(0972) Vestibular Dysfunction in Sickle Cell Patients

Introduction: Sickle cell disease (SCD) often leads to sensorineural hearing loss due to vaso-occlusive events in the cochlear vasculature. While the vestibule and cochlea share a blood supply, information on vestibulopathy in SCD is limited. This systematic review aims to consolidate current knowledge on vestibular dysfunction in SCD patients.

Methods: The study, registered on Prospero, involved a thorough electronic search using MEDLINE-Ovid, Embase, Google Scholar, The Cochrane Library, and Scopus databases from inception to December 2023. Data extraction adhered to PRISMA guidelines. Authors independently assessed bias and evidence quality using NIH Study Quality Assessment tools. Inclusion criteria covered articles mentioning vestibular symptoms in SCD patients, while exclusion criteria comprised non-English articles and vestibular symptoms limited to treatment side effects.

Results: Out of 2495 studies, only 12 met criteria. Among SCD patients undergoing head imaging, 19% reported inner ear complaints, and 70% experienced dizziness/imbalance. In a group of SCD children, there was a significant relationship between endothelial dysfunction and vertigo duration. The recommended imaging sequence is T1-weighted thin-section temporal MRIs, revealing abnormal findings even without clinical symptoms. Imaging shows labyrinthine hemorrhage and labyrinthitis ossificans, mostly unilateral. Vestibular symptoms emerge with age, suggesting cortical compensation keeps most asymptomatic. Symptoms may arise from brainstem ischemia or reduced mobility. In asymptomatic adult SCD patients, there's no significant difference compared to controls in tracking test batteries and positional tests; however, saccadic latency is longer in SCD patients.

Conclusions: The existing data on vestibulopathy in SCD is limited and often of poor quality. Although a connection between SCD and vestibular symptoms is noted, information on treatment approaches is scant. Further research in this area could contribute to the early diagnosis of vestibular dysfunction, potentially enhancing outcomes for SCD patients.