Audiovestibular Dysfunction Related to Anti-Phospholipid Syndrome: A Systematic Review

Abstract

Background: Anti-phospholipid syndrome (APS) has emerged as a significant issue in autoimmune diseases over recent decades. Its hallmark feature is thromboembolic events, potentially affecting any vascularized area including the microcirculation of the inner ear. Since the first case report of APS-related audiovestibular dysfunction described in 1993, numerous reports have explored the association between APS-related antibodies and audiovestibular dysfunction. These studies indicate a higher prevalence of APS-related antibodies in patients with sensorineural hearing loss compared to healthy controls. Unlike other idiopathic hearing loss disorders, audiovestibular dysfunction associated with APS may respond to appropriate treatments, highlighting the importance of timely recognition by clinicians to potentially achieve favorable outcomes. Therefore, this systematic review aims to consolidate current evidence on the characteristics, pathophysiology, assessment, and management of audiovestibular dysfunction linked to APS. Methods: This systematic review utilized electronic searches of the PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect online platforms. The initial search was performed on 27 January 2024, with the final update search completed on 20 June 2024. Results: Based on theoretical pathophysiology, anticoagulation emerges as a pivotal treatment strategy. Additionally, drawing from our preliminary data, we propose a modified protocol combining anticoagulants, steroids, and non-invasive brain stimulation to offer clinicians a novel therapeutic approach for managing these symptoms. Conclusions: Clinicians are encouraged to remain vigilant about the possibility of APS and its complex audiovestibular manifestations, as prompt intervention could stabilize audiovestibular function effectively.

Keywords

• anti-phospholipid syndrome
• cochleopathy
• vestibular
• sensorineural hearing loss
• treatment