Atypical Meniere's Disease Research Articles

Is Vestibular Meniere's Disease Associated With Endolymphatic Hydrops?

Background: Vestibular Meniere's disease (American Academy of Ophthalmology and Otolaryngology, 1972) also known as possible Meniere's disease (American Academy of Otolaryngology Head and Neck Surgery, 1995) or vestibular type of atypical Meniere's disease (V-AMD) (Japan Society for Equilibrium Research, 2017) is characterized by an episodic vertigo without hearing loss. Though named as Meniere's disease (MD), this entity may not be caused solely by endolymphatic hydrops (EH).Objective: To estimate the role of EH in vestibular Meniere's disease in comparison with definite Meniere's disease.Methods: Thirty patients with unilateral definite MD and 16 patients with vestibular Meniere's disease were included. Those who met the criteria for definite or probable vestibular migraine were excluded. All patients underwent vestibular assessments including inner ear MRI 4 h after intravenous gadolinium injection, bithermal caloric testing, directional preponderance of vestibulo-ocular reflex in rotatory chair test, cervical- and ocular-vestibular evoked myogenic potential, stepping test, dizziness handicap inventory (DHI), and hospital anxiety and depression scale (HADS). All above tests and frequency/duration of vertigo spells were compared between vestibular Meniere's disease and MD.Results: Even in unilateral MD, cochlear and vestibular endolymphatic hydrops (c-, v-EH) were demonstrated not only in the affected side but also in the healthy side in more than half of patients. Positive rate of v-EH in vestibular Meniere's disease (68.8%) was as high as that of MD (80%). In vestibular Meniere's disease, the number of bilateral EH was higher in the vestibule (56.3%) than that in the cochlea (25.0%). There were no differences in vestibular tests and DHI between vestibular Meniere's disease and MD; however, the frequency of vertigo spells was lower in vestibular Meniere's disease (p = 0.001). The total HADS score in the MD group was significantly higher than that in the vestibular Meniere's disease group.Conclusions: MD is a systemic disease with bilateral involvement of inner ears. V-EH is a major pathophysiology of vestibular Meniere's disease, which would precede c-EH in the development of vestibular Meniere's disease, a milder subtype of MD. MRI is useful for differentiating MD from other vertigo attacks caused by different pathologies in bringing EH into evidence.

Diagnostic and therapeutic strategies for Meniere's disease of the Japan Society for Equilibrium Research

ObjectiveWe provided diagnostic and therapeutic strategies for Meniere's disease in accordance with Japanese Clinical Practice Guideline of Meniere's disease and delayed endolymphatic hydrops 2nd ed. Tokyo: Kanehara Shuppan; 2020 edited by the Japan Society for Equilibrium Research. MethodsThe Committee for Clinical Practice Guidelines was entrusted with a review of the scientific literature on the above topic. Clinical Questions (CQs) concerning the treatment for Meniere's disease were produced, and the literature according to each of them including CQ was searched. The recommendations are based on the literature review and the expert opinion of a subcommittee. ResultsDiagnosis criteria of Meniere's disease are classified into Meniere's disease with typical cochlear and vestibular symptoms, and atypical Meniere's disease with either cochlear symptoms or vestibular symptoms. Treatment of Meniere's disease was composed of lifestyle changes, medications such as anti-vertigo drugs and diuretics, middle ear positive pressure treatment, and selective destruction of the vestibule. ConclusionMeniere's disease is diagnosed based on clinical histories and examination findings after processes of differential diagnosis. Treatment option of the disease should be selected in order of invasiveness, according to the severity of the disease and the response to each treatment.

Atypical Meniere Disease: Case Report of a Patient Treated as Sudden Hearing Loss

Meniere's disease is an idiopathic disease involving the inner ear, which is characterized by fluctuating low frequency sensorineural hearing loss, tinnitus, aural fullness, and recurrent vertigo. We could see the Meniere's disease without vestibular symptom in some cases. These cases have been reported as atypical (Cochlear) Meniere's disease. We have evaluated a patient of atypical Meniere's disease followed as sudden hearing loss with fluctuating low tone type sensorineural hearing loss without vertigo. Sudden deafness, cochlear sensorineural hearing loss, chronic progressive sensorineural hearing loss, presenile presbycusis, noise induced and autoimmune sensorineural hearing loss may be confused in diagnosis of Meniere disease. Key words: Atypical Meniere Disease; Hearing Loss; Diagnosis.

Endolymphatic hydrops revealed by magnetic resonance imaging in patients with atypical Meniere's disease

Conclusion: Our study showed that patients with atypical Meniere's disease had endolymphatic hydrops (EH) in both the cochlea and the vestibule. Using an imaging method, we provide evidence that atypical Meniere's disease is a true variant of classic Meniere's disease. Objectives: The aim of present study was to investigate images of the endolymphatic space in patients with atypical Meniere's disease (cochlear and vestibular Meniere's disease). Methods: Sixty-four patients divided into two groups were enrolled in this study. The first group included 36 patients who had fluctuating hearing loss without vertigo, as candidates for cochlear Meniere's disease (CMD). The second group included 28 patients who had recurrent vertigo without hearing loss, as candidates for vestibular Meniere's disease (VMD). The patients underwent 3 T magnetic resonance imaging (MRI) after injection of gadolinium enhancement medium intratympanically or intravenously. We evaluated EH using MRI. Results: Of 56 ears (36 patients) with CMD, 38 showed EH in the cochlea and 44 showed EH in the vestibule. Of 56 ears (28 patients) with VMD, 29 showed EH in the cochlea and 47 showed EH in the vestibule. The VMD group had a significant vestibular predominance in EH distribution whereas the CMD group showed no significant regional predominance of EH.

いわゆる「自律神経失調症」と診断された眩暈例における手掌冷温刺激試験とシエロンテストについて

いわゆる「自律神経失調症」と診断された眩暈例における手掌冷温刺激試験とシエロンテストについて

Semi-quantitative evaluation of endolymphatic hydrops by bilateral intratympanic gadolinium-based contrast agent (GBCA) administration with MRI for Meniere's disease

Conclusion: Bilateral intratympanic administration of a gadolinium-based contrast agent (GBCA) in MRI was successfully performed and proved to be beneficial in the semi-quantitative evaluation of endolymphatic hydrops. Such image-based diagnosis will lead to re-revaluation and reclassification of the diagnostic criteria for Meniere's disease (MD). Objective: To visualize endolymphatic hydrops semi-quantitatively in patients with MD, by using bilateral intratympanic GBCA administration with MRI. Patients and methods: A total of 13 patients were evaluated, including 12 with MD and one with acute low-tone sensorineural hearing loss. Diluted gadodiamide (a kind of GBCA) was administered to the bilateral tympanic cavity by injection through the tympanic membrane. After 24 h, the endolymphatic hydrops was evaluated with a 3.0 T MR scanner. The areas enhanced by gadodiamide were measured semi-quantitatively. Results: Three-dimensional, fluid-attenuated inversion recovery (3D-FLAIR) MRI showed that the gadodiamide successfully penetrated the round window membrane, entering the perilymphatic space and delineating the gadodiamide-enhanced perilymphatic and gadodiamide-negative endolymphatic spaces of the inner ear. All the patients with MD showed a reduced gadodiamide-enhanced area representing the perilymphatic space, and the quantitative ratio was 0.15 to 0.85. Furthermore, endolymphatic hydrops was also demonstrated in the patient with atypical MD who had fluctuating low frequency sensorineural hearing loss without vertigo.

CT and MR imaging of intralabyrinthine schwannoma: report of two cases and review of the literature.

Two cases of intralabyrinthine schwannoma were studied with computed tomography (CT) and magnetic resonance (MR) imaging. On CT scans, a soft-tissue mass was identified in the round window niche in both cases. Widening of the basilar turn of the cochlea and characteristic erosion of the promontory were noticed in one case. Preoperative MR imaging (performed in only one case) revealed a soft-tissue mass in the labyrnth, extending into the round window niche. Findings at CT and MR imaging are discussed, and a differential diagnosis is given. The literature is reviewed. The authors' findings suggest that CT and MR imaging may prove very valuable in the previously difficult preoperative diagnosis of these tumors. In the setting of progressive sensorineural hearing loss, atypical Meniere disease, or recurrent vertigo, the presence of a mass in the labyrinth or labyrinthine windows- delineated on CT or MR images, despite a normal internal auditory canal, cerebellopontine angle, or brain stem - is highly suggestive of intralabyrinthine schwannoma.

Elicitation of nystagmus by a new thermal test to the hand in the patients with atypical Meniere's disease.

As a causal condition of vertigenous attacks in Meniere's, the labile reaction of the autonomic nervous system has been considered to contribute a fundamental role.To assess the close correlation between the autonomic nerve and vestibular system, a new thermal test of the hand immersed in water at 20°C and 45°C was employed as an autonomic stimulus to the patients with atypical Meniere's disease. Spontaneous nystagmus was elicited in 40 to 70% of the patients during stimulation. Only 20% of the same group showed normal values in Schellong's test.These appears to be a complicated correlation between neural mechanisms and vegetative-somatoreactions.

A clinical classification of vertigo.

Abstract : A comprehensive survey of the literature on the clinical entities epidemic vertigo, acute toxic labyrinthitis, atypical Meniere's disease, and pseudo-Meniere's syndrome was made. Although these 5 syndromes have more similar than dissimilar characteristics, slight differences are pointed out. A review of 25 consultation patients for single or recurrent episodes of vertigo without significant cochlear involvement is presented. Finally, a clinical classification which offers promise is suggested. This classification system is based on anatomic location, not etiology, and is similar to Lederer's localization of facial nerve lesions by presenting symptoms. It classifies vertigo as that prodced by: (1) peripheral vestibulopathy, a term devised for involvement of the peripheral anatomic portions of the vestibular system; (2) central vestibulopathy, an involvement of the retrolabyrinthine central anatomic areas of the vestibular system; (3) idiopathic vestibulopathy, in which vertigo is produced by either an unknown cause or, in the case of postural vertigo, by involvement of either the central or p ripheral regions. As a catchall term, the use of idiopathic vestibulopathy or idiopathic vertigo is recommended. (Author)