Investigating the significance of vestibular aqueduct pneumatization and pediatric hearing loss

Introduction: Vestibular aqueduct enlargement on imaging is associated with pediatric hearing loss, though the mechanism is not well understood. After reviewing temporal bone imaging in pediatric patients from our institution with no obvious cause of hearing loss, we postulate that pneumatization of bone surrounding the vestibular aqueduct may also be associated with hearing loss. Methods: 342 temporal bone CT scans performed at Children's Hospital of Michigan between January 2018 and December 2020 were reviewed. Scans were assessed for the presence, laterality, and degree of vestibular aqueduct pneumatization (PVA). Electronic medical record data was collected on age, gender, hearing status, medical comorbidities, and otologic comorbidities. Cases were secondarily reviewed to ensure validity. 159 patients were included in the final analysis; excluded scans included duplicates, patients with unknown hearing status, patients older than 21 years old, and patients with another known cause of hearing loss including inner ear malformations. Results: 17.6% of patients demonstrated vestibular aqueduct pneumatization. Hearing loss percentage was comparable between the PVA and non-pneumatized group (42.9% vs 42.0%), but hearing loss was more likely to be sensorineural in the PVA group (91.7% vs 80.0%). When patients with only newborn hearing data available were excluded from the PVA group, hearing loss percentage was higher in the PVA group (50% vs 42.0%). Patients with hearing loss in the PVA group were older than those without hearing loss (14.92 years old vs 10.67 years old). Conclusion: Hearing loss in enlarged vestibular aqueduct syndrome is typically progressive, bilateral, and sensorineural. Our preliminary findings suggest that vestibular aqueduct pneumatization may be another anomaly associated with a primarily sensorineural hearing loss. Further studies are needed to strengthen this postulated link.