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Fig 1.

Pedigrees and audiograms of subjects from 5 families possibly carrying compound heterozygous CDH23 mutations.

Audiogram: Right (red) and left (blue) ear hearing thresholds. Pedigree: Filled symbols represent hearing-impaired individuals, and clear symbols denote those with normal hearing. Black arrow indicates the proband. Possible arrangement of CDH23 variants in SB116 family based on haplotype and segregation study.

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Table 1.

Genotype of individuals segregating homozygous or heterozygous mutations of CDH23 identified by TRS or WES, and from the previous study by Kim et al.

[9].

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Table 1 Expand

Table 2.

Ethnic-specific MAF and in silico pathogenicity prediction of CDH23 variants in our study.

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Table 2 Expand

Fig 2.

Suggestion of Genotypic Hierarchy of CDH23 mutations deciding the fate of USH1D and DFNB12.

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Fig 2 Expand