A Historical Perspective on Surgical Manipulation of the Membranous Labyrinth for Treatment of Meniere's Disease

Meniere's disease is an inner ear disorder without a known cause. Endolymphatic hydrops is a swelling of the endolymph spaces that has been observed consistently on post-mortem histology in patients with a history of Meniere's disease but can occur in asymptomatic individuals and in association with other diseases. Since its discovery, Meniere's disease has been a disorder managed primarily by otolaryngologists. Surgical treatments, therefore, have accompanied attempts at medical management. Inspired by patients' sensations of ear fullness and later by the histologic findings of hydrops, surgeons began manipulating the membranous labyrinth to relieve episodes of vertigo while attempting to preserve hearing. This review highlights this history of manipulation of the membranous labyrinth. These procedures indicate a rich history of innovation that parallels developments in otologic surgery. The studies involving patients are uniformly retrospective, with some procedures performed first in animal models of endolymphatic hydrops. Many approaches were endorsed by eminent otologic surgeons. Surgeries on the endolymphatic sac are performed by some surgeons today; however, procedures on the membranous labyrinth resulted in similar symptomatic relief through a minimally invasive technique, in many cases performed using only local anesthetic. Episodic vertigo in patients with Meniere's disease is a distressing symptom, yet spontaneous remissions are common. The reports of procedures on the membranous labyrinth reviewed here consistently indicated fewer vertigo episodes. Variable degrees of hearing loss were common following these procedures, and many were abandoned. Additional innovative surgeries are inevitable, but we must understand better the relationships among endolymphatic hydrops, Meniere's disease pathophysiology, and patient symptoms.

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

Frontiers in Surgery ◽

10.3389/fsurg.2021.680260 ◽

2021 ◽

Vol 8 ◽

Author(s):

Jose E. Alonso ◽

Gail P. Ishiyama ◽

Rance J. T. Fujiwara ◽

Nancy Pham ◽

Luke Ledbetter ◽

...

Keyword(s):

Hearing Loss ◽

Endolymphatic Hydrops ◽

Meniere’S Disease ◽

Menière’S Disease ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Menière's Disease ◽

Vestibular Testing ◽

History Of ◽

Ménière's Disease

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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MR Imaging of Cochlear Modiolus and Endolymphatic Hydrops in Patients With Menière's Disease

Frontiers in Surgery ◽

10.3389/fsurg.2021.667248 ◽

2021 ◽

Vol 8 ◽

Author(s):

Rita Sousa ◽

Carla Guerreiro ◽

Tiago Eça ◽

Jorge Campos ◽

Leonel Luis

Keyword(s):

Endolymphatic Hydrops ◽

Spin Echo ◽

Meniere’S Disease ◽

Menière’S Disease ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Slice Thickness ◽

Menière's Disease ◽

Membranous Labyrinth ◽

Ménière's Disease

Background: Menière's disease (MD) is an inner ear disorder characterized by recurrent episodes of spontaneous vertigo, unilateral low-frequency sensorineural hearing loss, tinnitus, and aural fullness. Current diagnosis still often has to rely on subjective and audiometric criteria only, although endolymphatic hydrops is recognized as the pathophysiological substrate of the disease, having been demonstrated in anatomical pathological studies and by magnetic resonance (MRI). The modiolus has a close functional and anatomical relationship with the cochlear nerve and membranous labyrinth and can be evaluated with MRI but no data exist on the modiolar size in MD.Purpose: Our purpose is to examine the following hypothesis. Is cochlear modiolus smaller in symptomatic ears in MD?Methods: We used a retrospective 3 Tesla MR study (heavily T2-weighted 3D fast asymmetric spin-echo images and 0.5 mm slice thickness) comparing the mean modiolar area (MMA) in the index and best ears of eight patients with definite MD based on audiometric data. The obtained MMA values were compared against the audiometric data and the presence of vestibular endolymphatic hydrops.Results: No differences were seen in MMA between best and worst ears. Ears with a pure tone average (PTA) ≥25 dB and more pronounced endolymphatic hydrops showed lower MMA (not statistically significant). Two patients with extreme endolymphatic hydrops showed a noteworthy ipsilateral decrease in the cochlear modiolus area.Conclusion: No differences were seen in MMA between best and worst ears in definite MD. Worse hearing function (PTA ≥ 25dB) and more pronounced endolymphatic hydrops seem to be associated with lower MMA. This might be related to bone remodeling as a consequence of endolymphatic hydrops. Further research is needed to corroborate and explore these findings.

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The Correlation of a 2D Volume-Referencing Endolymphatic-Hydrops Grading System With Extra-Tympanic Electrocochleography in Patients With Definite Ménière's Disease

Frontiers in Neurology ◽

10.3389/fneur.2020.595038 ◽

2021 ◽

Vol 11 ◽

Author(s):

Baihui He ◽

Fan Zhang ◽

Hui Zheng ◽

Xiayu Sun ◽

Junmin Chen ◽

...

Keyword(s):

Endolymphatic Hydrops ◽

Meniere’S Disease ◽

Menière’S Disease ◽

Meniere's Disease ◽

Grading System ◽

Ménière’S Disease ◽

Menière's Disease ◽

Membranous Labyrinth ◽

Ménière's Disease ◽

3D Flair

Background: Although magnetic resonance imaging (MRI) of the membranous labyrinth and electrocochleography (ECochG) have been used to diagnose endolymphatic hydrops (ELH) in patients with Ménière's disease (MD), the relationship between imaging and ECochG is not well-documented.Objectives: This study evaluates the ELH using 3D-FLAIR MRI and extra-tympanic ECochG (ET-ECochG) and correlates the results from 3D-FLAIR MRI to those from ET-ECochG.Materials and Methods: 3D-FLAIR MRI images of 50 patients were assessed using a 2D volume-referencing grading system (VR scores, relative scores according to the known volumes of the cochlea, vestibule, and semicircular canals). Forty healthy subjects were included and compared to 51 definite MD ears of 50 patients while analyzing the ET-ECochG, which used a self-made bronze foil electrode. The amplitude ratio of the summating potential (SP) to the action potential (AP) (SP/AP) and the area ratio of SP to AP (Asp/Aap) were collected. Relative ELH grade scores were then correlated to ET-ECochG (SP/AP, Asp/Aap).Results: The VR scores showed a better correlation (r = 0.88) with the pure tone average (PTA), disease duration, and vertigo frequency of MD than the Bernaerts scores (grading the cochlea and vestibule separately) (r = 0.22). The SP/AP and Asp/Aap of the unilateral MD patients were statistically comparable to those measured in contralateral ears and the results between the definite MD ears with healthy ears were statistically comparable (p < 0.05). In a ROC analysis Asp/Aap (area under curve, AUC 0.98) significantly (p = 0.01) outperformed SP/AP (AUC 0.91). The total score of ELH, vestibular ELH, and cochlear ELH were also correlated with SP/AP and Asp/Aap. The strongest correlation was found between the Asp/Aap and cochlear ELH (r = 0.60).Conclusion: The 2D volume-referencing grading system was more meaningful than the Bernaerts scores. A correlation was found between ELH revealed by 3D-FLAIR MRI and the SP/AP of ET-ECochG in evaluating definite MD patients. The Asp/Aap appeared a more sensitive and reliable parameter than SP/AP for diagnosing the ELH of the membranous labyrinth.

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Association between Chronic Obstructive Pulmonary Disease and Ménière’s Disease: A Nested Case–Control Study Using a National Health Screening Cohort

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph18094536 ◽

2021 ◽

Vol 18 (9) ◽

pp. 4536

Author(s):

So-Young Kim ◽

Chang-Ho Lee ◽

Dae-Myoung Yoo ◽

Chan-Yang Min ◽

Hyo-Geun Choi

Keyword(s):

Meniere’S Disease ◽

Menière’S Disease ◽

Chronic Obstructive ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Menière's Disease ◽

Obstructive Pulmonary Disease ◽

Region Of Residence ◽

History Of ◽

Ménière's Disease

This study explored the relation between Ménière’s disease and chronic obstructive pulmonary disease (COPD). The ≥40-year-old population of the Korean National Health Insurance Service-Health Screening Cohort was included. In total, 7734 Ménière’s disease patients and 30,936 control participants were enrolled. Control participants were matched for age, sex, income, and region of residence with Ménière’s disease participants. The odds of having Ménière’s disease given a history of COPD were analyzed using conditional logistic regression. Subgroup analyses were conducted according to age, sex, income, and region of residence. The odds of having Ménière’s disease were found to be 1.18-fold higher with a history of COPD than with no history of COPD (95% confidence intervals (CI) = 1.06–1.32, E-value (CI) = 1.64 (1.31)). The ≥60 years old, male, low-income, and rural subgroups showed increased odds of developing Ménière’s disease when a history of COPD was reported. A history of COPD was associated with an increased risk of Ménière’s disease in the adult population.

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