1Manor U,Kachar B.Dynamic length regulation of sensory stereocilia[J].Semin Cell Dev Biol,2008,19:502.

2Zhu M,Yang T,Wei S,et al.Mutationsinthe gamma-actin gene(ACTG1)are associated with dominant progressive deaf-ness(DFNA20/26)[J].AmJ Hum Genet2003,73:1082.

3Lynch ED,Lee MK,MorrowJE,et al.Nonsyndromic deaf-ness DFNA1associated with mutation of a human homolog of the Drosophila gene diaphanous[J].Science,1997,278:1315.

4Seipel K,O'Brien SP,Iannotti E,et al.Tara,a novel F-ac-tin binding protein,associates with the Trio guanine nucleo-tide exchange factor and regulates actin cytoskeletal organiza-tion[J].J Cell Sci,2001,114:389.

5Rzadzinska A,Schneider M,Noben-Trauth K,et al.Bal-anced levels of Espin are critical for stereociliary growth and length maintenance[J].Cell Motil Cytoskeleton,2005,62:157.

6Sekerkova G,Zheng L,Loomis PA,et al.Espins and the ac-tin cytoskeleton of hair cell stereocilia and sensory cell mi-crovilli[J].Cell Mol Life Sci,2006,63:2329.

7Kitajiri S,Fukumoto K,Hata M,et al.Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia[J].J Cell Biol,2004,166:559.

8Weil D,Blanchard S,Kaplan J,et al.Defective myosin VII A gene responsible for Usher syndrome type1B[J].Nature,1995,374:60.

9Weil D,Levy G,Sahly I,et al.Human myosin VII Arespon-sible for the Usher1B syndrome:a predicted membrane-as-sociated motor protein expressedin developing sensory epithe-lia[J].Proc Natl Acad Sci US A,1996,93:3232.

10Prosser HM,Rzadzinska AK,Steel KP,et al.Mosaic com-plementation demonstrates a regulatory role for myosin VIIa in actin dynamics of stereocilia[J].Mol Cell Biol,2008,28:1702.

11Watanabe S,Umeki N,Ikebe R,et al.I mpacts of Usher syndrome type IB mutations on human myosin VIIa motor function[J].Biochemistry,2008,47:9505.

12Sweeney HL,Houdusse A.What can myosin VI do in cells[J]-Curr Opin Cell Biol,2007,19:57.

13Hertzano R,Shalit E,Rzadzinska AK,et al.A Myo6muta-tion destroys coordination between the myosin heads,revea-ling newfunctions of myosin VI inthe stereocilia of mammali-aninner ear hair cells[J].PLoS Genet,2008,4:e1000207.

14Wells AL,Lin AW,Chen LQ,et al.Myosin VI is an actin-based motor that moves backwards[J].Nature,1999,401:505.

15Liang Y,Wang A,Belyantseva I A,et al.Characterization of the human and mouse unconventional myosin XV genes re-sponsible for hereditary deafness DFNB3and shaker2[J].Genomics,1999,61:243.

16Delprat B,Michel V,Goodyear R,et al.Myosin XVa and whirlin,two deafness gene products required for hair bundle growth,are located at the stereocilia tips andinteract directly[J].Hum Mol Genet,2005,14:401.

17Schneider ME,Dose AC,Salles FT,et al.A newcompart-ment at stereocilia tips defined by spatial and temporal pat-terns of myosin IIIa expression[J].J Neurosci,2006,26:10243.

18Walsh T,Walsh V,Vreugde S,et al.Fromflies'eyes to our ears:mutations in a human class III myosin cause progressive nonsyndromic hearing loss DFNB30[J].Proc Natl Acad Sci USA,2002,99:7518.

19Mogensen MM,Rzadzinska A,Steel KP.The deaf mouse mutant whirler suggests a role for whirlin in actin filament dynamics and stereocilia development[J].Cell Motil Cy-toskeleton,2007,64:496.

20Modamio-Hoybjor S,Mencia A,Goodyear R,et al.A mu-tationin CCDC50,a gene encoding an effector of epidermal growth factor-mediated cell signaling,causes progressive hearing loss[J].AmJ Hum Genet,2007,80:1076.

21Willecke K,Eiberger J,Degen J,et al.Structural and func-tional diversity of connexin genesinthe mouse and human ge-nome[J].Biol Chem,2002,383:725.

22Forge A,Marziano NK,Casalotti SO,et al.The inner ear contains heteromeric channels composed of cx26and cx30and deafness-related mutationsin cx26have a dominant negative effect on cx30[J].Cell Commun Adhes,2003,10:341.

23Cohen-Sal mon M,Ott T,Michel V,et al.Targeted abla-tion of connexin26in theinner ear epithelial gapjunction net-work causes hearingi mpairment and cell death[J].Curr Biol,2002,12:1106.

24Teubner B,Michel V,Pesch J,et al.Connexin30(GJB6)-deficiency causes severe hearingi mpairment andlack of endo-cochlear potential[J].Hum Mol Genet,2003,12:13.

25Chang Q,Tang W,Ahmad S,et al.Functional studies re-veal new mechanisms for deafness caused by connexin muta-tions[J].Otol Neurotol,2009,30:237.

26Zhao HB,Kikuchi T,Ngezahayo A,et al.Gapjunctions and cochlear homeostasis[J].J Membr Biol,2006,209:177.

27Ansel mi F,Hernandez VH,Crispino G,et al.ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2+signals across the inner ear[J].Proc Natl Acad Sci US A,2008,105:18770.

28Wilcox ER,Burton QL,Naz S,et al.Mutations in the gene encoding tight junction claudin-14cause autosomal recessive deafness DFNB29[J].Cell,2001,104:165.

29Riazuddin S,Ahmed ZM,Fanning AS,et al.Tricellulinis a tight-junction protein necessary for hearing[J].AmJ Hum Genet,2006,79:1040.

30Longo-Guess CM,Gagnon LH,Cook SA,et al.A mis-sense mutationin the previously undescribed gene Tmhs un-derlies deafness in hurry-scurry(hscy)mice[J].Proc Natl Acad Sci USA,2005,102:7894.

31Kazmierczak P,Sakaguchi H,Tokita J,et al.Cadherin23and protocadherin15interact to formtip-link filaments in sensory hair cells[J].Nature,2007,449:87.

32Yoshino T,Sato E,Nakashi ma T,et al.The i mmunohisto-chemical analysis of pendrinin the mouse inner ear[J].Hear Res,2004,195:9.

33Nakaya K,Harbidge DG,Wangemann P,et al.Lack of pen-drin HCO3-transport elevates vestibular endolymphatic[Ca2+]by inhibition of acid-sensitive TRPV5and TRPV6channels[J].AmJ Physiol Renal Physiol,2007,292:F1314.

34Zheng J,Long KB,Matsuda KB,et al.Genomic character-ization and expression of mouse prestin,the motor protein of outer hair cells[J].Mamm Genome,2003,14:87.

35Zheng J,Shen W,He DZ,et al.Prestinis the motor protein of cochlear outer hair cells[J].Nature,2000,405:149.

36Yasunaga S,Grati M,Cohen-Sal mon M,et al.A mutation in OTOF,encoding otoferlin,a FER-1-like protein,cau-ses DFNB9,a nonsyndromic form of deafness[J].Nat Gen-et,1999,21:363.

37Starr A,Picton TW,Sininger Y,et al.Auditory neuropathy[J].Brain,1996,119:741.

38Del mas P,Brown DA.Pathways modulating neural KCNQ/M(Kv7)potassiumchannels[J].Nat Rev Neurosci,2005,6:850.

39Tyson J,Tranebjaerg L,Bell man S,et al.IsK and Kv-LQT1:mutation in either of the two subunits of the slow component of the delayed rectifier potassium channel can cause Jervell and Lange-Nielsen syndrome[J].Hum Mol Genet,1997,6:2179.

40Schulze-Bahr E,Wang Q,Wedekind H,et al.KCNE1mu-tations cause jervell and Lange-Nielsen syndrome[J].Nat Genet,1997,17:267.

41Kharkovets T,Hardelin JP,Safieddine S,et al.KCNQ4,a K+channel mutated in a form of dominant deafness,is ex-pressed in the inner ear and the central auditory pathway[J].Proc Natl Acad Sci US A,2000,97:4333.

42Kharkovets T,Dedek K,Maier H,et al.Mice with altered KCNQ4K+channels i mplicate sensory outer hair cells in hu-man progressive deafness[J].Embo J,2006,25:642.

43Kuri ma K,Peters LM,Yang Y,et al.Dominant and reces-sive deafness caused by mutations of a novel gene,TMC1,required for cochlear hair-cell function[J].Nat Genet,2002,30:277.

44Marcotti W,Erven A,Johnson SL,et al.Tmc1is necessary for normal functional maturation and survival of inner and outer hair cellsinthe mouse cochlea[J].J Physiol,2006,574:677.

45Osman AA,Saito M,Makepeace C,et al.Wolframin ex-pressioninduces novel ion channel activity in endoplasmic re-ticulum membranes and increases intracellular calcium[J].J Biol Chem,2003,278:52755.

46McGuirt WT,Prasad SD,Griffith AJ,et al.Mutations in COL11A2cause non-syndromic hearing loss(DFNA13)[J].Nat Genet,1999,23:413.

47Jovine L,Park J,Wassarman PM.Sequence si milarity be-tween stereocilin and otoancorin points to a unified mechanism for mechanotransduction in the mammalian inner ear[J].BMC Cell Biol,2002,3:28.

48Robertson NG,Resendes BL,Lin JS,et al.Inner ear locali-zation of mRNAand protein products of COCH,mutated in the sensorineural deafness and vestibular disorder,DFNA9[J].Hum Mol Genet,2001,10:2493.

49Del maghani S,del Castillo FJ,Michel V,et al.Mutationsin the gene encoding pejvakin,a newly identified protein of the afferent auditory pathway,cause DFNB59auditory neuropa-thy[J].Nat Genet2006,38:770.

50Weiss S,Gottfried I,Mayrose I,et al.The DFNA15deaf-ness mutation affects POU4F3protein stability,localization,and transcriptional activity[J].Mol Cell Biol,2003,23:7957.

51Wayne S,Robertson NG,DeClau F,et al.Mutations in the transcriptional activator EYA4cause late-onset deafness at the DFNA10locus[J].Hum Mol Genet2001,10:195.

52Peters LM,Anderson DW,Griffith AJ,et al.Mutation of a transcription factor,TFCP2L3,causes progressive autosomal dominant hearing loss,DFNA28[J].Hum Mol Genet2002,11:2877.

53Weston MD,Pierce ML,Rocha-Sanchez S,et al.MicroR-NAgene expression in the mouse inner ear[J].Brain Res,2006,1111:95.

54Lewis MA,Quint E,Glazier AM,et al.An ENU-induced mutation of mi R-96associated with progressive hearingloss in mice[J].Nat Genet,2009,41:614.

55Soukup GA,Fritzsch B,Pierce ML,et al.Residual microR-NAexpression dictates the extent of inner ear development in conditional Dicer knockout mice[J].Dev Biol,2009,328:328.

56Abe S,Katagiri T,Saito-Hisaminato A,et al.Identifica-tion of CRYM as a candidate responsible for nonsyndromic deafness,through cDNA microarray analysis of human co-chlear and vestibular tissues[J].Am J Hum Genet,2003,72:73.

57Guipponi M,Vuagniaux G,Wattenhofer M,et al.The transmembrane serine protease(TMPRSS3)mutatedin deaf-ness DFNB8/10activates the epithelial sodium channel(ENaC)in vitro[J].Hum Mol Genet,2002,11:2829.

58Guan MX,Fischel-Ghodsian N,Attardi G.Biochemical ev-idence for nuclear gene involvement in phenotype of non-syndromic deafness associated with mitochondrial12S rRNA mutation[J].Hum Mol Genet,1996,5:963.

59Bykhovskaya Y,Estivill X,Taylor K,et al.Candidate locus for a nuclear modifier gene for maternally inherited deafness[J].AmJ Hum Genet,2000,66:1905.

60Ballana E,Mercader J M,Fischel-Ghodsian N,et al.MRPS18CP2alleles and DEFA3absence as putative chromo-some8p23.1modifiers of hearing loss due to mtDNA muta-tion A1555Gin the12S rRNA gene[J].BMC Med Genet,2007,8:81.

61Li X,Guan MX.A human mitochondrial GTP binding pro-tein related to tRNA modification may modulate phenotypic expression of the deafness-associated mitochondrial12S rRNA mutation[J].Mol Cell Biol,2002,22:7701.

62Li X,Li R,Lin X,et al.Isolation and characterization of the putative nuclear modifier gene MTO1involved in the patho-genesis of deafness-associated mitochondrial12S rRNA A1555G mutation[J].J Biol Chem,2002,277:27256.

63Yan Q,Li X,Faye G,et al.Mutations in MTO2related to tRNA modification i mpair mitochondrial gene expression and protein synthesis in the presence of a paromomycin resistance mutationin mitochondrial15SrRNA[J].J Biol Chem,2005,280:29151.

64Ramelli GP,Gallati S,Weis J,et al.Point mutation tRNA(Ser(UCN))in a child with hearing loss and myoclonus epi-lepsy[J].J Child Neurol2006,21:253.

65Schuelke M,Bakker M,Stoltenburg G,et al.Epilepsia par-tialis continua associated with a homoplasmic mitochondrial tRNA(Ser(UCN))mutation[J].Ann Neurol1998,44:700.