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Meniere's disease has been the focus of much controversy concerning its etiology and pathophysiology, not to mention treatment. A brief review is presented of the major proposed causative factors and therapy, both medical and surgical, founded upon these theories. We also present the diagnostic evaluation and therapeutic modalities employed by The Otology Group, P.C., as well as long-term, follow-up data on our patients.
Missense mutations in the coagulation factor C homology (COCH) gene (14q12-q13) cause the autosomal dominant sensorineural hearing loss and vestibular disorder DFNA9 (OMIM 603196), and a high prevalence of symptoms of Meniere disease (MD) has been described in families with a mutation in the COCH gene. In this study, we search for mutations in the COCH gene in peripheral blood from patients with definite MD. DNA was extracted from peripheral blood cells of 30 individuals with MD and 30 controls. Exons 4 and 5 of the COCH gene were amplified by PCR reaction, using primer pairs flanking both exons. Sequences were analysed by a DNA sequencing system and compared with the published COCH cDNA sequence. No differences were found in the nucleotide sequences of exons 4 and 5 in the COCH gene in patients with definite sporadic MD when they were compared with the control group. Patients with definite MD have a low prevalence of mutations in exons 4 and 5 of the COCH gene.
Meniere's disease can only be studied in patients, since it does not occur spontaneously in animals nor can it be induced in them. However, aspects of the disease such as endolymphatic hydrops can be usefully studied in animals. A study of the natural history (epidemiology) of Meniere's disease demonstrated the three major symptoms (triad) to be vestibular symptoms, auditory symptoms, and aural pressure. Bilaterality occurs in at least one out of 3 patients, and may approach 50% over full lifespans. Aural pressure (74.1%) was common, as was positional vertigo (85.9%) during and/or between attacks. Clinical variants such as vestibular Meniere's disease could persist for 25 years or more. Understanding the pathogenesis of Meniere's requires a study of known and unknown causes. In this study, Meniere's disease (cause unknown) was differentiated from Meniere's syndrome (cause known). Meniere's disease or syndrome can occur years after some inciting cause; thus all forms of Meniere's can be considered to have a delayed onset. Meniere's syndrome can occur as a sequel to syphilis, otosclerosis, infection (for example otitis media), or trauma. Endolymphatic hydrops explained on the basis of quantity and quality of endolymph is found in all forms of Meniere's disease and syndrome. Representative cases and pathological examples are discussed. Hydrops of the pars inferior (cochlear duct and saccule) is the most important finding in Meniere's disease. In some (but not most) cases, ruptures of the membranes are seen. The saccule can distend into the lateral semicircular canal. Symptomatic attacks are explained on the basis of both physical and biochemical phenomena. This study discusses concepts of pathogenesis of the disease, finding both theories of longitudinal (slow) and radial (fast) flow to be operational. Longitudinal flow, however, appears to be more important than radial flow, especially in advanced Meniere's where perilymph in the scala vestibuli and vestibule disappears and is replaced by membranous labyrinth. Meniere's disease (idiopathic) and Meniere's syndrome (cause known) probably occur as a result of endolymphatic absorptive dysfunction (the site being endolymphatic duct and sac). Hypocellularity of the mastoid and periaqueductal air cells, hypodevelopment of Trautmann's triangle, and anterior displacement of the lateral sinus are likely to be important findings associated with developmentally dysfunctional absorption of endolymph.(ABSTRACT TRUNCATED AT 400 WORDS)
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The efficacy of treatment of Menière's disease was investigated in 2 double blind study versus placebo. Forty patients were given 36 mg betahistine dimesilate daily for at least six weeks. The course of disease and findings one year before and one year after the treatment were noted and compared for assessing the therapeutic success. The findings on threshold audiograms, spontaneous and provoked nystagmus and caloric tests did not show any significant changes. A slight improvement was noted in the low frequency range of threshold audiograms. Dizziness, tinnitus and disturbances of walking were significantly improved. The incidence of vertigo attacks was reduced to 64%. In 89% of the patients no further vertigo attacks were seen after the treatment. The best results of betahistine dimesilate treatment were observed in the early stages of Menière's disease.
Within one year (1993) we found a positive family history in five out of forty-eight new patients with Menière's disease, corresponding to a frequency of 10.4%. We found between two and seven cases in each of five families. The disease followed a dominant autosomal hereditary pattern over two to four generations. Audiometric and vestibulometric examination confirmed the diagnosis. Clinical emphasis was placed on trigger factors such as infections, physical and psychological stress, the autonomic system, and metabolic and endocrinological disorders. There was no evidence of a significantly high incidence of any single trigger factor. The most important result of this study, which also included human leucocyte antigen (HLA) typing, is the fact that HLA A2 was represented in 90% of patients with positive family history of Menière's disease and in 75% of patients with solitary Menière's disease in contrast to only 28.9% in the average European population. The frequency of HLA B44 was 70% in family-linked Menière's disease and 37.5% in single Menière's disease. The frequency in the average European population is 12.3%. What is even more striking is the combination HLA A2 B44, occurring with respective frequencies of 60%, 37%, and 5%. These results suggest a multi-factor etiology of Menière's disease combined with a genetic predisposition, which might be caused by mutations on the short arm of chromosome 6.
The diagnosis of Menière's disease is uncertain when the typical symptoms do not occur completely and definitely. A reliable finding of an endolymphatic hydrops (EH) is the base for a correct prognosis and therapy. Electrocochleography is a proven diagnostic procedure but requires a lot of time and of technical know-how. The mobility of cochlear partition can be tested by low-frequency masking (LFM) recording the phase dependent subjective masked threshold of a short test tone. We performed electrocochleography and LFM in 29 patients with suspected Menière's disease at the same day. Both tests pointed at an EH in 62%, and in 24% the results showed correspondent negative results. In 14% the results were inconsistent. Both methods also showed in 59% of the contralateral ears without symptomatic signs the indication of an EH. Considering the good conformity of both tests the easier LFM can be recommended for detection of EH.
The authors treated 22 patients with intratympanic gentamicin. Vestibular function was measured using caloric and head impulse tests and vestibular evoked myogenic potentials induced by high amplitude sounds and short duration galvanic currents. Roughly one-third of the patients, after initially losing their caloric responses and displaying refixation saccades to head impulse tests, recovered within 2 years of the lesion. Vertigo did not recur in patients in whom the galvanic response was abolished.
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At its recent spring meeting, the American Neurotologic Society devoted a portion of its program to a symposium on staging Meniere's disease and reporting results. One of the problems discussed, was which preoperative audiogram should be used for reporting results of any treatment. I think that it is very important that we, as otologists, try to look at Meniere's disease or any inner ear dysfunction in a more scientific way. Some of you may feel, on initial evaluation of this material, that it is too cumbersome to be practical in your daily clinical work. However, we feel we have put forth a reasonable staging system and method of reporting results that can be quite helpful once the simple routine of using them has become established. The vertigogram, for example, which is designed to be very simple and not require any sophisticated equipment, should be as easy to read as an audiogram. If we try to approach the vagaries of Meniere's disease or any inner ear dysfunction in a more scientific fashion, I am sure that in several years we will be in a much better position collectively to evaluate our results and to help our patients. This type of comprehensive staging system for all of the components in Meniere's disease or any inner ear dysfunction will enable us to objectify diagnoses and treatment results so that they can be easily compared. We have put forth some of the ideas and methods that we think will help us arrive at a consensus for reporting results. We encourage you to try some of the suggestions presented in this article and welcome your comments and ideas for improving this staging system and method for reporting results.
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When Meniere's disease becomes refractory to medical management, a variety of highly effective surgical options are available. A selective nerve section can relieve the disabling vertigo of Meniere's disease while preserving hearing. When the hearing on the involved side is nonserviceable, a labyrinthectomy is an important alternative. An eighth nerve section makes a complete preganglionic ablation possible with the additional benefit of relieving tinnitus in over two thirds of selected patients.