Takasaki K, Tanaka F, Shigeno K, Kanda Y, Kawajiri I, Tashiro T, Kobayashi T
Department of Otolaryngology, Nagasaki University School of Medicine, Sakamoto, Nagasaki, Japan.
ORL J Otorhinolaryngol Relat Spec. 2000 Sep-Oct;62(5):270-3. doi: 10.1159/000027758.
This is a case of superficial siderosis of the central nervous system (SSCN). The diagnosis of SSCN was based on the result of T2-weighted magnetic resonance imaging and on suggestive clinical manifestations. The pure-tone audiogram showed bilateral progressive sensorineural hearing loss with a poor speech discrimination score and Jerger type IV. The remarkable elevation of the detective threshold of cochlear microphonics on electrocochleography was found and distortion product otoacoustic emission (DPOAE) showed no response: These electrophysiologic examinations, including electrocochleography and DPOAE, revealed that the progressive sensorineural hearing loss in this case was caused by both retrocochlear and cochlear damages.
这是一例中枢神经系统表面铁沉积症（SSCN）。SSCN的诊断基于T2加权磁共振成像结果及提示性的临床表现。纯音听力图显示双侧进行性感音神经性听力损失，言语辨别得分低，为耶格IV型。在耳蜗电图上发现耳蜗微音电位检测阈值显著升高，畸变产物耳声发射（DPOAE）无反应：包括耳蜗电图和DPOAE在内的这些电生理检查表明，该病例中的进行性感音神经性听力损失是由蜗后和耳蜗损伤共同导致的。
