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