Sato Kei, Ohi Takekazu, Shozaki Taisaku, Kariya Shin
Department of Neurology, Ajirogikai Uji Hospital.
Brain Nerve. 2018 Jan;70(1):73-80. doi: 10.11477/mf.1416200952.
We report the case of a 38-year-old man with gait disorder and hearing loss. The patient had developed gait disorder due to a cervical meningioma since 4 year-old disappeared for 15 years after the surgical removal of the meningioma. However, at the age of 21 year-old, the gait disorder reappeared and worsened progressively. Sensorineural hearing loss (SNHL) and epileptic seizures developed during the disease course, and he was diagnosed with superficial siderosis (SS). When he was 37 years old, he experienced sudden-onset of right-side hearing impairment and was considered a candidate for cochlear implantation (CI) at the otorhinolaryngology clinic of the nearby University Hospital. He underwent CI in November 2014. Eight months after the operation, his right side hearing improved although ataxia, hearing loss, and pyramidal sign persisted. At the long term follow-up of 29 months after CI, his hearing remained at the improved level. Thus, CI may be an effective long-term treatment for SNHL in patients with SS and could prevent the progression of his hearing loss. (Received February 1, 2017; Accepted August 23, 2017; Published January 1, 2018).
我们报告了一例38岁患有步态障碍和听力损失的男性病例。该患者自4岁起因颈椎脑膜瘤出现步态障碍，脑膜瘤手术切除后消失了15年。然而，在21岁时，步态障碍再次出现并逐渐加重。病程中出现感音神经性听力损失（SNHL）和癫痫发作，他被诊断为浅表性铁沉积症（SS）。37岁时，他突然出现右侧听力障碍，在附近大学医院的耳鼻喉科门诊被认为是人工耳蜗植入（CI）的候选者。他于2014年11月接受了CI手术。术后8个月，他的右侧听力有所改善，尽管共济失调、听力损失和锥体束征仍然存在。在CI术后29个月的长期随访中，他的听力保持在改善水平。因此，CI可能是SS患者SNHL的一种有效的长期治疗方法，并可防止其听力损失的进展。（2017年2月1日收到；2017年8月23日接受；2018年1月1日发表）
