(884) Sudden Sensorineural Hearing Loss and Vestibular Neuritis in Sickle Cell Disease: A Single Institution Analysis

Introduction: Vascular occlusion has been proposed as a cause of SSNHL and VN. Individuals with SCD and SCT are at increased risk for vaso-occlusive events and ischemic organ injury. This study sought to describe the prevalence, clinical presentation, management, and outcomes of SSNHL and VN in a cohort of patients with SCD or SCT.

Methods: A single-center retrospective chart review was conducted of adults with known SCD or SCT who presented with SSNHL or VN between January 2000 and August 2025. Characteristics were assessed via descriptive statistics.

Results: 344 individuals with SCD or SCT (n = 264 M, n = 165 F) were identified. Amongst this cohort, 17 (4.9%) developed SSNHL over the 25-year study period with a mean age of 44.3 (SD = 12.9) at onset. The mean 4-tone pure tone average (PTA) in affected ears was 39 dB (Range 15-115, SD = 33.7). Notably, some patients experienced significant SSNHL at high frequencies that is not reflected in the PTA. Corticosteroids were administered to 42.9% of these patients. Among those with documented follow-up, the mean duration to last follow-up was 78.5 months (SD = 99.6), with all experiencing persistent hearing loss. Additionally, 9 patients (2.6%) from the cohort developed VN and 2 (22.2%) received corticosteroids. The mean follow-up was 107.7 months (SD = 30.5), at which time 42.9% demonstrated persistent vestibular symptoms.

Conclusions: The incidence of SSNHL and VN amongst individuals with SCD or SCT is substantially higher than that reported in the general population, which is estimated to be between 5 to 27 per 100,000 people annually. Furthermore, the initiation of steroid therapy is lower than anticipated, likely reflecting the risk of steroid-induced sickle cell crisis. These data support vascular occlusion as a cause of SSNHL and VN. Further studies are needed to better understand the risk-benefit ratio of steroids in this population.