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A: Pedigree of the patient with SLC26A4 mutations. B: CT scans showing bilateral EVA (arrow heads) with C: cochlear hypoplasia (arrows). D: ASSR and E: COR findings. ASSR findings at six months old showing profound hearing loss with some residual hearing at 500 Hz. COR findings demonstrating hearing amplification with hearing aids, indicating insufficient amplification to obtain good language development.
