Liaci Enrico, Maccarrone Francesco, Negri Maurizio, Silvestri Martina, Paola Benincasa, Guida Maurizio, Minniti Rosa Maria, Tassi Sauro, Di Lella Filippo
Department of Medicine and Surgery, University of Parma and Department of Otolaryngology, Azienda Ospedaliero-Universitaria di Parma, Parma PR, Italy.
Department of Otolaryngology-Head and Neck Surgery, Azienda USL di Modena, Ospedale "B. Ramazzini" di Carpi, Carpi, Italy.
J Int Adv Otol. 2025 May 20;21(3):1-5. doi: 10.5152/iao.2025.241682.
Superficial siderosis (SS) is a chronic condition characterized by progressive hemosiderin accumulation in the central nervous system (CNS) due to chronic subarachnoid hemorrhage. Common clinical features include progressive bilateral sensorineural hearing loss (SNHL), cerebellar ataxia and myelopathy. The aim of the study was to analyze the clinical presentation, management, and outcomes of patients affected by SS with bilateral severe-to-profound hearing loss and treated with cochlear implantation (CI), with a particular focus on the course of hearing benefit.
A retrospective monocentric review was performed, including patients affected by CNS SS and profound SNHL that underwent CI between January 2012 and December 2021. Outcomes were assessed by comparing pre- and post-operative tonal and vocal hearing thresholds together with verbal perception tests.
A total of 4 patients were identified, with a mean follow-up time of 48.25 months. All patients showed significant improvement of hearing threshold and verbal perception in the first 6 months after surgery (preoperative mean aided PTA of 58.3 dB with a mean open-set speech perception score of 20.3% versus postoperative mean PTA in bimodal stimulation of 41.1 dB and open-set speech perception score of 60.1%). At the last follow-up evaluation, despite progressive deterioration of auditory performances, all patients were users and maintained subjective benefit from CIs.
Patients affected by SS who underwent CI showed a significant improvement in auditory and auditory-verbal performances in the first post-operative period. Despite a progressive decline of hearing benefit due to retrocochlear disease progression, CI represents valid support for lip-reading and spatial orientation even in the advanced stages of the disease.
表面性铁沉积症（SS）是一种慢性疾病，其特征是由于慢性蛛网膜下腔出血导致中枢神经系统（CNS）中铁蛋白进行性积聚。常见临床特征包括进行性双侧感音神经性听力损失（SNHL）、小脑共济失调和脊髓病。本研究的目的是分析患有双侧重度至极重度听力损失并接受人工耳蜗植入（CI）治疗的SS患者的临床表现、治疗方法及预后，特别关注听力获益过程。
进行了一项回顾性单中心研究，纳入2012年1月至2021年12月期间接受CI治疗的CNS-SS和重度SNHL患者。通过比较术前和术后的纯音及言语听力阈值以及言语感知测试来评估预后。
共确定4例患者，平均随访时间为48.25个月。所有患者术后6个月内听力阈值和言语感知均有显著改善（术前平均助听后PTA为58.3 dB，平均开放式言语感知得分20.3%，而术后双模式刺激下平均PTA为41.1 dB，开放式言语感知得分60.1%）。在最后一次随访评估时，尽管听觉表现逐渐恶化，但所有患者仍在使用人工耳蜗并维持主观获益。
接受CI治疗的SS患者在术后初期听觉及听觉言语表现有显著改善。尽管由于蜗后疾病进展听力获益逐渐下降，但即使在疾病晚期，人工耳蜗对唇读和空间定向仍有有效的支持作用。
