Figure 1.

The clinical features and genetic testing results of the proband and the family member. (a) Pedigree of the family. The incidence pattern of the pedigree shows X-linked hereditary; (b) The imaging tests of the proband. The high-resolution axial computed tomography reveals a dilatation of lateral end of the internal auditory meatus and a deficit or absence in the basal turn of the cochlea; (c) Audiograms of the proband. The pure tone audiometry shows belated severe to profound hearing loss, but bone hearing thresholds was normal (10 dB) at 250 Hz; (d) Sequencing chromatograms of POU3F4. The molecular testing result of proband and so family members.