An Update on Endotyping Meniere’s Patients – Implications for Diagnosis and Therapy of Meniere’s Disease

Introduction Meniere’s disease (MD) has been recently associated with either of two histopathologies of the endolymphatic sac (ES), i.e. ES degeneration or ES hypoplasia. These ES pathologies (“endotypes”) can be diagnosed using clinical imaging data. This presentation will give an overview on the therapeutic and prognostic implications of diagnosing endotypes in MD.

Methods The angular trajectory of the vestibular aqueduct was used as a radiographic surrogate marker to determine ES pathologies in clinical MD patients (n = 72). Clinical data was collected and compared between the MD patient groups exhibiting either a degenerative (MD-dg) or hypoplastic (MD-hp) ES pathology. Lastly, we investigated the presence of ES hypoplasia in the unaffected ear as a predictor for future bilateral MD in unilateral MD-hp patients.

Results We found several phenotypic differences between MD-dg and MD-hp patients, such as a higher average of vertigo attacks and more severely reduced vestibular function in MD-dg patients. MD-hp patients presented with higher frequencies of bilateral affection, positive family history of MD, and radiographic signs of semicircular canal dehiscence. In MD-hp patients, development of bilateral MD was exclusively observed in patients with bilateral ES hypoplasia.

Conclusion  Exploring different clinical disease features (phenotypes) associated with the two ES pathologies reveals crucial information on the etiology, pathophysiology, and prognosis of MD. This information is important in counselling and treating Meniere’s patients and may help to select more personalized treatment regimens.

Publication History

Article published online:
12 May 2023