Elsevier

The Lancet

Volume 372, Issue 9636, 2–8 August 2008, Pages 406-414
The Lancet

Seminar
Meniere's disease

https://doi.org/10.1016/S0140-6736(08)61161-7Get rights and content

Summary

Meniere's disease is a chronic illness that affects a substantial number of patients every year worldwide. The disease is characterised by intermittent episodes of vertigo lasting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus, and aural pressure. Although there is currently no cure, more than 85% of patients with Meniere's disease are helped by either changes in lifestyle and medical treatment, or minimally invasive surgical procedures such as intratympanic steroid therapy, intratympanic gentamicin therapy, and endolymphatic sac surgery. Vestibular neurectomy has a very high rate of vertigo control and is available for patients with good hearing who have failed all other treatments. Labyrinthectomy is undertaken as a last resort and is best reserved for patients with unilateral disease and deafness.

Introduction

Prosper Ménière first described this disease in 1861.1 His main contribution to its diagnosis was a description of the disease entity with episodic vertigo and ringing in the ears; he challenged general terminology at the time that named this disease apoplectic cerebral congestion, implying a disorder of the brain. Ménière described these episodes of vertigo and fluctuating hearing loss as being associated with the peripheral end organ of the inner ear rather than with the brain. He and other investigators2, 3 called it “glaucoma of the inner ear”.

In 1927, Guild4 identified the endolymphatic sac as the site of “outflow of endolymph” in his study of guineapigs. This study was a major insight into the mechanics of endolymphatic flow in the inner ear. Later that same year, Portmann3 described his first endolymphatic sac surgery for the Meniere's disease complex. Endolymphatic sac surgery has remained the main non-destructive form of surgical treatment for Meniere's disease since the early 1900s. 1 year after Portmann's description of sac surgery, Dandy5 proceeded with vestibular neurectomy, trying to isolate the vestibular system from the brain and thus cure patients of vertigo.

In 1943, Altmann and Fowler6 concluded that problems in production and absorption of endolymph can lead to Meniere's disease. In a landmark study in 1967, Kimura7 investigated the first animal model with guineapigs and showed that blockage of the endolymphatic sac and duct causes obstruction of endolymphatic outflow, leading to hydrops of the inner ear.

Section snippets

Epidemiology

Meniere's disease remains a difficult disease to diagnose, especially in the early stages when not all its symptoms might be present. Consequently, the incidence and prevalence of the disease in any population is difficult to ascertain.8 Frequently, patients with Meniere's disease present to the emergency department with sudden onset of vertigo and are inaccurately diagnosed as having labyrinthitis and discharged home.8 In early stages, Meniere's disease might present with only cochlear

Pathophysiology

Meniere's disease is characterised by intermittent episodes of vertigo lasting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus, and aural pressure.24 It has been classified into typical Meniere's disease, with all the cochlear and vestibular symptoms, and atypical Meniere's disease, with either cochlear symptoms (eg, hearing loss, tinnitus, aural pressure) or vestibular symptoms (eg, vertigo alone with aural pressure but no hearing loss or tinnitus).25 The primary

Diagnosis

The clinical picture of Meniere's disease includes fluctuating sensorineural hearing loss accompanied by aural pressure, tinnitus, and episodic vertigo. The hallmark of the disease is its fluctuation, waxing, and waning of symptoms. Typical Meniere's disease includes all symptoms; the episodic vertigo lasts from several minutes to hours, with positional vertigo in between and during attacks. Furthermore, almost all patients note a sense of pressure or fullness in the ear and various forms of

Medical and surgical management

Several medical and surgical remedies have been offered to patients with Meniere's disease over the past 150 years. The plethora of medical and surgical therapies signifies that no one effective treatment is available for these patients. However, most will be helped by a combination of medical therapy, psychological counselling and reassurance, and lifestyle and dietary changes.

The cure for this disease is yet to be discovered; it could lie in genetic re-engineering that might need a

Conclusion

Meniere's disease continues to afflict hundreds of thousands of patients every year on a global scale. Patients of all ethnic and racial backgrounds have been afflicted with this chronic illness. It is true that we still do not have a cure for this disease, as with many other illnesses in medicine. However, substantial improvements have been made over the centuries in dealing with this illness, especially in the past decade, and several safe and effective medical and surgical therapies are now

Search strategy and selection criteria

We searched Medline, ENT Rez, Pubmed, and the Cochrane Library for publications in the past 10 years, with the terms “Meniere's disease” and “Endolymphatic sac”, “Gentamicin middle ear perfusion”, and “Steroid middle ear perfusion”. We largely used materials published within the past 5 years but did not exclude older publications that were commonly referenced and highly regarded. We also searched the reference lists of articles identified by this search strategy and selected those that we

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