A cavernous change due to otosclerosis that extends to the cochlear axis is called cochlear otosclerosis, which causes progressive sensorineural hearing loss (SNHL); cochlear implantation (CI) is considered in cases with severe to profound deafness. There are many reports of CI for adult cochlear otosclerosis, whereas this is only the second report of CI for a pediatric case of cochlear otosclerosis.
In this study, we report the case of an 11-year-old girl in whom we performed bilateral CI for SNHL due to cochlear otosclerosis. She had no problems during the perinatal period, and could speak some words by the age of 2. Gradually thereafter, however, she developed bilateral severe SNHL and became almost totally deaf by the age of 10. Temporal bone CT showed marked decalcification and osteogenesis of the otic capsules on both sides, suggesting that the left basal turns of the cochleas were narrow. The first CI (Cochlear, Contour, CI24RE (CA)) was performed on the right ear when she was 12 years old. Following the CI, improvement was noted not only in the pure tone hearing threshold, but also in speech perception. Five months later, the second CI on the left side was performed to achieve the better performance with bilateral CI. During the second CI, the intracochlear electrode array could not be fully inserted because of resistance when inserting the electrodes, and the postoperative results of CI on the left side were worse than those on the right side. However, at present, she is able to perform very well in her daily life with bilateral CIs, and plays important roles in society.
It was demonstrated that CI can be useful even for pediatric patients with cochlear otosclerosis. The indications for CI in these children must be carefully considered by evaluating the results of imaging examinations, such as CT, and provision of adequate explanation prior to the procedure is considered as very important.
