This study aimed to evaluate cochlear angulation in patients with CHARGE syndrome and compare it with individuals with normal cochlear anatomy. 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 angulation 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. Cochlear hypoplasia type III was the most common anomaly in the CHARGE group. Cochlear nerves were frequently hypoplastic or absent, whereas all control ears were normal. Mean cochlear angulation was significantly narrower in the CHARGE group compared with controls (94.49° ± 10.02° vs. 114.02° ± 8.17°, p < 0.001). CHARGE syndrome is associated with significantly altered cochlear angulation, which may contribute to surgical challenges during cochlear implantation. Preoperative recognition of this feature may facilitate safer and more effective surgical planning.
Eroğlu et al. (2026) studied this question.