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PubMed2003

Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction, but not of Meniere's disease.

The COCH gene is the only gene identified in man that causes autosomal dominantly inherited hearing loss associated with vestibular dysfunction. The condition is rare and only five mutations have been reported worldwide. All affected families showed a similar progressive hearing loss and vestibular dysfunction. Since Meniere's disease-like symptoms have also been described in some families, it was suggested that COCH mutations might be present in some patients diagnosed with Meniere's disease. In this study, using a Japanese population, we performed a COCH mutation analysis in 23 patients from independent families with autosomal dominant hearing impairment, four of whom reported vestibular symptoms, and also in 20 Meniere's patients. While a new point mutation, A119 T, was found in a patient with autosomal dominant hearing loss and vestibular symptoms, no mutations were found in the Meniere's patients. Like all other previously identified COCH mutations, the mutation identified here is a missense mutation located in the FCH domain of the protein. The current mutation is located in close spatial proximity to W117, in which a mutation (W117R) had previously been associated with autosomal dominant hearing loss. Model building suggests that, like the W117R mutation, the A119 T mutation does not affect the structural integrity of the FCH domain, but may interfere with the interaction with a yet unknown binding partner. We conclude that mutations in the COCH gene are responsible for a significant fraction of patients with autosomal dominantly inherited hearing loss accompanied by vestibular symptoms, but not for dominant hearing loss without vestibular dysfunction, or sporadic Meniere's disease.

Adult↗
PubMed1982

Vascular mechanisms in Meniere's disease. Theoretical considerations.

Unimpeded venous drainage of the vestibular organs via the paravestibular canaliculus (PVC) vein is crucial to inner ear fluid mechanisms. With increased venous pressure, insufficient drainage may result in endolymphatic hydrops, unless collateral veins develop. Different mechanisms cause venous insufficiency in different cases of Meniere's. One suggested mechanism is alteration of the intermediate endolymphatic sac (IES) microcirculation with decreased regulation of blood held within subepithelial capillaries of the sac. Increased blood may thus drain into the veins, with increased venous pressure and inadequate vestibular drainage. Altered microcirculation may be morphological as in fibrosis or shortening of the IES, or physiological where the microcirculation receives abnormal stimuli or reacts abnormally to normal stimuli. Venous insufficiency may result from PVC vein anomalies. Variations in venous pattern whereby vestibular organs preponderantly drain through the PVC vein may predispose to Meniere's disease.

Collateral Circulation↗
PubMed1999

Autonomic nervous function in patients with Meniere's disease evaluated by power spectral analysis of heart rate variability.

OBJECTIVE: A hypothesis has been advanced that the autonomic nervous dysfunction (AND) relates to the development of vertigo in Meniere's disease (MD). We also studied the causal relationship between AND and vertigo in MD. METHODS: We evaluated autonomic nervous function in 17 patients with MD (five men and 12 women ranging in age from 16 to 70 years) by classifying them by their stages of attack and interval of vertigo and with power spectral analysis (PSA) of heart rate variability. Fourteen healthy volunteers were also tested as controls. RESULTS: At the interval stage, parasympathetic nervous hypofunction and significant depression of sympathetic response due to postural changes from the supine to the standing position were observed in many of those patients. At the attack stage, sympathetic nervous hypofunction was observed in some of the patients. CONCLUSION: These findings lead us to the conclusion that AND relates to vertigo in MD as a predisposing factor. However, the question of whether AND relates as a trigger or as a consequence of vertigo in MD has not been adequately solved in this study. We will make further studies on circadian variation of autonomic nervous function.

Adolescent↗
PubMed2004

Comparison of acute low-tone sensorineural hearing loss versus Meniere's disease by electrocochleography.

To clarify the pathogenesis of acute low-tone sensorineural hearing loss (ALHL), we retrospectively compared the electrocochleographic findings from 20 patients with ALHL with those from 58 patients with Meniere's disease (MD) classified into 4 groups (MD1 through MD4) according to their pure tone average. The mean summating potential-action potential ratio in the ALHL group was 0.35 +/- 0.13, which was significantly higher than the control ratio but similar to the ratio seen in the MD1 group (pure tone average < 25 dB hearing level). The mean detection threshold of the cochlear microphonics in the ALHL group was 32.0 +/- 9.4 dB normal hearing level, which was again similar to that seen in the MDI group. Moreover, more than 50% of patients with ALHL had normal cochlear microphonics input-output curves. We therefore conclude that the pathogenesis of ALHL arises from an endolymphatic hydrops with little or no impairment of hair cells that resembles early-stage MD.

Action Potentials↗
PubMed2004

Transtympanic perfusion: indications and limitations.

PURPOSE OF REVIEW: To review the most recent literature regarding the application of transtympanic inner ear perfusion in the treatment of inner ear disorders including Meniere disease, sudden sensorineural hearing loss, and autoimmune inner ear disease. RECENT FINDINGS: The use of gentamicin perfusion in the management of Meniere disease with intractable vertigo has been demonstrated to have a very high rate of success, and is much less invasive than alternative surgical procedures such as vestibular nerve section or labyrinthectomy. The technique for achieving the highest rate of success while still minimizing the risk of cochleotoxicity continues to be investigated. Sustained delivery techniques such as the Silverstein MicroWick appear to achieve the best pharmacokinetic profile within the inner ear fluids. The end point of treatment does not necessarily require complete vestibular ablation to cure the patient, and shorter courses of treatment may help to reduce the risk of hearing loss. Cochlear Meniere disease can be treated with dexamethasone 4 mg/cc perfusion of the inner ear, which may improve the hearing, tinnitus, and pressure in the ear. Sudden sensorineural hearing loss has been managed with transtympanic steroid delivery, and this appears to be beneficial for some patients who have failed to respond to oral steroids, or have medical contraindications to systemic steroids. SUMMARY: Inner ear perfusion via transtympanic delivery is an emerging technique in the management of inner ear disease. Improved results are expected over time as research in this area answers questions about dosage and delivery techniques, as well as identifying new applications and pharmaceuticals.

Adult↗