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In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears
Published on: February 21, 2018
Is there progression of endolymphatic hydrops in Ménière's disease? Longitudinal magnetic resonance study
Rita Sousa1,2, Mariana Lobo3, Hugo Cadilha4,5
1Neuroradiology Department, Hospital Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Avenida Professor Egas Moniz, 1649-028, Lisbon, Portugal. ritafsousa1@gmail.com.
Background:
Endolymphatic hydrops (EH) is universal in Ménière´s disease (MD). Given its chronic course, with variable interval before complete clinical picture is installed, it seems relevant to understand the progression of vestibular EH and hemato-perilymphatic barrier disruption in patients with MD and monosymptomatic presentations.
Methods:
239 consecutive patients were referred to us with suspected hydropic ear disease. 50 individuals accepted to participate in this study-final longitudinal sample included 24 patients (7 D1, 7 D2, 10 D3). Control group included ten patients. At recruitment, a clinical and MRI re-evaluation was done (3T, intravenous technique) (MR2) and 2 years after MRI was repeated (MR3). Previous MRI (MRI1) were retrospectively evaluated. Patients were classified as definite (D1), possible (D2) and atypical (D3-monosymptomatic) MD. Control group included non-typical symptoms (C2/C3) and 6 asymptomatic (C1). Vestibular endolymphatic ratio (vER) and grading, presence/absence of cochlear EH, asymmetry of cochlear perilymphatic enhancement, and rate of progression of vER were assessed by two independent neuroradiologists and compared between patient and control groups (index ear).
Results:
EH was universal and pronounced in D1 and remained stable. vER progression was more variable and higher in some D3 patients (index ear worse) and in D2 (non-index), although this observation was not statistically significant.
Conclusions:
Considering that many probable and monosymptomatic presentations progress years later into definite MD and given the bilateral tendency of the disease, these findings may indicate that there is an initial accelerated worsening of EH in initial stages of the disease. These data should be confirmed with controlled and larger sample studies.
Insights
Endolymphatic hydrops (EH) in Ménière´s disease (MD) appears to worsen rapidly in early stages. This progression, particularly in vestibular EH, suggests potential for earlier intervention in patients with monosymptomatic presentations.
Area of Science:
- Otolaryngology
- Neurology
- Radiology
Background:
- Endolymphatic hydrops (EH) is a hallmark of Ménière´s disease (MD).
- Understanding the progression of vestibular EH and barrier disruption is crucial for early diagnosis and management of MD.
- Monosymptomatic presentations of MD can evolve over time, necessitating longitudinal studies.
Purpose of the Study:
- To investigate the progression of vestibular endolymphatic hydrops (vER) and hemato-perilymphatic barrier disruption in patients with definite (D1), possible (D2), and atypical/monosymptomatic (D3) Ménière´s disease (MD).
- To compare the rate of progression of vER between patient groups and a control group.
Main Methods:
- A longitudinal study involving 24 patients with MD (D1, D2, D3) and 10 controls.
- Serial 3T MRI with intravenous contrast (MR2 and MR3) over two years to assess vestibular endolymphatic ratio (vER), cochlear EH, and perilymphatic enhancement.
- Retrospective evaluation of initial MRI (MRI1) and comparison of vER progression between groups.
Main Results:
- Endolymphatic hydrops (EH) was consistently observed and pronounced in definite MD (D1) and remained stable.
- Vestibular endolymphatic ratio (vER) progression showed greater variability and higher rates in some atypical (D3) and possible (D2) MD patients, though not statistically significant.
- Asymmetry in cochlear perilymphatic enhancement was also noted.
Conclusions:
- Findings suggest a potential initial accelerated worsening of EH in the early stages of MD, even in monosymptomatic presentations.
- This early progression may indicate a window for intervention before the disease fully manifests.
- Larger, controlled studies are recommended to confirm these observations and their clinical implications.

