CT diagnosis of temporal bone anomalies and cochlear implant surgery in CHARGE association

Children with CHARGE have multiple disabilities. Impairments of vision and hearing, balance problems and facial palsy are common. Few reports have dealt with their radiological temporal bone changes and none with cochlear implant surgery. The pre-operative temporal bone computed tomography (CT) and surgical findings of one child with CHARGE and one with a CHARGE-like condition who have received cochlear implants are reported. The findings include absent semicircular canals, narrow orifice for the cochlear nerve, and abnormalities of the oval and round window, the facial canal and the ossicles. CT can be used as a diagnostic tool as these combinations of temporal bone changes are extremely rare in other materials. Cochlear implants may help these often very isolated children to communicate.