Cochlear implantation in a patient with osteogenesis imperfecta.
Cochlear implantation in a patient with osteogenesis imperfecta.
复制标题
成骨不全患者的人工耳蜗植入。
DOI：
10.1016/j.anl.2012.10.006
复制
发表时间：
2012
期刊：
影响因子：
--
通讯作者：
Yamasoba T.
中科院分区：
文献类型：
--
作者：
Makizumi Y;Kashio A;Sakamoto T;Karino S;Kakigi A;Iwasaki S;Yamasoba T.
成骨不全症（OI）是一种结缔组织疾病，其特征是I型胶原合成缺陷。42-58%的成骨不全患者有听力损失，其中35-60%的患者发展为耳聋。对于成骨不全患者，人工耳蜗植入（CI）是唯一有希望的治疗选择。然而，关于成骨不全患者CI的文献相对较少。CI后，语音感知总体良好。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）的并发症，使一些患者无法每天使用人工耳蜗。在这里，我们报告了一例使用Nucleus CI24 Contour Advance人工耳蜗成功植入OI患者的人工耳蜗。尽管高分辨率计算机断层扫描（HRCT）显示耳蜗广泛脱矿，但耳蜗内电极被正确插入。使用一种固定装置和先进的脱针技术有助于成功植入，没有并发症，如FNS或电极错位。因此，CI可用于治疗伴有成骨不全的耳聋患者。
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.