Ménière’s disease is a long term, progressive 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 disease?
The incidence is between 1:1000 and 1:2000 of the population; depending on the source. Both sexes are equally affected and it can occur at any age. About 7-10% of sufferers have a family history of the disease.
What causes Ménière’s disease?
The cause of Ménière’s disease 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 disease however remains unclear.
How does Ménière’s disease 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 illness. 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 disease?
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.
It is widely believed that Ménière’s disease 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.
Consequences of hearing loss
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’ Ménière’s disease (stage 3) can result in a severe to profound hearing loss. Bilateral Ménière’s disease is reported in 17% to 50% of affected individuals and can be a cause of bilateral profound sensorineural hearing loss.
Fullness of the ear
Another characteristic of Ménière’s disease is the sensation of ‘fullness’ or aural pressure which can be incredibly uncomfortable. The fullness can also fluctuate and for some cause considerable distress. Some patients can gauge that their condition is starting again if they notice a change in the sensation of the ‘fullness’. For some this sensation may disappear completely however for others it can become chronic with the constant feeling of pressure. In patients with Ménière’s disease, fullness can fluctuate with the acuteness of the condition.
What investigations are necessary to make the diagnosis?
There is no specific test that, on its own, is reliable in diagnosing Ménière’s disease. 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 disease treated?
Treatment of Ménière’s disease 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 disease; however, if vertigo remains a problem surgical procedures can help.