Cochlear implantation in a familial rare syndromic ossification-related deafness and literature review.

CI is an effective treatment for KCS2 to restore hearing loss. More frequent programming and accurate adjustment of stimulation is of great necessity. A thorough examination, including temporal bone HRCT, 3D-MRI, audiological evaluations, and whole-exome sequencing, is essential for the diagnosis and treatment of KCS2.