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Original Article

Inner Ear Pathology in the Deafness Mutant Mouse

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Pages 39-47 | Published online: 08 Jul 2009

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Read on this site (10)

M. Cohen, M. Francis, R. Coffey, M. E. Pembrey & L. M. Luxon. (1997) Abnormal Audiograms and Elevated Acoustic Reflex Thresholds in Obligate Carriers of Autosomal Recessive Non-Syndromic Hearing Loss. Acta Oto-Laryngologica 117:3, pages 337-342.
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Satoshi Yonezawa, Yoshinobu Nodasaka, Tsutomu Kamada, Shinobu C. Fujita, Kanefusa Kato, Yasukazu Yamada, Nobuaki Ogasawara & Ryujiro Shoji. (1996) Cochlear Histopathology of the Mutant Bustling Mouse, BUS/Idr. Acta Oto-Laryngologica 116:3, pages 409-416.
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Ken Kitamura, Yasuya Nomura, Masato Yagi, Yasuhiro Yoshikawa & Fumiko Ochikubo. (1991) Morphological Changes of Cochlea in a Strain of New-mutant Mice. Acta Oto-Laryngologica 111:1, pages 61-69.
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Britt-Inger Wenngren & Matti Anniko. (1988) Age-related Auditory Brainstem Response (ABR) Threshold Changes in the Dancer Mouse Mutant. Acta Oto-Laryngologica 106:5-6, pages 386-392.
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Gregory R. Bock, Kathleen Horner & Karen P. Steel. (1985) Electrical Stimulation of the Auditory System in Animals Profoundly Deaf from Birth. Acta Oto-Laryngologica 99:sup421, pages 108-113.
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Karen P. Steel & Gregory R. Bock. (1985) Genetic Factors Affecting Hearing Development. Acta Oto-Laryngologica 99:sup421, pages 48-56.
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Olivier Périer, Jesus Alegria, Monique Buyse, Graziella D'alimonte, Didier Gilson & Willy Serniclaes. (1984) Consequences of Auditory Deprivation in Animals and Humans. Acta Oto-Laryngologica 97:sup412, pages 60-70.
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G. R. Bock & M. P. Frank. (1984) Brainstem Responses in the Quivering Mutant Mouse. Acta Oto-Laryngologica 98:3-4, pages 193-198.
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Olivier Périer, Jesus Alegria, Monique Buyse, Graziella D'alimonte, Didier Gilson & Willy Serniclaes. (1984) Consequences of Auditory Deprivation in Animals and Humans. Acta Oto-Laryngologica 98:sup411, pages 60-70.
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Articles from other publishers (50)

Lili Zheng, Stephen A. Adam, Jaime García‐Anoveros, Brian J. Mitchell & James R. Bartles. (2022) Espin overexpression causes stereocilia defects and provides an anti‐capping effect on actin polymerization. Cytoskeleton.
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Morag A. Lewis, Neil J. Ingham, Jing Chen, Selina Pearson, Francesca Di Domenico, Sohinder Rekhi, Rochelle Allen, Matthew Drake, Annelore Willaert, Victoria Rook, Johanna Pass, Thomas Keane, David J. Adams, Abigail S. Tucker, Jacqueline K. White & Karen P. Steel. (2022) Identification and characterisation of spontaneous mutations causing deafness from a targeted knockout programme. BMC Biology 20:1.
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Zongwei Chen, Shaoyuan Zhu, Kayla Kindig, Shengxuan Wang, Shih-Wei Chou, Robin Woods Davis, Michael R Dercoli, Hannah Weaver, Ruben Stepanyan & Brian M McDermottJrJr. (2020) Tmc proteins are essential for zebrafish hearing where Tmc1 is not obligatory. Human Molecular Genetics 29:12, pages 2004-2021.
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David P. Corey, Nurunisa Akyuz & Jeffrey R. Holt. (2019) Function and Dysfunction of TMC Channels in Inner Ear Hair Cells. Cold Spring Harbor Perspectives in Medicine 9:10, pages a033506.
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Muhammad Umair, Heide Seidel, Ishtiaq Ahmed, Asmat Ullah, Tobias B. Haack, Bader Alhaddad, Abid Jan, Afzal Rafique, Tim M. Strom, Farooq Ahmad, Thomas Meitinger & Wasim Ahmad. (2017) Ellis–van Creveld syndrome and profound deafness resulted by sequence variants in the EVC / EVC2 and TMC1 genes. Journal of Genetics 96:6, pages 1005-1014.
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Ellen Knierim, Esther Gill, Franziska Seifert, Susanne Morales-Gonzalez, Sathya D. Unudurthi, Thomas J. Hund, Werner Stenzel & Markus Schuelke. (2017) A recessive mutation in beta-IV-spectrin (SPTBN4) associates with congenital myopathy, neuropathy, and central deafness. Human Genetics 136:7, pages 903-910.
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Catherine J. Connelly, David K. Ryugo & Michael A. Muniak. (2017) The effect of progressive hearing loss on the morphology of endbulbs of Held and bushy cells. Hearing Research 343, pages 14-33.
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A. Eckhard, A. Dos Santos, W. Liu, M. Bassiouni, H. Arnold, C. Gleiser, B. Hirt, C. Harteneck, M. Müller, H. Rask-Andersen & H. Löwenheim. (2015) Regulation of the perilymphatic–endolymphatic water shunt in the cochlea by membrane translocation of aquaporin-5. Pflügers Archiv - European Journal of Physiology 467:12, pages 2571-2588.
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Hiroshi Nakanishi, Kiyoto Kurima, Yoshiyuki Kawashima & Andrew J. Griffith. (2014) Mutations of TMC1 cause deafness by disrupting mechanoelectrical transduction. Auris Nasus Larynx 41:5, pages 399-408.
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Claire J. Watson & Bruce L. Tempel. (2013) A new Atp2b2 deafwaddler allele, dfwi5, interacts strongly with Cdh23 and other auditory modifiers. Hearing Research 304, pages 41-48.
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A. V. BulankinaT. Moser. (2012) Neural Circuit Development in the Mammalian Cochlea. Physiology 27:2, pages 100-112.
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Morag A Lewis, Elizabeth Quint, Anne M Glazier, Helmut Fuchs, Martin Hrabé De Angelis, Cordelia Langford, Stijn van Dongen, Cei Abreu-Goodger, Matias Piipari, Nick Redshaw, Tamas Dalmay, Miguel Angel Moreno-Pelayo, Anton J Enright & Karen P Steel. (2009) An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice. Nature Genetics 41:5, pages 614-618.
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Gaston Sendin, Anna V. Bulankina, Dietmar Riedel & Tobias Moser. (2007) Maturation of Ribbon Synapses in Hair Cells Is Driven by Thyroid Hormone. The Journal of Neuroscience 27:12, pages 3163-3173.
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S Kitajiri, T Makishima, TB Friedman & AJ Griffith. (2007) A novel mutation at the DFNA36 hearing loss locus reveals a critical function and potential genotype-phenotype correlation for amino acid-572 of TMC1. Clinical Genetics 71:2, pages 148-152.
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Yoshihiro Noguchi, Kiyoto Kurima, Tomoko Makishima, Martin Hrabé de Angelis, Helmut Fuchs, Gregory Frolenkov, Ken Kitamura & Andrew J Griffith. (2006) Multiple Quantitative Trait Loci Modify Cochlear Hair Cell Degeneration in the Beethoven ( Tmc1Bth ) Mouse Model of Progressive Hearing Loss DFNA36 . Genetics 173:4, pages 2111-2119.
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Walter Marcotti, Alexandra Erven, Stuart L. Johnson, Karen P. Steel & Corné J. Kros. (2006) Tmc1 is necessary for normal functional maturation and survival of inner and outer hair cells in the mouse cochlea . The Journal of Physiology 574:3, pages 677-698.
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Lei Song, JoAnn McGee & Edward J. Walsh. (2006) Frequency- and level-dependent changes in auditory brainstem responses (ABRs) in developing mice. The Journal of the Acoustical Society of America 119:4, pages 2242-2257.
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Tomoko Makishima, Kiyoto Kurima, Carmen C. Brewer & Andrew J. Griffith. (2004) Early Onset and Rapid Progression of Dominant Nonsyndromic DFNA36 Hearing Loss. Otology & Neurotology 25:5, pages 714-719.
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Shin-ichiro Kitajiri, Kanehisa Fukumoto, Masaki Hata, Hiroyuki Sasaki, Tatsuya Katsuno, Takayuki Nakagawa, Juichi Ito, Shoichiro Tsukita & Sachiko Tsukita. (2004) Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia. The Journal of Cell Biology 166:4, pages 559-570.
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Lina Mullen, Yan Li & Allen Ryan. 2003. Genetic Hearing Loss. Genetic Hearing Loss.
Kiyoto Kurima, Yandan Yang, Katherine Sorber & Andrew J Griffith. (2003) Characterization of the transmembrane channel-like (TMC) gene family: functional clues from hearing loss and epidermodysplasia verruciformis☆. Genomics 82:3, pages 300-308.
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Glen G. ErnstromMartin Chalfie. (2002) Genetics of Sensory Mechanotransduction. Annual Review of Genetics 36:1, pages 411-453.
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Sarah Vreugde, Alexandra Erven, Corné J. Kros, Walter Marcotti, Helmut Fuchs, Kiyoto Kurima, Edward R. Wilcox, Thomas B. Friedman, Andrew J. Griffith, Rudi Balling, Martin Hrabé de Angelis, Karen B. Avraham & Karen P. Steel. (2002) Beethoven, a mouse model for dominant, progressive hearing loss DFNA36. Nature Genetics 30:3, pages 257-258.
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Kiyoto Kurima, Linda M. Peters, Yandan Yang, Saima Riazuddin, Zubair M. Ahmed, Sadaf Naz, Deidre Arnaud, Stacy Drury, Jianhong Mo, Tomoko Makishima, Manju Ghosh, P.S.N. Menon, Dilip Deshmukh, Carole Oddoux, Harry Ostrer, Shaheen Khan, Sheikh Riazuddin, Prescott L. Deininger, Lori L. Hampton, Susan L. Sullivan, James F. BatteyJr.Jr., Bronya J.B. Keats, Edward R. Wilcox, Thomas B. Friedman & Andrew J. Griffith. (2002) Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell function. Nature Genetics 30:3, pages 277-284.
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Karen P. Steel, Alexandra Erven & Amy E. Kiernan. 2002. Genetics of Auditory Disorders. Genetics of Auditory Disorders 247 296 .
M. Pearce, C.-P. Richter & M.A. Cheatham. (2001) A reconsideration of sound calibration in the mouse. Journal of Neuroscience Methods 106:1, pages 57-67.
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D.A. Scott, S. Drury, R.A. Sundstrom, J. Bishop, R.E. Swiderski, R. Carmi, A. Ramesh, K. Elbedour, C.R. Srikumari Srisailapathy, B.J. Keats, V.C. Sheffield & R.J.H. Smith. (2000) Refining the DFNB7–DFNB11 deafness locus using intragenic polymorphisms in a novel gene, TMEM2. Gene 246:1-2, pages 265-274.
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Anil K. Lalwani, William M. Luxford, Anand N. Mhatre, Ali Attaie, Edward R. Wilcox & Caley M. Castelein. (1999) A New Locus for Nonsyndromic Hereditary Hearing Impairment, DFNA17, Maps to Chromosome 22 and Represents a Gene for Cochleosaccular Degeneration. The American Journal of Human Genetics 64:1, pages 318-323.
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Ana M Viñas, Stacy S Drury, Margaret M DeAngelis, Zhining Den, Jer-Min Huang, Charles I Berlin, Jay D Hunt, Mark A Batzer, Prescott L Deininger & Bronya J.B Keats. (1998) The mouse deafness locus (dn) is associated with an inversion on chromosome 19. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1407:3, pages 257-262.
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Joanne Cable & Karen P Steel. (1998) Combined cochleo-saccular and neuroepithelial abnormalities in the Varitint-waddler-J (VaJ) mouse. Hearing Research 123:1-2, pages 125-136.
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Jer-Min Huang, Charles I Berlin, Shu-Tze Lin & Bronya J.B Keats. (1998) Low intensities and 1.3 ratio produce distortion product otoacoustic emissions which are larger in heterozygous (+/dn) than homozygous (+/+) mice. Hearing Research 117:1-2, pages 24-30.
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Silvia Heid, Rainer Hartmann & Rainer Klinke. (1998) A model for prelingual deafness, the congenitally deaf white cat – population statistics and degenerative changes. Hearing Research 115:1-2, pages 101-112.
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Brian T Faddis, Ruth M Hughes & James D Miller. (1998) Quantitative measures reflect degeneration, but not regeneration, in the deafness mouse organ of Corti. Hearing Research 115:1-2, pages 6-12.
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Rudolf Rübsamen & William R. Lippe. 1998. Development of the Auditory System. Development of the Auditory System 193 270 .
Jer-Min Huang, Matthew K. Money, Charles I. Berlin & Bronya J.B. Keats. (1996) Phenotypic patterns of distortion product otoacoustic emission in inbred and F1 hybrid hearing mouse strains. Hearing Research 98:1-2, pages 18-21.
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Masayuki Sadanaga & Tamotsu Morimitsu. (1995) Development of endocochlear potential and its negative component in mouse cochlea. Hearing Research 89:1-2, pages 155-161.
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Jer-Min Huang, Matthew K. Money, Charles I. Berlin & Bronya J.B. Keats. (1995) Auditory phenotyping of heterozygous sound-responsive (+/dn) and deafness (dn/dn) mice. Hearing Research 88:1-2, pages 61-64.
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B. J. B. Keats, N. Nouri, J.-M. Huang, M. Money, D. B. Webster & C. I. Berlin. (1995) The deafness locus (dn) maps to mouse Chromosome 19. Mammalian Genome 6:1, pages 8-10.
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Jeffrey P. Kirsch, Matthew K. Money & Douglas B. Webster. (1993) Mice heterozygous for the deafness gene have normal auditory thresholds. Hearing Research 67:1-2, pages 51-54.
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Günter Ehret & Raymond Romand. (1992) Development of tone response thresholds, latencies and tuning in the mouse inferior colliculus. Developmental Brain Research 67:2, pages 317-326.
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Dianne Durham, Edwin W Rubel & Karen P Steel. (1989) Cochlear ablation in deafness mutant mice: 2-deoxyglucose analysis suggests no spontaneous activity of cochlear origin. Hearing Research 43:1, pages 39-46.
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Karen P. Steel, Padma Moorjani & Gregory R. Bock. (1987) Mixed conductive and sensorineural hearing loss in LP/J mice. Hearing Research 28:2-3, pages 227-236.
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Karen P. Steel, Christopher Barkway & Gregory R. Bock. (1987) Strial dysfunction in mice with cochleo-saccular abnormalities. Hearing Research 27:1, pages 11-26.
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Karen P. Steel & Gregory R. Bock. (1984) Electrically-evoked responses in animals with progressive spiral ganglion degeneration. Hearing Research 15:1, pages 59-67.
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K.C. Horner & G.R. Bock. (1984) Inferior colliculus single unit responses to peripheral electrical stimulation in normal and congenitally deaf mice. Developmental Brain Research 15:1, pages 33-43.
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