title: Cochlear implantation in congenital cochlear abnormalities.
abstract: Many children have benefited from cochlear implant device including those with congenital malformation of the inner ear. The results reported in children with malformed cochlea are very encouraging. We describe 2 cases of Mondini's malformation with severe sensorineural hearing loss. Cochlear implantation was performed and both of them underwent post-implantation speech rehabilitation. Post-implantation, both of them were noted to respond to external sound. But the second case developed facial twitching a few months after the device was switched on. It is important to evaluate the severity of the inner ear deformity and the other associated anomalies in pre-implantation radiological assessment in order to identify the problem that may complicate the surgery and subsequent patient management.
authors.affiliation: Department of Otolaryngology-Head & Neck Surgery, Kulliyah of Medicine, International Islamic Universiti Malaysia, Kuantan, Pahang.
authors.forename: R L
authors.initials: RL
authors.lastname: Ahmad
authors.forename: S
authors.initials: S
authors.lastname: Lokman
mesh_terms: D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities*; D019929:Cochlear Implantation / Q000379:methods*; D003054:Cochlear Implants; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D011859:Radiography