以听力减退为主要表现的中枢神经系统表面铁沉积症1例并文献复习

doi:10.6040/j.issn.1673-3770.0.2023.417

摘要/Abstract

摘要： 目的探讨以听力减退为主要表现的中枢神经系统表面铁沉积症(superficial siderosis of the central nervous system, SSCNS)的早期诊疗手段,以及对首发症状为听力减退的疾病的诊断、鉴别、总结和思考。方法回复性分析我院耳鼻喉科收治的以进行性听力减退为首发症状、无明显脑外伤、出血及手术史SSCNS的1例患者病历资料,讨论其中枢神经系统表面铁沉积症的临床诊治及影像学特点,结合查阅国内外相关文献及经验,探讨该病种的早期诊疗手段以及对首发症状为听力减退的疾病的诊断、鉴别、总结和思考。结果经治后听力较前无明显改善,间有耳鸣,患者要求出院。出院后进行跟踪随访发现,患者长期在家未规律就诊及服用铁螯合剂,听力症状无明显进一步加重,自述步态如常,偶有不稳,拒绝进一步检查。结论耳鼻喉科医师在临床上对原因不明的特发性进行性感音神经性听力下降患者要更加重视,不仅要提高对SSCNS 的认识,更要规范化培训临床上“听力减退”患者的诊疗思路,加强对引起“听力减退”的原发病的定位定性诊断及鉴别。

关键词: 中枢神经系统表面铁沉积症, 感音神经性耳聋, 阵发性耳鸣

Abstract: Objective To explore the early diagnosis and treatment methods of superficial siderosis of the central nervous system(SSCNS)with hearing loss as the main manifestation, as well as to summarize and reflect on the diagnosis, differentiation, and treatment of diseases with hearing loss as the initial symptom. Methods A retrospective analysis was conducted on the medical records of a patient with SSCNS admitted to the Department of Otorhinolaryngology of our hospital, who presented with progressive hearing loss as the initial symptom, without a history of significant head trauma, hemorrhage, or surgery. The clinical diagnosis and treatment, as well as the imaging characteristics of the patient's superficial siderosis of the central nervous system, were discussed. Combined with the review of domestic and foreign literature and experience, the early diagnosis and treatment methods of this disease were explored, as well as the diagnosis, differentiation, summary, and reflection on diseases with hearing loss as the initial symptom. Results After treatment, the patient's hearing did not improve significantly, and intermittent tinnitus occurred. The patient requested discharge. Follow-up after discharge revealed that the patient had not visited the hospital regularly or taken iron chelators at home for a long time. The hearing symptoms did not worsen significantly, and the patient reported normal gait with occasional instability. The patient refused further examination. Conclusion Otorhinolaryngologists should pay more attention to patients with idiopathic progressive sensorineural hearing loss of unknown cause in clinical practice. Not only should they enhance their understanding of SSCNS, but also standardize the diagnosis and treatment thinking for patients with “hearing loss” in clinical practice, and strengthen the localization and qualitative diagnosis and differentiation of the primary diseases causing “hearing loss”.

Key words: Central nervous system surface iron deposition, Sensorineural deafness, Paroxysmal tinnitus

中图分类号:

R764.4

王莹,蔡燕文. 以听力减退为主要表现的中枢神经系统表面铁沉积症1例并文献复习[J]. 山东大学耳鼻喉眼学报, 2025, 39(5): 89-96.

WANG Ying, CAI Yanwen. A case of SSCNS with hearing loss as the main manifestation and literature review[J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2025, 39(5): 89-96.

参考文献

[1] Kumar N. Superficial siderosis: a clinical review[J]. Ann Neurol, 2021, 89(6): 1068-1079. doi:10.1002/ana.26083
[2] 杨英麦, 钱敏, 郝红林, 等. 中枢神经系统表面铁沉积症的临床特征和病因分析[J]. 中华神经科杂志, 2022, 55(2): 125-132. doi:10.3760/cma.j.cn113694-20210607-00393 YANG Yingmai, QIAN Min, HAO Honglin, et al. The clinical features and etiology analysis of superficial siderosis of the central nervous system[J]. Chinese Journal of Neurology, 2022, 55(2): 125-132. doi:10.3760/cma.j.cn113694-20210607-00393
[3] 戴昰旭, 张长青, 王展, 等. 中枢神经系统表面铁沉积症1例报道[J]. 中国卒中杂志, 2023, 18(3): 335-339. doi:10.3969/j.issn.1673-5765.2023.03.012 DAI Shixu, ZHANG Changqing, WANG Zhan, et al. A case report of iron deposition on the surface of central nervous system[J]. Chinese Journal of Stroke, 2023, 18(3): 335-339. doi:10.3969/j.issn.1673-5765.2023.03.012
[4] 王诺, 吴涛. 中枢神经系统表面铁沉积症临床表现及研究进展[J]. 中风与神经疾病杂志, 2019, 36(3): 280-282. doi:10.19845/j.cnki.zfysjjbzz.2019.03.022 WANG Nuo, WU Tao. Clinical manifestations and research progress of iron deposition on the surface of central nervous system[J]. Journal of Apoplexy and Nervous Diseases, 2019, 36(3): 280-282. doi:10.19845/j.cnki.zfysjjbzz.2019.03.022
[5] Wilson D, Chatterjee F, Farmer SF, et al. Infratentorial superficial siderosis: classification, diagnostic criteria, and rational investigation pathway[J]. Ann Neurol, 2017, 81(3): 333-343. doi:10.1002/ana.24850
[6] 叶毅良, 卢标清. 704例突发性耳聋疗效分析[J]. 山东大学耳鼻喉眼学报, 2019, 33(5): 44-47. doi:10.6040/j.issn.1673-3770.0.2019.052 YE Yiliang, LU Biaoqing. Therapeutic outcome analysis of 704 cases of sudden deafness[J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2019, 33(5): 44-47. doi:10.6040/j.issn.1673-3770.0.2019.052
[7] Schievink WI, Maya MM, Harris J, et al. Infratentorial superficial siderosis and spontaneous intracranial hypotension[J]. Ann Neurol, 2023, 93(1): 64-75. doi:10.1002/ana.26521
[8] 左瑶, 贾国勇, 孟桂月, 等. 中枢神经系统表面铁沉积症四例临床及影像学特征分析[J]. 中华神经科杂志, 2020, 53(4): 291-297. doi:10.3760/cma.j.cn113694-20191128-00747 ZUO Yao, JIA Guoyong, MENG Guiyue, et al. The clinical and radiological features of superficial siderosis of central nervous system: report of four cases[J]. Chinese Journal of Neurology, 2020, 53(4): 291-297. doi:10.3760/cma.j.cn113694-20191128-00747
[9] 中华耳鼻咽喉头颈外科杂志编辑委员会, 中华医学会耳鼻咽喉头颈外科学分会. 突发性聋诊断和治疗指南(2015)[J]. 中华耳鼻咽喉头颈外科杂志, 2015, 50(6): 443-447. doi:10.3760/cma.j.issn.1673-0860.2015.06.002
[10] Charidimou A, Perosa V, Frosch MP, et al. Neuropathological correlates of cortical superficial siderosis in cerebral amyloid angiopathy[J]. Brain, 2020, 143(11): 3343-3351. doi:10.1093/brain/awaa266
[11] 吴月林, 梁钊铨, 黄子祥, 等. 中枢神经系统表面铁沉积症合并腰椎管内病变1例报道[J]. 中国脊柱脊髓杂志, 2021, 31(5): 470-474. doi:10.3969/j.issn.1004-406X.2021.05.13 WU Yuelin, LIANG Zhaoquan, HUANG Zixiang, et al. Superficial siderosis of the central nervous system with lesion in the lumbar spinal canal: a case report[J]. Chinese Journal of Spine and Spinal Cord, 2021, 31(5): 470-474. doi:10.3969/j.issn.1004-406X.2021.05.13

多维度评价

Viewed

Full text

Abstract

Cited

Shared

Discussed