Makizumi Yoshimi, Kashio Akinori, Sakamoto Takashi, Karino Shotaro, Kakigi Akinobu, Iwasaki Shinichi, Yamasoba Tatsuya
Department of Otolaryngology and Head and Neck Surgery, Graduate School of Medicine, The University of Tokyo, Japan.
Auris Nasus Larynx. 2013 Oct;40(5):510-3. doi: 10.1016/j.anl.2012.10.006. Epub 2012 Dec 6.
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.
成骨不全症（OI）是一种结缔组织疾病，其特征是I型胶原蛋白合成不足。听力损失影响42%-58%的OI患者，其中35%-60%的患者会发展为耳聋。对于OI患者，人工耳蜗植入（CI）是唯一有希望的治疗选择。然而，关于OI患者人工耳蜗植入的文献相对较少。人工耳蜗植入后，言语感知通常良好。然而，在耳蜗严重脱矿的患者中，据报道大多数患者有面神经刺激（FNS）并发症，导致一些患者无法日常使用人工耳蜗。在此，我们报告1例使用Nucleus CI24 Contour Advance人工耳蜗成功植入的OI患者。尽管高分辨率计算机断层扫描（HRCT）显示耳蜗广泛脱矿，但耳蜗内电极插入顺利。使用蜗轴环抱装置和先进的无针芯技术促成了成功植入，未出现FNS或电极误置等并发症。因此，人工耳蜗植入可用于治疗OI致聋患者。
