Vestibular System and Hearing Involvement in Patients with Turner Syndrome

Background: Turner syndrome is a genotypic disorder in females characterized by the total or partial absence of an X chromosome. While cardiovascular issues and sensorineural hearing loss are well-documented, vestibular system involvement remains understudied. This study aims to examine vestibular system involvement in patients with Turner syndrome and assess if they exhibit a higher prevalence of peripheral vestibular pathology compared to the general population. Methods: A retrospective longitudinal study was conducted with 21 Turner syndrome patients and 21 age-matched controls. Evaluations included clinical history, otoscopy, pure tone audiometry, the Video Head Impulse Test (vHIT) to measure vestibulo-ocular reflex gain, and computerized dynamic posturography, specifically the Sensory Organization Test (SOT) and Stability Limits Analysis. Results: Turner syndrome patients showed significantly higher hearing thresholds across all frequencies compared to controls (p < 0.001). In the vHIT, 30% of the Turner group presented pathological results, with significant gain reductions in the right horizontal and left posterior semicircular canals. Posturography revealed a significant reduction in overall stability (p = 0.006) and a significantly lower vestibular index (p = 0.011) in the Turner group. Additionally, patients with Turner syndrome demonstrated significant impairments in directional control, reaction time, and excursion points during Stability Limits Analysis. Conclusions: Patients with Turner syndrome are more likely to experience vestibular disorders, a finding likely associated with estrogen deficiency and the loss of its protective effect on the inner ear. These results highlight the necessity of including vestibular and posturographic assessments in the routine clinical follow-up of these patients to facilitate early detection and rehabilitation, even in the absence of overt symptoms like vertigo.