1 Morton NE.Genetic epidemiology of hearing impairment[J].Annals of the New York Academy of Sciences,1991,630:16.

2 Reardon W.Genetic deafness[J].J Med Genet,1992,29:521.

3 Hilgert N,Smith RJ,Van Camp G.Forty-six genes causing nonsyndromic hearing impairment:which ones should be ana-lyzed in DNA diagnostics[J].Mutation Research,2009,681:189.

4 Van Camp G,Smith R.Hereditary hearing loss homepage.ht-tp://hereditaryhearingloss.org/.2011,10.

5 Kelsell DP,Dunlop J,Stevens HP,et al.Connexin 26mutations in hereditary non-syndromic sensorineural deafness[J].Na-ture,1997,387:80.

6 Loffler J,Nekahm D,Hirst-Stadlmann A,et al.Sensorineural hearing loss and the incidence of Cx26 mutations in Austria[J].European Journal of Human Genetics,2001,9:226.

7 Xia JH,Liu CY,Tang BS,et al.Mutations in the gene encoding gap junction protein beta-3associated with autosomal domi-nant hearing impairment[J].Nature Genetics,1998,20:370.

8 Kunst H,Marres H,Huygen P,et al.Nonsyndromic autosomal dominant progressive sensorineural hearing loss:audiologic a-nalysis of a pedigree linked to DFNA2[J].The Laryngoscope,1998,108:74.

9 Coucke PJ,Van Hauwe P,Kelley PM,et al.Mutations in the KCNQ4gene are responsible for autosomal dominant deafness in four DFNA2families[J].Human molecular genetics,1999,8:1 321.

10 Robertson NG,Lu L,Heller S,et al.Mutations in a novel co-chlear gene cause DFNA9,a human nonsyndromic deafness with vestibular dysfunction[J].Nature Genetics,1998,20:299.

11 de Kok YJ,Bom SJ,Brunt TM,et al.A Pro51Ser mutation in the COCH gene is associated with late onset autosomal domi-nant progressive sensorineural hearing loss with vestibular defects[J].Human Molecular Genetics,1999,8:361.

12 Usami S,Takahashi K,Yuge I,et al.Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction,but not of Meniere's disease[J].European Journal of Human Genetics,2003,11:744.

13 Wayne S,Robertson NG,DeClau F,et al.Mutations in the transcriptional activator EYA4cause late-onset deafness atthe DFNA10locus[J].Human Molecular Genetics,2001,10:195.

14 Pfister M,Toth T,Thiele H,et al.A 4-bp insertion in the eya-homologous region(eyaHR)of EYA4causes hearing impairment in a Hungarian family linked to DFNA10[J].Mol Med,2002,8:607.

15 Makishima T,Madeo AC,Brewer CC,et al.Nonsyndromic hearing loss DFNA10and a novel mutation of EYA4:evidence for corre-lation of normal cardiac phenotype with truncating mutations of the Eya domain[J].American Journal of Medical Genetics Part A,2007,143(A):1 592.

16 Hildebrand MS,Coman D,Yang T,et al.A novel splice site mutation in EYA4causes DFNA10hearing loss[J].American Journal of Medical Genetics Part A,2007,143(A):1 599.

17 Kubisch C,Schroeder BC,Friedrich T,et al.KCNQ4,a no-vel potassium channel expressed in sensory outer hair cells,is mutated in dominant deafness[J].Cell,1999,96:437.

18 Talebizadeh Z,Kelley PM,Askew JW,et al.Novel mutation in the KCNQ4gene in a large kindred with dominant progres-sive hearing loss[J].Human Mutation,1999,14:493.

19 Van Hauwe P,Coucke PJ,Ensink RJ,et al.Mutations in the KCNQ4K+channel gene,responsible for autosomal domi-nant hearing loss,cluster in the channel pore region[J].A-merican Journal of Medical Genetics,2000,93:184.

20 Van Camp G,Coucke PJ,Akita J,et al.A mutational hot spot in the KCNQ4gene responsible for autosomal dominant hear-ing impairment[J].Human Mutation,2002,20:15.

21 Kamada F,Kure S,Kudo T,et al.A novel KCNQ4one-base deletion in a large pedigree with hearing loss:implication for the genotype-phenotype correlation[J].Journal of Hu-man Genetics,2006,51:455.

22 Su TR,Chen CH,Huang SJ,et al.Functional study of the effect of phosphatase inhibitors on KCNQ4 channels ex-pressed in Xenopus oocytes[J].Acta Pharmacologica Sinica,2009,30:1 220.