BACKGROUND: Malformations of the cochlea can cause deafness and otogenic meningitis. Cochlear implantation in children with such malformations, which are sometimes quite complex, require special attention before, during and after surgery. PATIENT: The concept developed for these patients at Hannover Medical School is illustrated in a patient with severe bilateral inner ear malformation and bilateral stapes defect. Implantation of a cochlear implant was performed in this 2-year-old girl. A large endostal sack originating from the inner ear, which had caused meningitis twice, was observed in the right middle ear. Both ears had to be obliterated to prevent recurrent meningitis. RESULTS: The child had no post-operative problems and the initial rehabilitation efforts have been successful. The immediate and differentiated reactions to stimulation are very encouraging. This article briefly summaries all diagnostic and therapeutic procedures established over the last three years at Hannover Medical School in cases of implantation into a malformated cochlea. In particular, we describe perioperative radiology (CT scans, MRI and plain radiographs), facial nerve monitoring and intraoperative screening for "hearing sensations", details of surgical technique, and possible postoperative complications. CONCLUSIONS: Based on experience with 13 of these cases, we discuss the different aspects of implantation into a malformated cochlea. Given certain preconditions implantation into a malformated cochlea may be recommended.
BACKGROUND:Malformations of the cochlea can cause deafness and otogenic meningitis. Cochlear implantation in children with such malformations, which are sometimes quite complex, require special attention before, during and after surgery. PATIENT: The concept developed for these patients at Hannover Medical School is illustrated in a patient with severe bilateral inner ear malformation and bilateral stapes defect. Implantation of a cochlear implant was performed in this 2-year-old girl. A large endostal sack originating from the inner ear, which had caused meningitis twice, was observed in the right middle ear. Both ears had to be obliterated to prevent recurrent meningitis. RESULTS: The child had no post-operative problems and the initial rehabilitation efforts have been successful. The immediate and differentiated reactions to stimulation are very encouraging. This article briefly summaries all diagnostic and therapeutic procedures established over the last three years at Hannover Medical School in cases of implantation into a malformated cochlea. In particular, we describe perioperative radiology (CT scans, MRI and plain radiographs), facial nerve monitoring and intraoperative screening for "hearing sensations", details of surgical technique, and possible postoperative complications. CONCLUSIONS: Based on experience with 13 of these cases, we discuss the different aspects of implantation into a malformated cochlea. Given certain preconditions implantation into a malformated cochlea may be recommended.