Figure 10.

A: Pedigree of a patient with GJB2 mutations. B: Postoperative X‐ray findings. C: ABR findings at three months old showing profound hearing loss. D: ASSR findings at three months old showing hearing loss in all frequencies and no residual hearing in the lower frequencies. E: Hearing amplification with hearing aids indicating insufficient amplification to obtain good language development. F: Hearing thresholds after bilateral CI. G: Auditory behavioral development assessed by LittlEARS auditory questionnaire. The development curve shows rapid improvement in auditory behavior, reaching the curve of normally developed children. H. Speech perception test results at Age 7 were dramatically improved (monosyllable: rt 80%, lt 95%; bil word: 100%; bil sentence: 96%).