TIGA KASUS IMPLAN KOKLEA PADA DISPLASIA MONDINI

Muhammad Arif Sudianto Utama, Artono Artono
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Abstract

Mondini dysplasia is a kind of cochlear malformation caused by a failure growth of cochlea in the seventh week of pregnancy, results in cochlear turn which only reaches 1,5 turn until less than 2,5 turn. There is sensorineural hearing loss in Mondini dysplasia caused by anatomical malformation. The diagnosis of Mondini dysplasia is made by accurate anamnesis, audiology assessments and imaging results. This case study explains the importance of the holistic process of diagnosis and treatment of Mondini's dysplasia. Purpose: To explain that Mondini dysplasia should be diagnosed immediately and given the cochlear implant. Cases: There had been reported three cases of cochlear malformation of Mondini dysplasi type. They were first diagnosed with sensorineural hearing loss and speech delay in the age of 2-4 years. After had been done sequence of audiology assessments and imaging CT-scan and MRI simultaneously there were obtained that those three patients had Mondini dysplasia. Case Management: The use of hearing aid in those three patients had no effectiveness that brought them to the decision of cochlear implantation. Cochlear implant became such a great challenge even through for experienced physicians considering the scarcity of the case and the risk for complications like pelymph gusher and incorrect insertion of electrode. Cochlear implant surgeries had been done to those three patients with carefu consideration and the selection for appropriate type of electrode. On the first case there had been applied right ear cochlear implant, on the second case was bilateral, and on the third case was in the left ear. Complications of the surgeries were bleeding and perilymph gusher but they had been solved properly. Electrode insertion in all three cases had been placed correctly. Conclusion: Found three cases of Mondini dysplasia with symptoms of bilateral hearing loss, speech development, bilateral Mondini dysplasia, techniques and complications of cochlear implants. These three cases differ in the shape, width and size of the cochlear anatomy if they are studied more specifically. Two cases of unilateral cochlear implant placement and one bilateral case.
蒙迪尼发育不良是指在妊娠第7周耳蜗发育不全,导致耳蜗转数仅达1.5转,直至小于2.5转的一种耳蜗畸形。蒙迪尼发育不良患者存在由解剖畸形引起的感音神经性听力损失。诊断蒙迪尼发育不良是通过准确的记忆,听力学评估和影像学结果。本案例研究解释了诊断和治疗蒙迪尼发育不良的整体过程的重要性。目的:说明蒙迪尼发育不良应立即诊断并给予人工耳蜗植入。病例:报告了3例Mondini型耳蜗畸形。他们在2-4岁时首次被诊断为感音神经性听力丧失和语言迟缓。经一系列听力学检查、影像学ct扫描及MRI检查,确定为蒙迪尼发育不良。病例管理:这3例患者使用助听器没有效果,导致他们决定人工耳蜗植入。考虑到病例的稀缺性和并发症的风险,如淋巴喷涌和不正确的电极插入,人工耳蜗即使对经验丰富的医生来说也是一个巨大的挑战。经慎重考虑,选择合适的电极,对3例患者行人工耳蜗植入手术。1例为右耳人工耳蜗,2例为双侧人工耳蜗,3例为左耳人工耳蜗。手术的并发症是出血和淋巴管周围喷涌,但都得到了妥善的解决。三个病例的电极插入位置均正确。结论:发现3例双侧蒙迪尼发育不良患者,症状包括双侧听力损失、言语发育、双侧蒙迪尼发育不良、人工耳蜗植入技术及并发症。如果对这三种情况进行更具体的研究,它们在耳蜗解剖结构的形状、宽度和大小上都有所不同。单侧人工耳蜗植入2例,双侧人工耳蜗植入1例。
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