Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease

Maxwell, Anne K.; Ishiyama, Gail; Karnezis, Stellios; Ishiyama, Akira

doi:10.1097/mao.0000000000003051

Cited by 8 publications

(7 citation statements)

References 33 publications

(50 reference statements)

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“…We speculate this patient progressed from cochlear EH to Meniere's disease, and that a subset of the isolated cochlear hydrops patients may represent an early form of Meniere's disease. Similarly, in our study of isolated saccular hydrops, one patient presented first with only sudden SNHL and then 3 years later developed full spectrum Meniere's disease with recurrent spells of roomspinning vertigo and the MRI demonstrated cochlear, saccular, and utricular hydrops (26).…”

Section: Discussionsupporting

confidence: 69%

“…It is particularly instructive to compare the present study cohort of patients with MRI demonstrating isolated cochlear hydrops to patients presenting with isolated saccular hydrops from our prior study. In the case of isolated saccular hydrops, the clinical presentation is the full spectrum of Meniere's disease with 12 out of 18 patients meeting criteria for Meniere's disease and four out of 18 meeting criteria for delayed endolymphatic hydrops ( 26 ). Thus, the presence of endolymphatic hydrops in the saccule in the case of sudden SNHL may be predictive of the development of Meniere's disease.…”

Section: Discussionmentioning

confidence: 99%

“…Demographically, it appears cochlear hydrops more often affects men (mean age 67.6 years, 78% male). However, females were the majority in isolated saccular hydrops (mean age 60.8 years, 61% female) ( 26 ). Larger studies are indicated to evaluate these demographic differences between patients with isolated cochlear hydrops and those with isolated saccular hydrops.…”

Section: Discussionmentioning

confidence: 99%

“…In Gurkov et al, the degree of audiovestibular hydrops correlated with a progressive loss of auditory function, and a trend toward decrement of amplitude of VEMP ( 27 ). The presence of isolated saccular hydrops was indicative of Meniere's disease, and correspondingly 53% of those tested had a significant caloric paresis and 29% had reduced or absent VEMP responses ( 26 ).…”

Section: Discussionmentioning

confidence: 99%

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Cochlear Meniere's: A Distinct Clinical Entity With Isolated Cochlear Hydrops on High-Resolution MRI?

et al. 2021

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Objective: Describe the clinical characteristics of patients with isolated cochlear endolymphatic hydrops (EH).Study design: Clinical case series.Setting: Tertiary Neurotology referral clinic.Patients: All subjects presenting to a University Neurotology clinic during a 1-year period from July 2015 until August 2016 who had isolated cochlear EH on MRI. Patients with a history of temporal bone surgery prior to the MRI were excluded.Intervention: High-resolution delayed-intravenous contrast MRI.Main outcome measures: Audiometric and vestibular testing, clinical history analysis.Results: 10 subjects demonstrated isolated, unilateral cochlear hydrops on MRI. None of these patients met the criteria for Meniere's disease. Mean age of the group was 66.4 years and most were males (70%). Unilateral aural fullness (70%), tinnitus (80%), and hearing loss (90%) were frequently observed. Only one patient presented with unsteadiness (10%) and one patient had a single isolated spell of positional vertigo 1 month prior to the MRI (10%) but no further vertigo spells in the 4 years following the MRI. The mean PTA was 37.8 dB which was significantly decreased from the non-affected ear with PTA of 17.9 (p < 0.001). One patient developed vertiginous spells and unsteadiness 4 years after initial presentation and a repeat MRI revealed progression to utricular, saccular and cochlear hydrops. Vestibular testing was obtained in five patients with one patient presenting with 50% caloric paresis and all others normal. The most common treatment tried was acetazolamide in seven patients with 86% reporting subjective clinical improvement. Two out of the 10 patients had a history of migraine (20%).Conclusions: Patients with MRI exhibiting isolated cochlear EH present with predominantly auditory symptoms: mild to moderate low-frequency hearing loss, aural fullness, tinnitus without significant vertigo. Isolated cochlear hydrops is more common in males, average age in mid-60's and there is a low comorbidity of migraine headaches. This contrasts significantly with patients with isolated saccular hydrops on MRI from our prior studies. We believe that isolated cochlear EH with hearing loss but no vertigo is distinct from Meniere's disease or its variant delayed endolymphatic hydrops. We propose that cochlear Meniere's disease represents a distinct clinical entity that could be a variant of Meniere's disease.

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Section: Discussionsupporting

confidence: 69%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Cochlear Meniere's: A Distinct Clinical Entity With Isolated Cochlear Hydrops on High-Resolution MRI?

et al. 2021

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“…Despite their strong association, the relationship between EH and MD remains unclear. In the last decade, the application of delayed gadolinium (Gd)-enhanced MRI has provided novel insights in the clinical effect of EH (22)(23)(24)(25)(26)(27)(28)(29)(30). The degree of EH is reported to correspond with disease duration and the degree of sensorineural hearing loss in MD patients (27,28,31,32).…”

Section: Introductionmentioning

confidence: 99%

Hydropic Ear Disease: Correlation Between Audiovestibular Symptoms, Endolymphatic Hydrops and Blood-Labyrinth Barrier Impairment

et al. 2021

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Research Objective: To investigate the correlation between clinical features and MRI-confirmed endolymphatic hydrops (EH) and blood-labyrinth barrier (BLB) impairment.Study Design: Retrospective cross-sectional study.Setting: Vertigo referral center (Haga Teaching Hospital, The Hague, the Netherlands).Methods: We retrospectively analyzed all patients that underwent 4 h-delayed Gd-enhanced 3D FLAIR MRI at our institution from February 2017 to March 2019. Perilymphatic enhancement and the degree of cochlear and vestibular hydrops were assessed. The signal intensity ratio (SIR) was calculated by region of interest analysis. Correlations between MRI findings and clinical features were evaluated.Results: Two hundred and fifteen patients with MRI-proven endolymphatic hydrops (EH) were included (179 unilateral, 36 bilateral) with a mean age of 55.9 yrs and median disease duration of 4.3 yrs. Hydrops grade is significantly correlated with disease duration (P < 0.001), the severity of low- and high-frequency hearing loss (both P < 0.001), and the incidence of drop attacks (P = 0.001). Visually increased perilymphatic enhancement was present in 157 (87.7%) subjects with unilateral EH. SIR increases in correlation with hydrops grade (P < 0.001), but is not significantly correlated with the low or high Fletcher index (P = 0.344 and P = 0.178 respectively). No significant differences were found between the degree of EH or BLB impairment and vertigo, tinnitus or aural fullness.Conclusion: The degree of EH positively correlates with disease duration, hearing loss and the incidence of drop attacks. The BLB is impaired in association with EH grade, but without clear contribution to the severity of audiovestibular symptoms.

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Comparing the Saccule‐to‐Utricle Ratio in Early‐ Versus Late‐Stage Meniere's Disease Patients

Deng,

Wu,

Chen

et al. 2024

The Laryngoscope

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ObjectiveTo compare the saccule‐to‐utricle ratio in early‐ versus late‐stage Meniere's disease (MD) patients based on magnetic resonance imaging (MRI) images.MethodsIn this retrospective study, we performed 3‐dimensional real inversion recovery (3D‐real IR) MRI 24 h after intratympanic gadolinium administration in unilateral MD patients at early‐stage (n = 56) and late‐stage (n = 70). Two radiologists independently graded endolymphatic hydrops (EH) and the saccule‐to‐utricle ratio inversion (SURI) was compared between the two groups. Furthermore, early‐stage MD patients were further divided into two subgroups based on disease duration: ≤6 months (n = 20) and >6 months (n = 36) and the SURI was compared.ResultsAmong the 56 patients in the early‐stage group, 26 cases (46.43%) exhibited an enlarged saccule that is larger than the utricle, showing SURI. In contrast, among the late‐stage MD, only four cases (5.71%) showed SURI (p < 0.001). In the early‐stage MD subgroup with a disease duration of ≤6 months, the proportion of SURI was 70% (14/20), which was higher than that in the subgroup with a disease duration of >6 months (33.33%, 12/36, p = 0.02).ConclusionSURI may serve as an effective imaging marker for diagnosis of early‐stage MD. Our finding suggests that endolymphatic hydrops in MD may primarily originate from the saccule.Level of EvidenceLevel 3 Laryngoscope, 2024

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Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease

Cited by 8 publications

References 33 publications

Cochlear Meniere's: A Distinct Clinical Entity With Isolated Cochlear Hydrops on High-Resolution MRI?

Cochlear Meniere's: A Distinct Clinical Entity With Isolated Cochlear Hydrops on High-Resolution MRI?

Hydropic Ear Disease: Correlation Between Audiovestibular Symptoms, Endolymphatic Hydrops and Blood-Labyrinth Barrier Impairment

Comparing the Saccule‐to‐Utricle Ratio in Early‐ Versus Late‐Stage Meniere's Disease Patients

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