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Pedigree, temporal bone CT, variant analysis, and audiogram of family C. (a) Affected subjects are denoted in black. Arrow shows the proband. (b) Temporal bone CT of the IV:2 shows no structural change. (c) Chromatogram shows POU4F3 heterozygous c.635T>C detected in patients. (d) Audiograms of the affected subjects. Audiogram configuration of IV:2 was U-shaped. Downsloping audiogram configurations were observed in III:6 and II:4 (red: right ear; blue: left ear).
