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Updated: May 9, 2026
Robotic Cochlear Implantation for Direct Cochlear Access
Published on: June 16, 2022
Cochlear implantation in a familial rare syndromic ossification-related deafness and literature review
Xinyue Zou1, Biao Chen1, Jingyuan Chen1
1Department of Otorhinolaryngology and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, P. R. China.
Background:
Kenny-Caffey Syndrome type 2 (KCS2) is a genetic disease affecting bone metabolism. However, cochlear implantation (CI) results have yet to be published in detail.
Objective:
This study presents the gene, clinical characteristics, surgical outcomes, and literature review of 2 patients with sensorineural hearing loss related to KCS2. To enhance diagnostic detection and accuracy, we also compare the differential diagnosis between KCS2, otosclerosis, and Cogan's syndrome (CS).
Methods:
Prior to CI, patients with KCS2 and CS underwent comprehensive audiological and radiological evaluations. Postoperative auditory speech outcomes and impedance values were recorded and analyzed statistically. A systematic search of the literature was conducted to summarize clinical characteristics.
Results:
Patients diagnosed with KCS2 exhibit more pronounced changes in the inner ear. The impedance values in the KCS2 cohort were considerably higher (Mean = 12.13 kΩ) than those with CS (Mean = 8.8 kΩ) one year post-activation. The literature review exhibits the clinical manifestations associated with KCS2.
Conclusion:
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.
