Vestibular Dysfunction in Patients With Sickle Cell Disease: A Systematic Review.

Introduction: Sickle cell disease (SCD) often leads to sensorineural hearing loss due to vaso-occlusive events in the cochlear vasculature. Although 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: This 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, whereas exclusion criteria comprised non-English articles and vestibular symptoms limited to treatment side effects.

Results: Out of 2,495 studies, only 12 met the 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 was T1-weighted thin-section temporal bone MRIs, which revealed abnormal findings even without clinical symptoms. Imaging showed labyrinthine hemorrhage and labyrinthitis ossificans, mostly unilateral. Vestibular symptoms emerged with older age, suggesting cortical compensation kept most subjects asymptomatic. In asymptomatic adult SCD patients, there was no significant difference compared with controls in tracking test batteries and positional tests; however, saccadic latency was longer in SCD patients.

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