A Novel Spontaneous Mutation of the SOX10 Gene Associated with Waardenburg Syndrome Type II

Figure 2

Clinical audiology examination and temporal bone CT scan of the proband. (a) ASSR (auditory steady-state responses) of the left ear: 105 dB, 105 dB, and 90 dB at 1, 2, and 4 kHz, respectively; the remaining frequencies showed no response. (b) ASSR of the right ear: 90 dB, 105 dB, and 100 dB at 500 Hz, 1, and 4 kHz; the remaining frequencies showed no response. (c) Semicircular canal abnormalities shown on high-resolution axial CT in the red square (left ear). (d) Semicircular canal abnormalities shown on high-resolution axial CT in the red square (right ear).