成骨不全引起的耳硬化症之人工耳蝸植入－病例報告

Cochlear Implantation in an Advanced Otosclerosis Patient Caused by Osteogenesis Imperfecta-Case Report

陳冠瑋(Kuan-Wei Chen) ； 吳振吉(Chen-Chi Wu) ； 楊宗翰(Tsung-Han Yang) ； 黃弘孟(Hung-Meng Huang)

《台灣耳鼻喉頭頸外科雜誌》 55卷1期 (2020/03) Pp. 27-31

https://doi.org/10.6286/jtohns.202003_55(1).027

摘要

成骨不全（osteogenesis Imperfecta）是由第一型膠原蛋白製造缺陷所引起减少骨量及強度的遺傳疾病，發生率約為1/20,000。在臨床及組織學上除了肢體變形外，也發現耳硬化症等臨床表徵。患者在成年早期發生聽力損失時，可能是傳導性失聰，感覺神經性失聰或混合性失聰。1名53歲女性患者，於30歲時發現聽損，左耳於7、8年前聽力急遽下降至極重度聽損，1年前右耳聽力也急遽下降至極重度聽損，佩戴助聽器亦無法辨識語音與人溝通。純音聽力檢查：右耳90 dB／左耳105 dB。高解析度顳骨電腦斷層顯示雙耳都有Grade 2C的耳硬化症。病患接受右側人工耳蝸植入，植入時圓窗不明顯，吾等使用cochlear nucleus CI 24RE預彎電極以advance off-stylet方式植入。術後NRT 22個電極皆有反應。病人術後2週開頻，1週後調頻即可講電話，沒有面神經刺激等併發症。因病例罕見，特提出報告。

關鍵字

cochlear implantation ； facial nerve stimulation ； osteogenesis imperfecta（人工耳蝸植入 ； 顏面神經刺激 ； 成骨不全症）

並列摘要

Osteogenesis imperfecta (OI) is a connective tissue disease caused by a defect in the synthesis of type 1 collagen. The prevalence of OI is approximately 1/20000. Otosclerosis has been observed both clinically and histopathologically in patients with OI, and is associated with conductive, sensorineural, or mixed type hearing loss. We present the case of a 53-year-old woman with OI who had bilateral profound hearing loss and could not communicate even with hearing aids. High-resolution computed tomography (HRCT) revealed bilateral Grade 2C otosclerosis, as described by Rotteveel et al. We used a cochlear nucleus CI 24RE pre-curved electrode for insertion of cochlear implant with the advance off-stylet technique. Round window niche was not apparent during the operation. The patient was able to communicate even on the phone after the procedure. There was neither facial nerve stimulation nor other severe complications.

並列關鍵字

cochlear implantation ； facial nerve stimulation ； osteogenesis imperfecta