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              Meniere's Disease


 

                
Meniere's disease is the name applied to recurrent vertigo accompanied by tinnitus and deafness. The latter symptoms may be absent during the initial attack(s) of vertigo, but they invariably appear as the disease progresses and are increased in severity during an acute attack. 

With milder forms of the syndrome the patient may complain more  of head discomfort, slight instability, and difficulty in concentration than of vertigo and may be considered to be anxious or depressed.

It may be associated with hyper- or hypotension, intracranial lesions, lesions, syphilis, or disseminated sclerosis

The Meniere's disease has its onset most frequently in the fifth decade of life, though younger adults and elderly are not spared.

The pathologic changes are said to consist of a dilatation of the endolymphatic system which leads to a degeneration of the delicate vestibular and cochlear hair cells. The relation of these pathologic changes to the paroxysmal disorder of labyrinthine function is unknown.

                  

Conscientiousness in patients with Ménière's disease. Otol Neurotol. 2007 Apr;28(3):365-8.

OBJECTIVE: To assess whether a conscientious personality profile is more prevalent in patients with Ménière's disease (MD) in comparison with other vertigo patients. Second, to investigate a sex effect and compare the obtained conscientiousness scores with published normative values. PATIENTS: A total of 108 patients were included and subdivided into one of five pathology groups: MD, benign paroxysmal positional vertigo, vestibular neuritis, vestibular schwannoma, and posttraumatic nonbenign paroxysmal positional vertigo. INTERVENTION: The conscientiousness domain of the self-reporting NEO Five-Factor Inventory questionnaire. MAIN OUTCOME MEASURE: Conscientiousness score. RESULTS: There was no significant difference between the diagnostic groups or a sex effect. MD patients had a higher score than published normal values. CONCLUSION: As assessed with the NEO Five-Factor Inventory questionnaire, MD patients do not demonstrate a higher conscientious personality profile in comparison with other vertigo patients. Therefore, this limits the clinical use of the personality profile to discriminate between patient groups.

Otosclerosis associated with Ménière's disease: a histological study. Adv Otorhinolaryngol. 2007;65:50-2.

A histological study of a pair of temporal bones was performed in a case of Menière's disease. A severe endolymphatic hydrops and extensive capsular otosclerosis bilaterally was found. Severe endolympathic hydrops results from otosclerotic endolympahtic duct occlusion. Our unique histopathological findings show that a causal association exists between these two entities.

Non-specific immunological determinations in Meniere's disease: any role in clinical practice? Eur Arch Otorhinolaryngol 2007 Jan;264(1):15-9. Epub 2006 Oct 11.

Several studies supported the hypothesis that an autoimmune response may be a pathogenetic factor in Meniere's disease occurrence. It has been demonstrated that the endo-lymphatic sac has an important role in the immuno-mediated reaction and it has also become evident that an immunological mechanism may be involved in the development of endo-lymphatic hydrops. The present study was carried out in order to analyse the validity of serological non-specific immune tests in determining the possible role of immune pathology in 200 Meniere's suffering patients. The immune activity was investigated determining the erythrocyte sedimentation rate (ESR), the C-reactive protein levels, the rheumatoid factor, the serum immunoglobulin levels by group, the complement levels, the lymphocyte sub-populations, the cryoglobulines, the circulating immune complexes (CIC), organ and non-organ auto-antibodies. Levels of Ig, ESR, C-reactive protein, rheumatoid factor, complement fractions and cryoglobulines were either negative or non-significant in the considered cohort of Meniere's disease suffering patients. On the other hand, in more than half of the sample group high values of CIC were found. Data concerning T-lymphocyte sub-population indicates a clear increase in T-helper compared to T-cytotoxic cells, with an increase in the CD4/CD8 ratio. The datum of particular interest concerns analysis of T-cells activation complexes. Our present findings show that early activated T-lymphocytes change and in particular changes occur in the expression of the interleukin-2 receptor. Moreover, it seems to be possible to correlate the alteration of the immunological tests and the phase of the disease. Present data showed that the immunological factor should be considered a relevant element in most of the cases of Menière's disease.

Ménière's disease review 2005. J Am Acad Audiol. 2006 Jan;17(1): 16-26.

Ménière's disease (MD) is a complex, multifactorial disorder of the inner ear that is the most common cause of the syndrome of episodic vertigo combined with fluctuating hearing loss. In spite of a century of investigation, the etiology and pathophysiology of MD remain controversial and incompletely understood. Among the factors that have contributed to these controversies are the absence of (1) a validated clinical test, (2) an appropriate animal model, and (3) a specific treatment. Nonetheless, physicians are able to assist MD patients with a variety of tailored, symptom-specific medications and therapies. Given that the vertigo induced by MD, in general, is self-limited, the long-term outlook for balance function is good. The same cannot be said for the hearing dysfunction of MD.

Diameter of the cochlear nerve in endolymphatic hydrops: implications for the etiology of hearing loss in Ménière's disease. Laryngoscope.2005 Sep;115(9):1525-35.

OBJECTIVE/HYPOTHESIS: Endolymphatic hydrops (ELH) is an important histopathological hallmark of Ménière's disease. Experimental data from human temporal bones as well as animal models of the disorder have generally failed to determine the mechanism by which ELH or related pathology causes hearing loss. Hair cell and spiral ganglion cell counts in both human and animal case studies have not, for the most part, shown severe enough deterioration to explain associated severe sensorineural hearing loss. However a limited number of detailed ultrastructural studies have demonstrated significant reductions in dendritic innervation densities, raising the possibility that neurotoxicity plays an important role in the pathology of Ménière's disease (MD) as well as experimental endolymphatic hydrops (ELH). This study tests the hypothesis that neurotoxicity is an important primary mediator of injury to the hydropic ear and is reflected in measurable deterioration of the cochlear nerve in the animal model of ELH. This study also explores the previously presented hypothesis that cochlear injury in ELH is mediated through the actions of nitric oxide (NO) by evaluating whether hearing loss or various measures of cochlear damage can be ameliorated by administration of an agent that limits excess production of NO. STUDY DESIGN: Part one of the project involves the surgical induction of endolymphatic hydrops and correlation of long term hearing loss with histological parameters of ELH severity as well as cochlear nerve and eighth cranial nerve diameter measurements. In part two, aminoguanidine is administered orally to a separate set of hydropic animals in an attempt to limit cochlear injury presumably mediated by NO. METHODS: Guinea pigs are subjected to surgical induction of unilateral endolymphatic hydrops after establishing baseline ABR thresholds at 2, 4, 8, 16, and 32 kHz. Threshold shifts are established prior to sacrifice at 4 to 6 months and temporal bones processed for light microscopy. Measurements of cochlear nerve and eighth cranial nerve maximal diameters as well as average maximal diameters are carried out and correlated to hearing loss and a semi-quantitative measure of hydrops severity. The identical experiments are carried out in animals treated with aminoguanidine, an inhibitor of inducible nitric oxide synthase. RESULTS:: The mean maximal diameter (n = 14) of the hydropic cochlear nerve was significantly reduced (432.14 +/- 43.18 vs. 479.28 +/- 49.22 microns, P = .0025) as compared to the control nerve. This was also seen in measures of the eighth cranial nerve (855.71 +/- 108.82 vs. 929 +/- 81.53 microns, P = 0.0003). Correlation studies failed to show correlation between hydrops severity and a cochlear nerve deterioration index (r = -0.0614, P = .8348). Similarly, hearing loss severity failed to correlate with cochlear nerve deterioration (r = 0.1300, P = .6577). There was a significant correlation between hearing loss and hydrops severity (r = 0.6148, P = .0193). Aminoguanidine treated animals (n = 5) also sustained nerve deterioration to the same degree as non-treated animals and there appeared to be no protective effect (at the dosage administered) against ELH related hearing loss, hydrops formation, or nerve deterioration. CONCLUSION: ELH results in significant deterioration of cochlear nerve and eighth cranial nerve maximal diameters in the guinea pig model. These findings are in accord with previous studies which detected ultrastructural evidence of dendritic damage and indicate that neural injury is of sufficient severity to result in light microscopic evidence of cochlear nerve and eighth cranial nerve deterioration. These data support the concept that the principle pathological insult in ELH is a form of neurotoxicity, especially in light of previous studies which indicate relative preservation of hair cells at similar points in time. The lack of correlation between the severity of hydrops and nerve deterioration suggests that nerve deterioration is independent of hydrops severity.


November 2007

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Histopathology of the vestibular end organs after intratympanic gentamicin failure for Meniere's disease. Acta Otolaryngol. 2007 Jan;127(1):34-40.

CONCLUSION: To our knowledge, this is the first report of the histopathology of the vestibular end organs following intratympanic gentamicin for intractable Meniere's disease. There was relative sparing of the utricular macula, compared with the cristae ampullares. However, the utricular macula exhibited severe hair cell loss. Clinically, the patient has been free from vertigo spells for 3 years following labyrinthectomy. Objective: To describe the histopathology and morphometry of the vestibular end organs from a 59-year-old Meniere's patient who underwent transmastoid labyrinthectomy for recurrent vertigo after failed intratympanic gentamicin. MATERIALS AND METHODS: Light and transmission electron microscopy were utilized; with unbiased stereology-physical fractionator for type I, type II hair cell, and supporting cell counts. Comparison with end organ histopathology in a 56-year-old with Meniere's disease without gentamicin treatment was carried out. RESULTS: Histopathological analysis of the semicircular canal cristae ampullares showed severe atrophy of the neuroepithelium with undifferentiated cells, and fibrosis and edema of the stroma. The utricular macula had some remaining type I and type II vestibular hair cells, and nerve fibers and terminals within the underlying stroma. Morphometric measures were obtained from the utricular macula: 2000 type I and 500 type II hair cells, representing 7.3% of type I hair cells and 4.9% of type II hair cells compared with normative controls, and 24 000 supporting cells were obtained.

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