Evaluation of Cochlear Angular Orientation in Patients with CHARGE Syndrome

Abstract

Purpose This study aimed to evaluate cochlear angulation in patients with CHARGE syndrome and compare it with indi-viduals with normal cochlear anatomy. Methods Eighteen patients with CHARGE syndrome followed for sensorineural hearing loss were retrospectively reviewed and compared with 18 age-matched controls with normal cochlear anatomy. Temporal bone computed tomography (CT) and magnetic resonance imaging were analyzed to assess inner ear malformations and cochlear nerve status. Cochlear angula-tion was measured on axial CT images by calculating the angle formed between lines passing through the basal turns of both cochleae at the level of the round window. Statistical analyses were performed using chi-square and independent samples t-tests. Results Cochlear hypoplasia type III was the most common anomaly in the CHARGE group. Cochlear nerves were fre-quently hypoplastic or absent, whereas all control ears were normal. Mean cochlear angulation was significantly narrower in the CHARGE group compared with controls (94.49 degrees +/- 10.02 degrees vs. 114.02 degrees +/- 8.17 degrees, p < 0.001). Conclusion CHARGE syndrome is associated with significantly altered cochlear angulation, which may contribute to surgi-cal challenges during cochlear implantation. Preoperative recognition of this feature may facilitate safer and more effective surgical planning