Visualization and clinical relevance of the endolymphatic duct and sac in Ménière's disease

Lisa M H de Pont; Maartje T P M Houben; Thijs O Verhagen; Berit M Verbist; Mark A van Buchem; Claire C Bommeljé; Henk M Blom; Sebastiaan Hammer

doi:10.3389/fneur.2023.1239422

Visualization and clinical relevance of the endolymphatic duct and sac in Ménière's disease

Front Neurol. 2023 Aug 31:14:1239422. doi: 10.3389/fneur.2023.1239422. eCollection 2023.

Authors

Lisa M H de Pont^{1

2}, Maartje T P M Houben^{1

3}, Thijs O Verhagen^{1

3}, Berit M Verbist², Mark A van Buchem², Claire C Bommeljé³, Henk M Blom^{3

4

5}, Sebastiaan Hammer¹

Affiliations

¹ Department of Radiology, Haga Teaching Hospital, The Hague, Netherlands.
² Department of Radiology, Leiden University Medical Center, Leiden, Netherlands.
³ Department of Otorhinolaryngology, Haga Teaching Hospital, The Hague, Netherlands.
⁴ Department of Otorhinolaryngology, Leiden University Medical Center, Leiden, Netherlands.
⁵ Department of Otorhinolaryngology, Antwerp University Hospital, Antwerp, Belgium.

Abstract

Background: Ménière's disease (MD) is a chronic inner ear disorder with a multifactorial etiology. Decreased visualization of the endolymphatic duct (ED) and sac (ES) is thought to be associated with MD, although controversy exists about whether this finding is specific to MD. Recent literature has revealed that two distinct ES pathologies, developmental hypoplasia and epithelial degeneration, can be distinguished in MD using the angular trajectory of the vestibular aqueduct (ATVA) or ED-ES system as a radiographic surrogate marker. It has been suggested that these two subtypes are associated with distinct phenotypical features. However, the clinical differences between the ATVA subtypes require further validation.

Research objective: The objective of this study is to investigate whether (1) non-visualization of the ED-ES system is a discriminative radiological feature for MD in a cohort of vertigo-associated pathologies (VAPs) and whether (2) different angular trajectories of the ED-ES system in MD are associated with distinguishable clinical features.

Setting: The study was conducted in the Vertigo Referral Center (Haga Teaching Hospital, The Hague, the Netherlands).

Methods: We retrospectively assessed 301 patients (187 definite MD and 114 other VAPs) that underwent 4h-delayed 3D FLAIR MRI. We evaluated (1) the visibility of the ED-ES system between MD and other VAP patients and (2) measured the angular trajectory of the ED-ES system. MD patients were stratified based on the angular measurements into α_exit ≤ 120° (MD-120), α_exit 120°-140° (MD-intermediate), or α_exit ≥ 140° (MD-140). Correlations between ATVA subgroups and clinical parameters were evaluated.

Results: Non-visualization of the ED-ES system was more common in definite MD patients compared with other VAPs (P < 0.001). Among definite MD patients, the MD-140 subtype demonstrated a longer history of vertigo (P = 0.006), a higher prevalence of bilateral clinical disease (P = 0.005), and a trend toward a male preponderance (p = 0.053). No significant differences were found between ATVA subgroups regarding the presence or severity of auditory symptoms, or the frequency of vertigo attacks.

Conclusion: Non-visualization of the ED-ES system is significantly associated with MD. Among MD patients with a visible ED-ES system, we demonstrated that the MD-140 subtype is associated with a longer disease duration, a higher prevalence of bilateral MD, and a trend toward a male preponderance.

Keywords: MRI; Ménière; clinical features; endolymphatic duct; endolymphatic sac.

Grants and funding

BV received grants from Oticon and Advances Bionics paid to her institution. SH received a Radiology Research Fund from the Radiological Society of the Netherlands. The funder was not involved in the study design, collection, analysis, interpretation of data, the writing of this article, or the decision to submit it for publication.