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Deafness and permanently reduced potassium channel gene expression and function in hypothyroid Pit1dw mutants.

Mustapha M, Fang Q, Gong TW, Dolan DF, Raphael Y, Camper SA, Duncan RK.

J Neurosci. 2009 Jan 28;29(4):1212-23. doi: 10.1523/JNEUROSCI.4957-08.2009.


Genetic background of Prop1(df) mutants provides remarkable protection against hypothyroidism-induced hearing impairment.

Fang Q, Giordimaina AM, Dolan DF, Camper SA, Mustapha M.

J Assoc Res Otolaryngol. 2012 Apr;13(2):173-84. doi: 10.1007/s10162-011-0302-3. Epub 2011 Dec 6.


Autonomous functions of murine thyroid hormone receptor TRα and TRβ in cochlear hair cells.

Dettling J, Franz C, Zimmermann U, Lee SC, Bress A, Brandt N, Feil R, Pfister M, Engel J, Flamant F, Rüttiger L, Knipper M.

Mol Cell Endocrinol. 2014 Jan 25;382(1):26-37. doi: 10.1016/j.mce.2013.08.025. Epub 2013 Sep 6.


Dietary thyroid hormone replacement ameliorates hearing deficits in hypothyroid mice.

Karolyi IJ, Dootz GA, Halsey K, Beyer L, Probst FJ, Johnson KR, Parlow AF, Raphael Y, Dolan DF, Camper SA.

Mamm Genome. 2007 Aug;18(8):596-608. Epub 2007 Sep 22.


Distortion product otoacoustic emissions and outer hair cell defects in the hyt/hyt mutant mouse.

Li D, Henley CM, O'Malley BW Jr.

Hear Res. 1999 Dec;138(1-2):65-72.


Deafness in TRbeta mutants is caused by malformation of the tectorial membrane.

Winter H, Rüttiger L, Müller M, Kuhn S, Brandt N, Zimmermann U, Hirt B, Bress A, Sausbier M, Conscience A, Flamant F, Tian Y, Zuo J, Pfister M, Ruth P, Löwenheim H, Samarut J, Engel J, Knipper M.

J Neurosci. 2009 Feb 25;29(8):2581-7. doi: 10.1523/JNEUROSCI.3557-08.2009.


Hearing dysfunction in heterozygous Mitf(Mi-wh) /+ mice, a model for Waardenburg syndrome type 2 and Tietz syndrome.

Ni C, Zhang D, Beyer LA, Halsey KE, Fukui H, Raphael Y, Dolan DF, Hornyak TJ.

Pigment Cell Melanoma Res. 2013 Jan;26(1):78-87. doi: 10.1111/pcmr.12030. Epub 2012 Nov 16.


Loss of the tectorial membrane protein CEACAM16 enhances spontaneous, stimulus-frequency, and transiently evoked otoacoustic emissions.

Cheatham MA, Goodyear RJ, Homma K, Legan PK, Korchagina J, Naskar S, Siegel JH, Dallos P, Zheng J, Richardson GP.

J Neurosci. 2014 Jul 30;34(31):10325-38. doi: 10.1523/JNEUROSCI.1256-14.2014.


Spatiotemporal loss of K+ transport proteins in the developing cochlear lateral wall of guinea pigs with hereditary deafness.

Jin Z, Ulfendahl M, Järlebark L.

Eur J Neurosci. 2008 Jan;27(1):145-54. Epub 2007 Dec 17.


Degeneration of sensory outer hair cells following pharmacological blockade of cochlear KCNQ channels in the adult guinea pig.

Nouvian R, Ruel J, Wang J, Guitton MJ, Pujol R, Puel JL.

Eur J Neurosci. 2003 Jun;17(12):2553-62.


Mice deficient in H+-ATPase a4 subunit have severe hearing impairment associated with enlarged endolymphatic compartments within the inner ear.

Lorente-Cánovas B, Ingham N, Norgett EE, Golder ZJ, Karet Frankl FE, Steel KP.

Dis Model Mech. 2013 Mar;6(2):434-42. doi: 10.1242/dmm.010645. Epub 2012 Oct 12.


Deafness in LIMP2-deficient mice due to early loss of the potassium channel KCNQ1/KCNE1 in marginal cells of the stria vascularis.

Knipper M, Claussen C, Rüttiger L, Zimmermann U, Lüllmann-Rauch R, Eskelinen EL, Schröder J, Schwake M, Saftig P.

J Physiol. 2006 Oct 1;576(Pt 1):73-86. Epub 2006 Aug 10.


Compromised potassium recycling in the cochlea contributes to conservation of endocochlear potential in a mouse model of age-related hearing loss.

Yang H, Xiong H, Huang Q, Pang J, Zheng X, Chen L, Yu R, Zheng Y.

Neurosci Lett. 2013 Oct 25;555:97-101. doi: 10.1016/j.neulet.2013.09.028. Epub 2013 Sep 20.


Thyroid hormone receptors TRalpha1 and TRbeta differentially regulate gene expression of Kcnq4 and prestin during final differentiation of outer hair cells.

Winter H, Braig C, Zimmermann U, Geisler HS, Fränzer JT, Weber T, Ley M, Engel J, Knirsch M, Bauer K, Christ S, Walsh EJ, McGee J, Köpschall I, Rohbock K, Knipper M.

J Cell Sci. 2006 Jul 15;119(Pt 14):2975-84. Epub 2006 Jun 27.


Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness.

Kharkovets T, Dedek K, Maier H, Schweizer M, Khimich D, Nouvian R, Vardanyan V, Leuwer R, Moser T, Jentsch TJ.

EMBO J. 2006 Feb 8;25(3):642-52. Epub 2006 Jan 26.


Retardation of cochlear maturation and impaired hair cell function caused by deletion of all known thyroid hormone receptors.

Rusch A, Ng L, Goodyear R, Oliver D, Lisoukov I, Vennstrom B, Richardson G, Kelley MW, Forrest D.

J Neurosci. 2001 Dec 15;21(24):9792-800.


Deafness in Claudin 11-null mice reveals the critical contribution of basal cell tight junctions to stria vascularis function.

Gow A, Davies C, Southwood CM, Frolenkov G, Chrustowski M, Ng L, Yamauchi D, Marcus DC, Kachar B.

J Neurosci. 2004 Aug 11;24(32):7051-62.


Loss of KCNJ10 protein expression abolishes endocochlear potential and causes deafness in Pendred syndrome mouse model.

Wangemann P, Itza EM, Albrecht B, Wu T, Jabba SV, Maganti RJ, Lee JH, Everett LA, Wall SM, Royaux IE, Green ED, Marcus DC.

BMC Med. 2004 Aug 20;2:30.


A deafness mutation isolates a second role for the tectorial membrane in hearing.

Legan PK, Lukashkina VA, Goodyear RJ, Lukashkin AN, Verhoeven K, Van Camp G, Russell IJ, Richardson GP.

Nat Neurosci. 2005 Aug;8(8):1035-42. Epub 2005 Jul 3.

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