muchinsky, SJ, Filoteo, AG, Penniston, JT, Tempel, BL. Low endolymph calcium concentrations in deafwaddler2J mice suggest that PMCA2 contributes to endolymph calcium maintenance. Assoc Res Otolaryngol 2004;5:99110
117Lopez, I, Ishiyama, G, Ishiyama, A, Jen, JC, Liu, F, Balow, RW. Differential subcellular immunolocalization of voltage-gated calcium channel alpha1 subunits in the chinchilla cristae ampullaris. Neuroscience 1999;92:773–82
118Davis, RR, Kozel, P, Erway, LC. Genetic influences in individual susceptibility to noise: a review. Noise Health 2003;5:1928
119Takumida, M, Fredelius, L, Bagger-Sjoback, D, Harada, Y, Wersall, J. Effect of acoustic overstimulation on the glycocalyx and the ciliary interconnections in the organ of Corti: high resolution scanning electron microscopic investigation. J Laryngol Otol 1989;103:1125–9
120Clark, JA, Pickles, JO. The effects of moderate and low levels of acoustic overstimulation on stereocilia and their tip links in the guinea pig. Hear Res 1996;99:119–28
121Nordmann, AS, Bohne, BA, Harding, GW. Histopathological differences between temporary and permanent threshold shift. Hear Res 2000;139:1330
122Gao, WY, Ding, DL, Zheng, XY, Ruan, FM, Liu, YJ. A comparison of changes in the stereocilia between temporary and permanent hearing losses in acoustic trauma. Hear Res 1992;62:2741
123Slepecky, N, Hamernik, R, Henderson, D, Coling, D. Correlation of audiometric data changes in cochlear hair cell stereocilia resulting from impulse noise trauma. Acta Otolaryngol 1982;93:329–40
124Patuzzi, R. Non-linear aspects of outer hair cell transduction and the temporary threshold shifts after acoustic trauma. Audiol Neurootol 2002;7:1720
125Canlon, B. The effect of acoustic trauma on the tectorial membrane, stereocilia, and hearing sensitivity: possible mechanisms underlying damage, recovery, and protection. Scand Audiol Suppl 1988;27:145
126Saunders, JC, Canlon, B, Flock, A. Growth of threshold shift in hair-cell stereocilia following overstimulation. Hear Res 1986;23:245–55
127Wang, JC, Raybould, NP, Luo, L, Ryan, AF, Cannell, MB, Thorne, PR et al. Noise induces up-regulation of P2X2 receptor subunit of ATP-gated ion channels in the rat cochlea. Neuroreport 2003;14:817–23
128Housley, GD, Kanjhan, R, Raybould, NP, Greenwood, D, Salih, SG, Järlebark, L et al. Expression of the P2X2 receptor subunit of the ATP-gated ion channel in the cochlea: implications for sound transduction and auditory neurotransmission. J Neurosci 1999;19:83778388
129Takumida, M, Urquiza, R, Bagger-Sjoback, D, Wersall, J. Effect of gentamicin on the carbohydrates of the vestibular end organs: an investigation by the use of FITC-lectins. J Laryngol Otol 1989;103:357–62
130Takumida, M, Bagger-Sjoback, D, Wersall, J, Harada, Y. The effect of gentamicin on the glycocalyx and the ciliary interconnections in vestibular sensory cells: a high resolution scanning electron microscopic investigation. Hear Res 1989;37:163–70
131Richardson, GP, Forge, A, Kros, CJ, Fleming, J, Brown, SD, Steel, KP. Myosin VIIa is required for aminoglycoside accumulation in cochlear hair cells. J Neurosci 1997;17:9506–19
132Rydmarker, S, Horner, KC. Atrophy of outer hair cell stereocilia and hearing loss in hydropic cochleae. Hear Res 1991;53:113–22
133Ruding, PR, Veldman, JE, Berendsen, W, Huizing, EH. Scanning electron microscopy of hair cells, stereocilia and cross-linkage in experimentally induced endolymphatic hydrops. Eur Arch Otorhinolaryngol 1991;248:313–18
134Van Benthem, PP, De Groot, JC, Albers, FW, Veldman, JE, Huizing, EH. Structure and composition of stereocilia cross-links in normal and hydropic cochleas of the guinea pig. Eur Arch Otorhinolaryngol 1993;250:73–7
135Van Benthem, PP, Albers, FW, De Groot, , Veldman, JE, Huizing, EH. Glycocalyx heterogeneity in normal and hydropic cochleas of the guinea pig. Acta Otolaryngol 1992;112:976–84
136Schwaber, MK. Medical evaluation of tinnitus. Otolaryngol Clin North Am 2003;36:287–92
137Zhou, Y, Zhai, S, Yang, W. The protective effects of ciliary neurotrophic factor on inner ear damage induced by intensive impulse noise [in Chinese]. Zhonghua Er Bi Yan Hou Ke Za Zhi 1999;34:150–3
138Kang, S, He, C, Shi, X. Protective effect of ciliary neurotrophic factor against the ototoxicity of gentamicin in guinea pigs [in Chinese]. Zhuongua Ying Yong Sheng Li Xue Za Zhi 1997;13:124–7
139Zine, A, De Ribaupierre, F. Tissue-specific levels and cellular distribution of epidermal growth factor receptors within control and neomycin-damaged neonatal rat organ of Corti. J Neurobiol 1999;38:313–22
140Kimberling, WJ, Moller, C. Clinical and molecular genetics of Usher syndrome. J Am Acad Audiol 1995;6:6372
141Bougham, JA, Vernon, M, Shaver, KA. Usher syndrome: definition and estimate of prevalence from two high risk populations. J Chron Dis 1983;36:595603
142Vernon, M. Usher syndrome-deafness and progressive blindness. Clinical cases, prevention, theory and literature survey. J Chron Dis 1969;22:133–51
143Otterstede, CR, Spandau, U, Blankenagel, A, Kimberling, WJ, Reisser, C. A new clinical classification for Usher's syndrome based on a new subtype of Usher's syndrome type 1. Laryngoscope 2001;111:84–6
144Auffarth, GU, Tetz, MR, Krastel, H, Blanckenagel, A, Volcker, HE. Complicated cataracts in various forms of retinitis pigmentosa. Type and incidence [in German]. Ophthalmologe 1997;94:642–6
145Loundon, N, Marlin, S, Busquet, D, Denoyelle, F, Roger, G, Renaud, F et al. Usher syndrome and cochlear implantation. Otol Neurotol 2003;24:216–21
146Smith, RJH, Berlin, CI, Hejtmack, JF, Keats, BJ, Kimberling, WJ, Lewis, RA et al. Clinical diagnosis of the Usher syndromes. Usher syndrome consortium. Am J Med Genet 1994;50:32–8
147Moller, CG, Kimberling, WJ, Davenport, SL, Priluck, I, White, V, Biscone-Halterman, K et al. Usher syndrome: an otoneurologic study. Laryngoscope 1989;99:73–9
148Adato, A, Michel, V, Kikkawa, Y, Reiners, J, Alagramam, KN, Weil, D et al. Interactions in the network of Usher syndrome type 1 proteins. Hum Mol Genet 2005;14:347–56
149Keats, BJ, Corey, DP. The usher syndromes. Am J Med Genet 1999;89:158–66
150Bolz, H, Bolz, SS, Schade, G, Kothe, C, Mohrmann, G, Hess, M et al. Impaired calmodulin binding of myosin-7A causes autosomal dominant hearing loss (DFNA11). Hum Mutat 2004;24:274–5
151Wilson, SM, Householder, DB, Coppola, V, Tessarollo, L, Fritzsch, B, Lee, EC et al. Mutations in Cdh23 cause nonsyndromic hearing loss in waltzer mice. Genomics 2001;74:228–33
152Bolz, H, Von Brederlow, B, Ramirez, A, Bryda, EC, Kutsche, K, Nothwang, HG et al. Mutation of VDH23, encoding a new member of the cadherin gene family, causes Usher syndrome type 1D. Nat Genetics 2001;27:108–12
153Ahmed, ZM, Riazuddin, S, Ahmad, J, Bernstein, SL, Guo, Y, Sabar, MF et al. PCDH15 is expressed in the neurosensory epithelium of the eye and ear mutant alleles are responsible for both USH1F and DFNB23. Hum Mol Genet 2003;12:3215–23
154Liang, Y, Wang, A, Belyantseva, IA, Anderson, DW, Probst, FJ, Barber, TD et al. Characterization of the human and mouse unconventional myosin XV genes responsible for hereditary deafness DFNB3 and shaker 2. Genomics 1999;61:243–58
155Zhu, M, Yang, T, Wei, S, DeWan, AT, Morell, RJ, Elfenbein, JL et al. Mutations in the gamma-actin gene (ACTG1) are associated with dominant progressive deafness (DFNA20/26). Am J Hum Genet 2003;73:1082–91
156Jovine, L, Park, J, Wassarman, PM. Sequence similarity between stereocilin and otoancorin points to a unified mechanism for mechanotransduction in the mammalian inner ear. BMC Cell Biol 2002;3:28. doi: 10.1186/1471-2121-3-28
157Verpy, E, Masmoudi, S, Zwaenepoel, I, Leibovici, M, Hutchin, TP, Del Castillo, I et al. Mutations in a new gene encoding a protein of the hair bundle cause non-syndromic deafness at the DFNB16 locus. Nat Genet 2001;29:345–9
158Zwaenepoel, I, Mustapha, M, Leibovici, M, Verpy, E, Goodyear, R, Liu, XZ et al. Otoancorin, an inner ear protein restricted to the interface between the apical surface of sensory epithelia and their overlying acellular gels, is defective in autosomal recessive deafness DFNB22. Proc Natl Acad Sci U S A 2002;99:6240–5
159Bearer, EL, Chen, AF, Chen, AH, Li, Z, Mark, HF, Smith, RJ et al. 2E4/kaptin (KPTN) – a candidate gene for the hearing loss locus, DFNA4. Ann Hum Genet 2000;64:189–96
160Bearer, EL, Abraham, MT. 2E4 (kaptin): a novel actin-associated protein from human blood platelets found in lamellipodia and the tips of the stereocilia of the inner ear. Eur J Cell Biol 1999;78:117–26
161Loomis, PA, Zheng, L, Sekerkova, G, Changyaleket, B, Mugnaini, E, Bartles, JR. Espin cross-links cause the elongation of microvillus-type parallel actin bundles in vivo. J Cell Biol 2003;163:1045–55
162Donaudy, F, Zheng, L, Ficarcella, R, Ballana, E, Carella, M, Melchionda, S et al. Espin gene (ESPN) mutations associated with autosomal dominant hearing loss cause defects in microvillar elongation or organization. J Med Genet 2006;43:157–61
163Naz, S, Griffith, AJ, Riazuddin, S, Hampton, LL, Battey, JF Jr, Khan, SN et al. Mutations of ESPN cause autosomal recessive deafness and vestibular dysfunction. J Med Genet 2004;41:591–5

Keywords

Auditory and vestibular hair cell stereocilia: relationship between functionality and inner ear disease

  • R R Ciuman (a1)

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed