Figure 20.

Findings for an EAS patient with CDH23 mutations. A: Endoscopic view of a round window insertion, B: postoperative CT findings, C: imaging with the putative location of the electrode and the referential tonotopic map, D: preoperative ASSR findings and postoperative COR audiogram findings. E: Pedigree and mutations. F: Auditory behavioral development assessed by LittlEARS auditory questionnaire, indicating rapid improvement in auditory behavior reaching the curve of normally developed children (A–F; Usami et al., 2012). G: Speech perception score at the age of seven showing good outcome.