What are the results of Menieres disease?

Description

Ménière disease is a disorder of the inner ear that affects balance and hearing. This condition is characterized by sudden episodes of extreme dizziness (vertigo), a roaring sound in the ears (tinnitus), a feeling of pressure or fullness in the ears, and fluctuations in hearing. Episodes are often associated with nausea and vomiting, and they can severely disrupt activities of daily living.

The episodes associated with Ménière disease generally last several hours. Studies suggest that episodes can be triggered by stress, tiredness (fatigue), emotional upset, illness, and dietary factors. The timing of these episodes is unpredictable; affected individuals may experience a cluster of episodes within a short period, followed by months or years without any symptoms.

Ménière disease usually appears in adulthood, most often in a person's 40s or 50s. It is much less common in children and young adults. The symptoms of the disorder typically begin in one ear, although they may later involve both ears.

Some people with Ménière disease have no symptoms of the disorder between episodes, particularly in the early stages of the disease. Over time, however, many affected individuals develop ongoing problems with unsteadiness, tinnitus, and a feeling of fullness in the ears. Additionally, permanent hearing loss eventually develops in many people with this disorder.

Frequency

The prevalence of Ménière disease varies in different geographic regions and ethnic groups. It appears to be more common in people of European descent than in those with other backgrounds. In the United States, there are an estimated 615,000 people with Ménière disease, and more than 45,000 new cases are diagnosed each year.

Causes

The cause of Ménière disease is unknown, although it probably results from a combination of environmental and genetic factors.

Ménière disease is thought to be related to abnormalities of the inner ear, which contains structures that are needed for normal hearing and balance. Episodes of vertigo, tinnitus, and hearing loss likely result from fluctuating amounts of fluid in the inner ear. These changes disrupt signals sent from the inner ear to the brain that are related to sound and the body's position and movement.

Researchers have studied many possible risk factors for Ménière disease, including viral infections, trauma to the inner ear, noise pollution, allergies, abnormal immune system responses, and migraines. Studies have also examined changes in more than a dozen genes that might contribute to the signs and symptoms of this condition. However, none of the factors studied so far appears to play a major role in Ménière disease. Researchers are looking for additional environmental and genetic factors that are associated with this complex disorder.

Inheritance

Most cases of Ménière disease are sporadic, which means they occur in people with no history of the disorder in their family. A small percentage of all cases have been reported to run in families.

When the disorder is familial, it most often has an autosomal dominant pattern of inheritance. Autosomal dominant inheritance means one copy of an altered gene in each cell is sufficient to increase the risk of the disorder. However, no associated genes have been identified.

Other Names for This Condition

  • Auditory vertigo
  • Aural vertigo
  • Meniere disease
  • Meniere's disease
  • Meniere's syndrome
  • Ménière's disease
  • Ménière's vertigo
  • Otogenic vertigo
  • Primary endolymphatic hydrops

References

  • American Hearing Research Foundation
  • Hamid MA. Ménière's disease. Pract Neurol. 2009 Jun;9(3):157-62. doi: 10.1136/jnnp.2009.176602. Citation on PubMed
  • Morrison AW, Bailey ME, Morrison GA. Familial Ménière's disease: clinical and genetic aspects. J Laryngol Otol. 2009 Jan;123(1):29-37. doi: 10.1017/S0022215108002788. Epub 2008 Jul 11. Citation on PubMed
  • Morrison AW, Johnson KJ. Genetics (molecular biology) and Meniere's disease. Otolaryngol Clin North Am. 2002 Jun;35(3):497-516. Review. Citation on PubMed
  • Paparella MM, Djalilian HR. Etiology, pathophysiology of symptoms, and pathogenesis of Meniere's disease. Otolaryngol Clin North Am. 2002 Jun;35(3):529-45, vi. Review. Citation on PubMed
  • Sajjadi H, Paparella MM. Meniere's disease. Lancet. 2008 Aug 2;372(9636):406-14. doi: 10.1016/S0140-6736(08)61161-7. Review. Citation on PubMed

Ménière’s disease/syndrome is a long term, progressive vestibular condition affecting the balance and hearing parts of the inner ear. Symptoms are acute attacks of vertigo (severe dizziness), fluctuating tinnitus, increasing deafness, and a feeling of pressure in the ear.

Who is affected by Ménière’s ?

The incidence of Ménière's is between 1:1000 and 1:2000 of the population; depending on the source. Méniére's can affect anyone and it can occur at any age. About 7-10% of those affected have a family history of the condition.

What causes Ménière’s ?

The cause of Ménière’s is unknown. Many factors are thought to be involved in the development of the condition, e.g. increased pressure of the fluid in the endolymphatic sac; allergic factors damaging the inner ear or other unknown factors. The relationship between these factors and the progression of the condition, however, remains unclear.

How does Ménière’s affect you?

Symptoms vary between people and over time. The main problems are unpredictable attacks of vertigo with nausea and vomiting. Attacks can last from a few minutes to 24 hours. There may also be tinnitus, hearing loss and a feeling of fullness in the affected ear. Periods of remission between attacks can vary from days to months or even years; making Ménière’s an unpredictable and distressing condition. As it progresses the vertigo may be less severe; however there may be periods of imbalance, adding to the distress. In the later stages tinnitus is more prominent and fluctuating hearing loss develops. There is permanent damage to the balance organ and significant balance problems are common. Usually only one ear is affected, but up to 50% of sufferers may develop the condition in both ears. It is useful to divide the course of the illness into three stages:

Stage one (early): unpredictable attacks of vertigo

The main feature is intermittent attacks of vertigo which can last from a few minutes to hours. During the attack there is a variable amount of hearing loss along with a sensation of fullness in the affected ear.

Some people may experience tinnitus or an increase in tinnitus in the affected ear. The fullness in the ear and tinnitus may precede the attacks of vertigo, but they will often occur without warning. In between the attacks the hearing and sensation in the ear return to normal. There are periods of remission between the attack, which vary in each person making Ménière’s disease an unpredictable and distressing illness.

Stage two (intermediate): attacks of vertigo; tinnitus; hearing loss

The attacks of vertigo continue with variable remissions however may be less severe. After or perhaps before the attack the person may experience a period of imbalance and movement induced giddiness. Permanent hearing loss develops and continues to fluctuate with the vertigo attacks. Tinnitus becomes more prominent often fluctuating or increasing with the attacks.

Stage three (late): hearing loss; balance difficulties; tinnitus

In the later stages the hearing loss increases and often the attacks of vertigo diminish or stop. Hearing loss can be severe and distortion, loudness discomfort and recruitment can be a problem. There is permanent damage to the balance organ in the ear and significant general balance problems are common, especially in the dark.

Why does tinnitus happen in Ménière’s ?

Ménière’s disease causes damage to the hair cell receptors in the inner ear. These damaged hair cells spontaneously fire and send disorganised signals up the auditory nerve to the brain. In Ménière’s the hair cells concerned with low frequency sound are the first damaged, and therefore the tinnitus in Ménière’s is usually a rumbling, low frequency noise. In a survey in the USA of 51 people with Ménière’s disease, 49% reported the tinnitus as moderately annoying (tinnitus while listening to a speaker) or severely annoying (difficulty with work, relationships and sleeping). For those with Ménière’s disease in its first stages noticing the tinnitus starting to change may be a warning sign that they will have a bad day or an attack.

Why do people with Ménière's get hearing loss?

It is widely believed that Ménière’s develops as a result of an increase in the pressure in the endolymphatic space. The symptoms of ear fullness and reduced hearing are likely to be related to this increase in pressure. The sudden release in pressure accounts for the sudden attacks of vertigo. Repeated episodes of high pressure and sudden releases of that pressure damage the delicate structures of the inner ear and the balance structures of the semi-circular canals. This cumulative damage results in a decline in hearing levels over time.

Kumagami et al (1982) describes three stages of Ménière’s disease:

  • Stage 1 - hearing levels return to normal levels between attacks
  • Stage 2 - hearing levels fluctuate but do not return to normal
  • Stage 3 - hearing levels remain down below 60 dB HL

The increase in cochlear endolymphatic pressure affects normal hearing function. The principle consequence of hearing loss is a reduced sensitivity to quiet sound. In early Ménière’s disease (stages 1 & 2) the hearing loss usually involves the low frequencies. Sounds may seem distorted as the pressure increase affects the fine tuning functions of the basilar membrane and outer hair cells. These effects are initially reversible between attacks but over time the inner and outer hair cells sustain permanent damage resulting in a non-reversible hearing loss. This can often lead to a reduced tolerance to louder sound.

When there is a hearing loss in one ear of greater than 20 dB HL many of the advantages of bilateral hearing are lost. This results in difficulty hearing in adverse listening situations such as in noise, group conversations, listening at a distance or in a reverberant environment. This can lead to significant disability and handicap. Difficulty with localisation may compromise safety.

In some individuals ‘end stage’ (stage 3) Ménière’s can result in a severe to profound hearing loss. Bilateral Ménière’s is reported in 17% to 50% of affected individuals and can be a cause of bilateral profound sensorineural hearing loss.

Why is there a feeling of fullness / pressure in the ear?

Another characteristic of Ménière’s is the sensation of ‘fullness’ or aural pressure which can be incredibly uncomfortable. Some patients tell us they can gauge their condition is starting again if they notice a change in the sensation of the ‘fullness’. The fullness can also fluctuate with the acuteness of the condition. For some people this sensation may disappear completely,  however for others it can become chronic with the constant feeling of pressure and this can cause considerable distress.

What investigations are necessary to make the diagnosis?

There is no specific test that, on its own, is reliable in diagnosing Ménière’s. The three main symptoms of vertigo, hearing loss and tinnitus occur in many other illnesses, and these may need to be excluded by tests (e.g. blood tests, MRI scan) before a final diagnosis can be made.

How is Ménière’s treated?

Treatment of Ménière’s is aimed at reducing and controlling symptoms. As Ménière’s is symptomatic, treatment will vary with the needs of each individual and includes medication, vestibular rehabilitation, diet and lifestyle changes, tinnitus management, hearing aids and counselling. In four out of five people non-surgical measures are sufficient to control the symptoms of Ménière’s; however, if vertigo remains a problem surgical procedures can help.

Further information about Ménière's disease/syndrome

Please contact us for further information about Meniere's or to chat with a member of our team. 

Some of our supporters have shared their experience of Ménière's, read their case studies here: 

  • Ménière’s disease
  • Bilateral Ménière’s

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What are the long term effects of Meniere's disease?

It's common to experience a loss of balance or dizziness before or after these attacks. Permanent hearing loss and tinnitus continue to develop and may be worse during attacks of vertigo. During the later stages, the episodes of vertigo tend to occur less frequently and sometimes stop altogether over time.

What happens when you have Meniere's disease?

Ménière's disease is a disorder of the inner ear that causes severe dizziness (vertigo), ringing in the ears (tinnitus), hearing loss, and a feeling of fullness or congestion in the ear. Ménière's disease usually affects only one ear.

Does Meniere's disease affect the brain?

When the head moves, the endolymph also moves, which causes nerve receptors in the membranous labyrinth to signal the brain about the body's motion. Endolymph buildup in the labyrinth can interfere with the normal balance and hearing signals between the inner ear and the brain, resulting in Ménière's disease.

What triggers Ménière's disease?

The exact cause of Ménière's disease is unknown, but it's associated with a problem with pressure deep inside the ear. Factors that are thought to increase your risk include: poor fluid drainage in your ear. an immune system disorder.