Cochlear implantation in a patient with osteogenesis imperfecta

Abstract

Osteogenesis imperfecta (OI) is a connective tissue disorder characterized by a deficit in the synthesis of type I collagen. Hearing loss affects 42-58% of OI patients and progresses to deafness in 35-60% of these patients. For OI patients, cochlear implantation (CI) is the only promising treatment option. However, literature on CI in patients with OI is relatively rare. After CI, speech perception is generally good. However, among patients with severe demineralization of the cochlea, most patients are reported to have complications of facial nerve stimulation (FNS), preventing some patients from using the cochlear implant on a daily basis. Here we report a successful CI using a Nucleus CI24 Contour Advance cochlear implant in a patient with OI. Although high-resolution computed tomography (HRCT) showed extensive demineralization of the cochlea, intracochlear electrodes were inserted properly. The use of a modiolus-hugging device and the advance off-stylet technique contributed to the successful implantation, with no complications such as FNS or misplacement of electrodes. Therefore, CI can be used for treating deaf patients with OI.