Impact of Superior Canal Dehiscence Syndrome on Health Utility Values: A Prospective Case-Control Study

Abstract

Introduction: Superior canal dehiscence syndrome (SCDS) is a condition characterized by a defect in the bone overlying the superior semicircular canal, creating a third mobile window into the inner ear. Patients can experience disabling symptoms and opt for surgical management. Limited data are available on the impact of SCDS on health-related quality of life (HRQoL) and disease-specific HRQoL more specifically. Objective: To perform a prospective analysis on generic HRQoL in SCDS patients compared to healthy age-matched controls. Methods: A prospective study was performed on patients diagnosed with SCDS and who did not undergo reconstructive surgery yet. Patients were recruited between November 2017 and January 2020 and asked to complete the Health Utility Index (HUI) Mark 2 (HUI2)/Mark 3 (HUI3) questionnaire. For the control group, age-matched participants without otovestibular pathology or other chronic pathology were recruited. The multi-attribute utility function (MAUF) score was calculated for the HUI2 and HUI3. Results of both groups were compared using the Mann-Whitney U test. Results: A total of 20 patients completed the questionnaire. Age ranged from 37 to 79 years with a mean age of 56 years (45% males and 55% females). The control group consisted of 20 participants with a mean age of 56.4 years and ranged from 37 to 82 years (35% males and 65% females). For the case group, median HUI2 MAUF score was 0.75 and median HUI3 MAUF score was 0.65. For the control group, the median scores were 0.88 and 0.86 respectively. There was a statistically significant difference for both HUI2 ( p = 0.024) and HUI3 ( p = 0.011). SCDS patients had a worse generic HRQoL than age-matched healthy controls. One patient with unilateral SCDS had a negative HUI3 MAUF score (−0.07), indicating a health-state worse than death. Conclusion: SCDS patients have significantly lower health utility values than an age-matched control group. This confirms the negative impact of SCDS on generic HRQoL, even when using an instrument that is not designed to be disease-specific but to assess health state in general. These data can be useful to compare impact on HRQoL among diseases.

Case Report: Bow Hunter Syndrome—One Reason to Add Non-gravity Dependent Positional Nystagmus Testing to Your Clinical Neuro-Otologic Exam

“…Patients with vestibular conditions are known to report HUI scores lower than healthy controls. For example, HUI scores for superior semicircular canal dehiscent syndrome are reported to be 0.65, similar with unilateral vestibular hypofunction (0.63), but much higher than HUI scores reported by those with bilateral vestibular hypofunction (0.39) (23,24). As comparison, patient with diabetes report HUI scores ∼0.8 (25).…”

Association Between Superior Canal Dehiscence Syndrome and Anxiety and Depressive Disorders

“…Evidence regarding the prevalence of psychiatric disorders in rare disease patients is conflicting, and there is limited research on the specific prevalence in SCDS patient populations. 10,11 We determined the prevalence of anxiety and depressive disorders in patients with SCDS and controlled for potentially confounding variables via a matched cohort design. Our data indicate that SCDS is associated with significantly higher rates of anxiety and depressive disorders relative to a matched control population.…”

“…8,9 Research evaluating psychiatric illness among patients with SCDS is limited to qualitative reports of associated depression and anxiety and one study demonstrating decreased health-related quality of life. 10,11 Our study aims to examine the prevalence of anxiety and depressive disorders in patients with SCDS diagnosed within a metropolitan medical system between 2016 and 2021.…”

The Supine Superior Semicircular Canal Dehiscence Test

“…SCDS is characterized by an abnormal opening in the roof of the superior semicircular canal, producing an aberrant third mobile window within the vestibular labyrinth. Individuals with SCDS reported worse health-related quality of life than age-matched healthy controls (Ocak et al, 2020). Their reported symptoms can interfere with social activities, leading to alienation and possibly depression (Öhman et al, 2018).…”