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Understanding Ménière’s Disease 1: Causes and diagnosis

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Abstract
Pritchard, M.J. (2007) Understanding Ménière’s disease 1: causes and diagnosis. Nursing Times; 103: 45, 28–29.
This is part 1 of a two-part unit on Ménière’s disease and tinnitus. It examines the definition and symptoms of Ménière’s, its causes, incidence and risk factors. It identifies the difficulties faced by patients, as well as the problems healthcare professionals have in making this diagnosis.

Keywords: Ménière’s disease, Tinnitus, Assessment, hearing and balance

Click here for PDF articles and Portfolio Pages corresponding to this unit

Learning objectives

1. Describe the main symptoms experienced by a patient with Ménière’s disease.

2. Consider some of the possible causes of Ménière’s disease and tinnitus.

Author
Michael John Pritchard, ENG, BA with ENB Higher Award, RGN, DipHE, is advanced nurse practitioner in general surgery, breast, urology and orthopaedics, Wirral University Teaching Hospital NHS Foundation Trust.

Definition
Ménière’s disease, also known as idiopathic endolymphatic hydrops, is a condition characterised by recurring vertigo, hearing loss and tinnitus.

Tinnitus – derived from the Latin word ‘tinnire’ meaning ‘to ring’ or ‘a ringing’ – is the sensation of a sound in the ear or head that is not being produced by an external source. The sound can be of any pitch or type, continuous or intermittent. Attacks of Ménière’s disease can last from 20 minutes to several hours (NIDCD, 2001).

Background

Ménière’s disease was named after the French physician Prosper Ménière, who first described it in 1861. The disease is believed to originate within the inner ear. Fluid (endolymph) moves in the membranous labyrinth or semicircular canals within the bony labyrinth of the inner ear. When a person moves their head or body, the endolymph moves. This causes nerve receptors in the membranous labyrinth to send signals to the brain about the body’s motion.

A change in the volume of the endolymph fluid, or swelling or rupture of the membranous labyrinth, is thought to result in symptoms of Ménière’s disease (Ruckenstein and Harrison, 1999; Hallpike and Cairns, 1938).

Causes and incidence

The original triggers for Ménière’s disease are unknown. Possible causes include noise pollution, viral infections or biological factors such as slight abnormalities of the bones around the middle ear.

In Europe, around one in 500 people develops the condition (James and Thorp, 2005). It usually begins between the ages of 40 and 60 but can start at a younger age (Moffat and Ballagh, 1997). It affects men and women equally. In most patients, only one ear is affected but in a small number (approximately 15%) both ears are involved (NIDCD, 2001).

Other conditions, such as injury and infection, may have similar symptoms to Ménière’s disease. Tumours in the inner ear or auditory nerve may also cause deafness, tinnitus or vertigo. However, Ménière’s disease is the likely cause if the symptoms are intermittent.

Genetic factors may be involved. Around 8% of relatives of people with Ménière’s develop the disease, compared with one in 1,000 in the general population.

Tinnitus itself can occur in isolation or in association with any type of hearing loss. Apart from Ménière’s disease, it may be a feature of presbycusis (age-related hearing loss), the environment, acoustic neuroma or caused by drug toxicity. It is common in industrial countries – as many as 18% of the general population may suffer with chronic tinnitus, with some 0.5% being severely affected (Coles, 1984).

Symptoms

Apart from tinnitus, symptoms of Ménière’s disease include severe dizziness or vertigo, hearing loss and a sensation of pain or pressure in the affected ear. Patients can feel nauseous and vomit. They may also sweat profusely and have uncontrollable eye movements. However, some individuals may only experience a single symptom such as an occasional bout of dizziness or periodic intense ringing in the ear.

Symptoms appear suddenly and can last from minutes to over eight hours, and can occur as often as daily or as infrequently as once a year (NIDCD, 2001; Friberg et al, 1984).

Attacks of severe vertigo can force the person to sit or lie down. They can also cause headaches, nausea, vomiting or diarrhoea. After an attack, a person may be sleepy and this can last for some hours. Hearing tends to recover between attacks but becomes progressively worse over time.

Tinnitus is not a minor symptom: it is a serious health issue. It can affect patients’ lives at all levels, including the psychological level. The noise never completely disappears, and remains even if patients become deaf.

It can also cause other problems – not only sleep disruption but also an inability to concentrate. In some cases, it can lead to depression (Zoger et al, 2001).

Tinnitus has an insidious onset and because of this there is a long delay before patients become aware of it. The evaluation of a patient with tinnitus requires a comprehensive history.

The following areas should be covered when assessing a patient to determine the likely cause:

  • Medical history;

  • Onset;

  • Location;

  • Pattern;

  • Characteristics (such as pitch, complexity);

  • Associated vertigo, aural fullness and hearing loss;

  • Exposure to ototoxic medications/factors;

  • Exacerbating/alleviating factors;

  • Hyperlipidaemia, thyroid disorder, vitamin B12 deficiency, anaemia.

The underlying mechanism of tinnitus is poorly understood. The condition can originate anywhere along the auditory pathway from the cochlear nucleus to the auditory cortex. It can take many forms and can be classified as vibratory/non-vibratory, or as subjective/objective (Crummer and Hassan, 2004).

Vibratory tinnitus is caused by the transmission to the cochlea of vibrations from adjacent tissues or organs, while non-vibratory tinnitus is produced by biochemical changes in the nerve mechanism of hearing.

Subjective tinnitus, which is the most common form, is heard only by the patient, whereas objective tinnitus can be heard through a stethoscope placed near the ear.

Tinnitus can affect a person in two ways: between episodes it is like a ringing noise, while during an attack it becomes a roar. Over time, hearing loss and tinnitus may either become permanent or subside.

Healthcare professionals should consider acoustic neuroma (an uncommon benign tumour) as a possible cause of Ménière’s disease. This slowly destroys the vestibular nerve so symptoms of dizziness or vertigo may be minimal or transient.

The first symptom the patient presents with is usually tinnitus, which may be present for months or years before hearing loss or vertigo is noticed.

Diagnoses of ménières

A diagnosis of Ménière’s is based on a full medical history and physical examination and also on a hearing and balance test. These tests also evaluate the extent of hearing loss.

In patients with the disease, hearing is not the only sense affected – in about 50% the balance function is reduced in the affected ear. This can be tested by electronystagnography (ENG). Since the eyes and ears work together to coordinate balance, ENG is used to test the balance system. Other tests, such as a CT and/or an MRI scan of the head, can be carried out to rule out any tumours.

The tests for tinnitus include hearing, balance and blood. Patients may be sent to a specialist ENT surgeon for further examinations such as MRI scans to investigate the inner ear.

Depending on symptoms and after X-rays of the head and face, they may be referred to an orthodontist if the problem is linked to the temporomandibular joint. The aim of these tests is to rule out infections, general autoimmune diseases or neuromas.

For tinnitus there are a number of tests that focus on the head and neck looking at the oral cavity, outer ear, tympanic membranes, cranial nerves, the temporomandibular joint and auscultation of the heart, carotid arteries, and periaural region.

A comprehensive audiological evaluation is vital to quantify any hearing loss and identify any treatable conductive component of hearing loss. The tests include the measurement of pure-tone thresholds with air and bone conduction.

Treatment

While there is no cure for Ménière’s disease, symptoms can be treated with medication, surgery, or dietary and behavioural changes.
Unfortunately, there are few options for those who have chronic tinnitus with no easily treatable cause. One non-clinical approach advocated for these patients is the idea of ‘not listening to the tinnitus’ or ‘tuning out’, the key being to concentrate on other things. If hearing is impaired, another method that could be considered is the use of a ‘masking out’ hearing aid.

This looks like a conventional hearing aid but is in fact a sound generator that produces a particular sound to mask the tinnitus. This, in conjunction with training, can help minimise the irritating aspects of tinnitus.

The most important tool in the treatment of this condition is to manage the patient’s psychological attitude, as it has been shown that depression or anxiety can make tinnitus worse.

Conclusion

Ménière’s disease can overwhelm some people. It can have severe psychological effects, especially when they have to deal with the constant noise of tinnitus.

Unfortunately, there is no cure for Ménière’s at this time. It is a progressive condition that can fluctuate unpredictably: patients may face years of symptoms or there may be a natural resolution.

Part 2 of this unit, published in next week’s issue, will discuss the treatment options for
Ménière’s disease.

Key references

Crummer, R.W., Hassan, G.A. (2004) Diagnostic approach to tinnitus. American Family Physician; 69: 1, 120–126.

James, A., Thorp, M. (2005) Ménière’s disease. Clinical Evidence; 14: 659–664.

National Institute on Deafness and other Communication Disorders (NIDCD) (2001) Ménière’s disease. www.nidcd.nih.gov

Ruckenstein, M.J., Harrison, R.V. (1999) Cochlear pathology in Meniere`s disease. In: Harris, J.P. (ed) Ménière’s Disease. Netherlands: Kugler Publications.

Zoger, S. et al (2001) Psychiatric disorders in tinnitus patients without severe hearing impairment: 24 month follow-up of patients at an audiological clinic. Audiology; 40: 133–140.

The full reference list for this part of the unit is available in Portfolio Pages

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