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The ultrastructure of the endolymphatic sac in Ménière's disease.

Meniere's disease is a well recognized clinical entity, yet the etiology and pathophysiology of the disease is not fully understood. Impaired endolymphatic sac function resulting in faulty reabsorption of endolymph has been implicated in the production of the disease. The histopathological findings from biopsied sac specimens and observed by transmission (TEM) and scanning (SEM) electronmicroscopy in two patients with early Meniere's disease are presented and discussed. Extensive subepithelial fibrosis with loss of vascularity and obliteration of portions of the lumen of the endolymphatic sac by an ingrowth of collagen is noted in both specimens. The implications of these findings are discussed and the need for more TEM and SEM studies of inner ear disorders is stressed.

Adult↗

The history of Meniere's disease.

Prosper Meniere is widely credited with the first description of endolymphatic hydrops. Meniere's discovery was made possible through a combination of technical and scientific advances, a supportive scientific environment, and a keen intellect able to put the pieces of a puzzle together. Meniere's novel concept, however, that vertiginous disorders were caused by a pathologic process in the semicircular canals was not accepted overnight, but realized only after another 70 years of scientific debate. This article traces the story of endolymphatic hydrops from its anatomical beginnings to its current place in otolaryngology and the individuals who brought us to this point today.

History, 16th Century↗

Epidemiological survey of definite cases of Meniere's disease collected by the seventeen members of the Meniere's Disease Research Committee of Japan in 1975--1976.

Between Aril 1975 and December 1976, the second nationwide survey of Meniere's disease in Japan was made by the 17 members of the Meniere's Disease Research Committee of Japan. The epidemiological data from 520 patients with definite Meniere's disease were analyzed in comparison with those from the 126 patients in the non-Meniere vertiginous group (Control A) and the 228 patients in the rhinolaryngological group (Control B). The male:female ratio of definite cases of Meniere's disease was almost the same, and the age distribution peaked at the age group of 40--49 years for males, while the peak for females was at the age group of 30--39 years. 5.8% of the 520 patients had a close relative who also suffered from Meniere's disease. From the epidemiological features, it may appear that the occurrence of vertiginous attacks in Meniere's disease is influenced much more by individual than by environmental factors. However, this feature can be considered as another evidence of the psychosomatic disorders involved in Meniere's disease.

Age Factors↗

Vestibular evoked myogenic potentials versus vestibular test battery in patients with Meniere's disease.

OBJECTIVE: The present study was undertaken to assess the sensitivity of vestibular evoked myogenic potentials testing to side-of-disease in Meniere's disease patients and to test the hypothesis that information supplied by vestibular evoked myogenic potentials is complementary to that provided by a conventional vestibular test battery. STUDY DESIGN: Prospective cohort study. SETTING: Large specialty hospital, department of otolaryngology. SUBJECTS: Twenty consenting adults (9 men and 11 women) with unilateral Meniere's disease by American Academy of Otolaryngology-Head and Neck Surgery diagnostic criteria. INTERVENTIONS: All subjects underwent bilateral vestibular evoked myogenic potentials testing using ipsilateral broadband click and short-toneburst stimuli at 250, 500, and 1,000 Hz. All subjects also underwent electronystagmography and sinusoidal vertical axis rotation testing. MAIN OUTCOME MEASURES: Accuracy of side-of-disease assignment by vestibular evoked myogenic potentials, caloric asymmetry, and multivariate analysis. RESULTS: Side-of-disease assignment was most accurate using caloric asymmetry with a 5% interaural difference criterion, achieving 85% correct assignment. The next best method was vestibular evoked myogenic potentials using 250-Hz toneburst stimuli, achieving 80% correct assignment. The least accurate method was caloric asymmetry using a traditional 30% interaural difference limen, achieving 55% correct assignment. Comparison of 5% interaural difference criterion and vestibular evoked myogenic potentials using 250-Hz toneburst stimuli showed discordant results, but in no case did both 5% interaural difference criterion and vestibular evoked myogenic potentials using 250-Hz toneburst stimuli make an incorrect assignment. CONCLUSION: Vestibular evoked myogenic potentials threshold was shown to be highly sensitive to side-of-disease in unilateral Meniere's disease. We observed instances of discordance in side-of-disease assignment by caloric asymmetry and vestibular evoked myogenic potential methods but no case in which both methods were incorrect. This supports the hypothesis that vestibular evoked myogenic potentials supplies information complementary to that provided by other components of the vestibular test battery.

Adult↗

Meniere's disease and endolymphatic hydrops without Meniere's symptoms: temporal bone histopathology.

We studied temporal bone histopathology in 21 ears with Meniere's disease and 24 ears with endolymphatic hydrops without Meniere's symptoms and compared the findings to those in 10 ears with presbycusis and 11 ears with normal hearing. Normal hearing ears showed less degeneration of cochlear structures than the other ears. In ears with endolymphatic hydrops without Meniere's symptoms, the degeneration of spiral ligament, hair cells, dendrites (peripheral processes) and apical spiral ganglion cells was more severe than in the other three groups. In ears with Meniere's disease and endolymphatic hydrops without Meniere's symptoms, the hair cells and dendrites were more affected than ganglion cells and there was no correlation between hair cell and ganglion cell degeneration. These findings suggest that a permanent threshold shift in late stage endolymphatic hydrops is not related to ganglion cell loss but rather to degeneration of sensory elements.

Aged↗

Sudden hearing loss: frequency of abnormal findings on contrast-enhanced MR studies.

BACKGROUND AND PURPOSE: Our purpose was to determine the frequency of abnormal findings on contrast-enhanced high-resolution MR imaging studies in patients with sudden hearing loss. METHODS: Seventy-eight consecutive patients with sudden hearing loss underwent contrast-enhanced MR imaging of the temporal bone, cerebellopontine angle, and brain. Additional tests included audiologic examination, electrocochleography, fistula tests, and serologic tests for viral agents and autoimmune disorders. RESULTS: Probable causes of the sudden hearing loss in these patients included viral or immune-mediated disease, Meniere disease, vascular disorder, syphilis, neoplasm, multiple sclerosis, and perilymphatic fistula. Twenty-four (31%) of the 78 patients were found to have abnormal imaging results early in the course of their work up and treatment. CONCLUSION: The prevalence of abnormal findings on contrast-enhanced MR studies is higher than previously reported in patients with sudden hearing loss.

Adult↗

Meniere's disease in children.

Meniere's disease is an uncommon cause of dizziness or hearing loss in children. Clinicians must maintain a high level of suspicion to make this diagnosis in the pediatric age group. Careful attention to history and diagnostic testing can help in differentiating Meniere's disease from other more common childhood conditions. Treatment is similar to adult patients and may be tried empirically in difficult or suspicious cases.

Adolescent↗

The usefulness of computerized dynamic posturography for the study of equilibrium in patients with Meniere's disease: correlation with clinical and audiologic data.

The use of computerized dynamic posturogaphy (CDP) in the evaluation of patients with balance alterations not only allows quantification of the subject's capacity to maintain a stable centre of gravity, but also analysis of the degree to which the subject is able to use different types of sensory information. The present study investigated the possible use of CDP for clinical staging of vestibular diseases, specifically Meniere's disease (MD). We applied CDP sensory organization tests to 75 patients with definitive MD (AAO-HNS 1995 criteria). A total of 98 CDP sessions were included in the analysis, which focused on four CDP parameters specifically related to vestibular function (condition-5 score, condition-6 score, overall balance score, and VEST, a measure of the relative importance of vestibular information for maintenance of balance). We found a statistically significant relationship between audiometric hearing threshold and CDP scores, especially in patients with audiometrically advanced disease. In addition, CDP scores showed statistically significant variation with time elapsed since the last typical vertigo attack, suggesting that patients can be usefully grouped into three MD activity-level categories: recent post-attack (less than 1 week since last vertigo attack), late post-attack (1 week - 60 days since last attack), and inactive MD (more than 60 days since last attack). On the basis of these results, we propose expected ranges for each of the four CDP parameters in each of the three MD activity-level categories, allowing staging in terms of balance and posture. This staging system complements existing staging systems (based on audiometric criteria, and on subjective assessment of the severity of vertigo attacks and their implications for quality of life).

Adult↗

Inner ear perfusion: indications and applications.

PURPOSE OF REVIEW: To examine the indications and applications of inner ear perfusion in the treatment of common otologic diseases, including Meniere disease, sudden sensorineural hearing loss, and autoimmune inner ear disease. RECENT FINDINGS: The number of surgical procedures, including vestibular neurectomy, labyrinthectomy, and endolymphatic sac surgery, decreased during the 1990s. Intratympanic gentamicin therapy has become the most frequently performed invasive procedure for treatment of Meniere disease. Reports on successful control of vertigo in patients with Meniere disease have ranged from 71.4 to 100%. There is a correlation between the degree of vestibular ablation in transtympanic gentamicin, the control of vertigo, and the risk of hearing loss. Patients with less than 75% ice-reduced vestibular response had a statistically lower risk of hearing loss, but they had a higher rate of persistent vertigo than patients with 100% reduced vestibular response. One hundred percent reduced vestibular response may not be indicative of complete vestibular ablation and may not be necessary for control of vertigo symptoms. In preliminary studies, inner ear perfusion of methylprednisolone in patients with severe, profound sudden sensorineural hearing loss who fail to respond to standard treatment is beneficial, with improvement in hearing thresholds of 16 to 25 dB and dramatic improvement in speech discrimination scores. No immunosuppressive medication has been found effective in suppressing inner ear inflammatory infiltrates or reducing hearing loss in an animal model. SUMMARY: Transtympanic chemical perfusion of the inner ear is safe, inexpensive, and easily performed by an otologic surgeon. With inner ear perfusion, high inner ear concentrations of medication can be achieved, and systemic side effects are minimized.

Autoimmune Diseases↗

The summating potential and Meniere's disease. I. Summating potential amplitude in Meniere and non-Meniere ears.

We investigated the relationship between Meniere's disease and the abnormally enlarged summating potential (SP) in a series of patients and normal subjects. Cochlear and auditory nerve responses to rectangular-pulse clicks, delivered at a rate of 8/s and an intensity of 115 dB peak-equivalent sound pressure level, were recorded from ear canal skin. We performed a parabolic regression for SP amplitudes as a function of auditory nerve action potential (AP) amplitudes in normal ears and obtained the 95% confidence interval (+/- 2 SD) for normal scatter around the fitted curve. Sixty-eight percent of SP amplitudes from ears with Meniere's disease exceeded the upper normal confidence limit. In contrast, only 7% of the cochlear ears and none of the retrocochlear ears had SP amplitudes above this normal upper limit. Cochlear microphonic potentials and APs from the ears with Meniere's disease also tended to be larger than normal, but this tendency was not strong enough to be of diagnostic use.

Action Potentials↗

Submillimeter magnetic resonance imaging of the temporal bone in Meniere's disease.

The pathoetiology of Meniere's disease remains elusive. Histopathologic and imaging studies have implicated congenital or developmental abnormality of the endolymphatic duct as a likely predisposing factor to the development of endolymphatic hydrops and clinical Meniere's disease. Recently, improved high-resolution magnetic resonance imaging (MRI) protocols have allowed better demonstration of the soft tissues of the endolymphatic duct and sac. This study is a prospective evaluation of the ability of submillimeter MRI to detect the endolymphatic duct in Meniere's disease patients and control subjects. In addition, the development of the temporal bone in the region of the endolymphatic sac and duct is evaluated by measurements of the distance from the posterior semicircular canal to the subarachnoid space and the distance from the vestibule to the subarachnoid space. Visualization of the endolymphatic duct was found to be significantly less frequent in the Meniere's disease patients (29%) than in the control subjects (91%). Temporal bone measurements in the region of the endolymphatic duct showed patients with Meniere's disease to have smaller dimensions than control subjects.

Adult↗