Kharytaniuk Natallia, Cowley Peter, Werring David J, Bamiou Doris-Eva
Ear Institute, University College London, London, United Kingdom.
National Institute for Health Research, University College London Hospitals Biomedical Research Centre (Deafness and Hearing Problems Theme), London, United Kingdom.
Front Neurol. 2021 Jan 14;11:610819. doi: 10.3389/fneur.2020.610819. eCollection 2020.
Hearing and balance impairment are the most frequently reported features of infratentorial (classical) superficial siderosis (iSS). There are few comprehensive descriptions of audiovestibular function in iSS and therefore limited understanding of the affected segment(s) of the audiovestibular pathway. In addition, monitoring disease progression and response to treatment is challenging and currently mainly guided by subjective patient reports and magnetic resonance imaging. To the best of our knowledge, there have been no previous reports assessing central auditory function in iSS. We describe such findings in a patient with iSS in an attempt to precisely localize the site of the audiovestibular dysfunction, determine its severity and functional impact. We confirm the presence of (asymmetrical) auditory neuropathy and identify central auditory processing deficits, suggesting involvement of the central auditory pathway beyond the brainstem. We correlate the audiological and vestibular findings with self-report measures and the siderosis appearances on brain magnetic resonance images.
听力和平衡障碍是幕下（经典型）表面性铁沉积症（iSS）最常报道的特征。关于iSS患者听觉前庭功能的全面描述较少，因此对听觉前庭通路受影响节段的了解有限。此外，监测疾病进展和治疗反应具有挑战性，目前主要以患者主观报告和磁共振成像为指导。据我们所知，此前尚无评估iSS患者中枢听觉功能的报道。我们描述了1例iSS患者的此类发现，试图精确确定听觉前庭功能障碍的部位，确定其严重程度和功能影响。我们证实了（不对称性）听觉神经病的存在，并识别出中枢听觉处理缺陷，提示中枢听觉通路受累范围超出脑干。我们将听力学和前庭检查结果与自我报告测量结果以及脑磁共振图像上的铁沉积表现进行了关联。
