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. 2001 Jan 25;68(3):559–568. doi: 10.1086/318803

Table 1.

Frequency of Common GJB2 Recessive Mutations in Different NSHL Population Groups

No. (%) of GJB2-Mutation Alleles Screeneda
PopulationEthnicity Total No. of NSHL Alleles Screened 35delG 167delT 235delC M34T Reference(s)
Spanish and Italian 1,546b 533 (34.48) 11 (.71) ND 1 (.07) Zelante et al. (1997); Estivill et al. (1998); Murgia et al. (1999); Rabionet et al. (2000b)
United Kingdom 142b 21 (14.79) ND ND ND Denoyelle et al. (1997); Lench et al. (1998)
United States 116c 33 (28.45) 9 (7.76) ND 2 (1.72) Kelley et al. (1998)
Japanese 308b ND ND 24 (7.80) ND Fuse et al. (1999); Abe et al. (2000); Kudo et al. (2000)
Ashkenazi Jewish 60c 13 (21.67) 34 (56.67) ND ND Morell et al. (1998); Lerer et al. (2000)
French 204b 78 (38.24) ND ND ND Denoyelle et al. (1997); (1999)
a

ND = not detected.

b

Sib-pair cases and sporadic cases of prelingual NSHL.

c

Autosomal recessive prelingual cases of NSHL.