Figure 2. Audiometry. Audiological evaluation of Patient 1 (A), Patient 2 (B), and Patient 3 (C) shows the hearing thresholds at all tested frequencies pre- and post-cochlear implantation. Pure tone sound field audiograms are represented by unaided (S), Aided (A), and Implanted (I). “O” and “X” symbols correspond to the right and left ear pure tone air conduction audiograms, respectively. Audiometric measurements were obtained at specific ages for each patient as denoted by each hearing assessment. All three affected individuals had bilateral profound sensorineural hearing loss before cochlear implantation. Post-implantation, all affected acquired hearing threshold measurements in the normal range.