(0958) Prevalence of Vestibular Pathology in Patients With Congenital Inner Ear Malformation

Introduction: Patients with congenital inner ear malformations (IEM) may be at greater risk of vestibular symptoms due to involvement of vestibular structures. The prevalence of such pathologies has not been well articulated. This study aims to determine the risk of vestibular pathology in patients with IEM.

Methods: A retrospective analysis was conducted using de-identified medical data from 117,065,877 patients in the last 20 years at 83 large healthcare organizations in the TriNetX global federated health research network. From them, 5,943 IEM patients were identified. Prevalence and relative risk of vertigo and BPPV was assessed between the IEM and general populations across sexes and between different age ranges. The secondary outcome was to determine if presence of hearing loss is associated with development of vertigo in IEM patients.

Results: IEM patients have an increased risk of vertigo than the general population (RR: 20.2, 95% CI: 19.9-20.5). This remained true above the age of 50 (RR: 19.2, 95% CI: 18.6-19.8) and above the age of 65 (RR: 17.3, 95% CI: 16.3-18.3). They are also at greater risk of vertigo at a younger age (IEM mean age: 49.3; general mean age: 63.4; p < 0.0001) and at a greater risk of BPPV (RR: 5.89, 95% CI: 5.82-5.97). IEM patients who have hearing loss are at a significantly increased risk of vertigo (RR: 2.87, 95% CI: 2.5-3.2) and those with vertigo have an elevated risk of hearing loss (RR: 1.254, 95% CI: 1.251-1.257). Additionally, in the general and IEM populations, age >50, age >65, or female sex was not associated with increased risk of vertigo.

Conclusions: This analysis establishes the increased risk of vestibular pathologies in IEM patients and suggests that vertigo may be a presenting symptom of IEM. Additionally, the elevated risk of hearing loss in IEM patients with vertigo (and the reverse) is an important association for clinicians to be aware of to ensure that they appropriately identify and screen IEM patients who may benefit from vestibular therapy. This analysis also challenges traditional clinical commentary that vertigo is more prevalent in older patients and women.