Meniere’s Disease – The Healing Space Clinic

Meniere's Disease

BASICS

DESCRIPTION

An inner ear (labyrinthine) disorder in which there is an increase in volume and pressure of the inner-most fluid of the inner ear (endolymph), resulting in recurrent attacks of hearing loss, tinnitus, vertigo, and fullness

Usually unilateral, but in 10-50% may later involve the second ear
Severity and frequency may diminish over the years, but with increasing loss of hearing. It is not a synonym for dizziness
System(s) affected: Nervous
Genetics: N/A
Incidence/Prevalence in USA:
- No reliable figures are available to provide comprehensive numbers for incidence and prevalence by age and sex, but using incidence figures from a Swedish study conducted in 1973, it is estimated that the incidence of Ménière's disease in the US is 46 (new cases/100,000/year). No figures for sex and age are available, but the disease is relatively equal in males and females, and is extremely rare in children
- Using extrapolation, the estimated prevalence is 1,150 (cases/100,000 population)
Predominant age: Usual age of onset 20-60
Predominant sex: Male = Female

SIGNS AND SYMPTOMS

Hearing loss - low frequency, fluctuating
Vertigo - spontaneous attacks, duration 20 minutes to several hours
Ear fullness
Occurs as attacks, with intervening remission
During severe attacks
- Pallor
- Sweating
- Nausea and vomiting
- Falling
- Prostration
- All symptoms aggravated by motion
- Between attacks may experience motion-related imbalance without vertigo

CAUSES

Unknown. Best theory is inner ear response to variety of injuries (reduced middle ear pressure, allergy, endocrine disease, lipid disorders, vascular, viral, luetic)
Recent theory is intracranial compression of balance nerve by blood vessel

RISK FACTORS

Caucasian
Stress
Allergy
Increased salt intake
Noise

DIAGNOSIS

LABORATORY

Lab studies done to rule out other conditions
Serologic tests specific for Treponema pallidum - microhemagglutination (MHA), fluorescent treponemal antibody (FTA), Treponema immobilization test (TPI)
Thyroid studies
Lipid studies

Drugs that may alter lab results: Any medication that produces a significant degree of sedation is likely to affect vestibular testing and invalidate it
Disorders that may alter lab results: Many conditions may produce auditory and vestibular findings identical to those associated with Ménière's disease, making it a diagnosis of exclusion. A low frequency sensorineural hearing loss (nerve loss as opposed to conductive loss) is seen on audiometry, and a reduced caloric response on caloric testing is usual.

PATHOLOGICAL FINDINGS

Autopsy only. Shows dilation of inner ear fluid system (endolymph).

SPECIAL TESTS

Otoscopy with air pressure applied to the tympanic membrane
Auditory
- Hearing test (audiometry using pure tone and speech) to show low frequency sensorineural [nerve] loss and impaired speech discrimination
- Tuning fork test (Weber and Rinne) to confirm validity of audiometry
- Auditory Brainstem Response audiometry (ABR) to rule out acoustic neuroma
Vestibular
- Spontaneous nystagmus (rapid rhythmic eye motion) seen visually. Must avoid eye fixation by having patient use 40 diopter glasses for test
- Caloric testing - electronystagmography (ENG) may show reduced caloric response. Can obtain reasonably comparable information with use of 0.8 cc ice water instilled in ear canal, then noting duration and frequency of resulting nystagmus with 40 diopter lenses in place. Reduced activity on either side is consistent with Ménière's diagnosis, but is not diagnostic

IMAGING

MRI to rule out acoustic tumor, which can produce identical symptoms and findings

DIAGNOSTIC PROCEDURES

N/A

TREATMENT

APPROPRIATE HEALTH CARE

Can usually be managed in outpatient setting. Inpatient for surgery.

GENERAL MEASURES

Medications are given primarily for symptomatic relief of vertigo and nausea. There is no medication available that influences the disease process
For attacks, bedrest with eyes closed and protection from falling. Attacks rarely last longer than four hours
Streptomycin therapy for bilateral Ménière's disease, when conventional management has failed. Streptomycin may be administered over a period of several days or weeks intentionally to damage the neuro-epithelium of the balance centers and reduce their function. Hearing must be carefully monitored during this time so that the treatment does not proceed to the point of damaging the hearing structures. This form of treatment should be administered only by an otolaryngologist and after careful patient education

SURGICAL MEASURES

Hearing good: Endolymphatic sac surgery, either (1) decompression or (2) drainage of endolymph into mastoid or subarachnoid space. Alternative procedure is to cut the vestibular nerve (intracranial procedure). A newer procedure involves placement of gentamicin through the tympanic membrane into the middle ear space
Hearing poor, but usable: Can do sac procedure, nerve section, or gentamicin instillation depending on quality of hearing. For poor hearing, can decompress cochlea (cochleocentesis), or perfuse cochlea with streptomycin
Hearing not useful: Destruction of inner ear (labyrinthectomy)

ACTIVITY

Limit activity during attacks
Between attacks patient may be fully active, but this may be limited by
- Fear of impending attack
- Unsteadiness following attacks
- Ear fullness or tinnitus
- Hearing loss in involved ear that may severely limit the patient's ability to perform work duties or to participate in social life

DIET

Limit total intake during attacks because of nausea. Otherwise diet is usually not a factor unless attacks are brought on by certain foods. A restricted salt diet may be useful in some cases.

PATIENT EDUCATION

Many otolaryngologists keep booklets on Ménière's disease as handouts. Ask your otolaryngology consultant for a supply.

FOLLOW UP

PREVENTION/AVOIDANCE

Reduce stress
Reduce salt intake
Don't smoke
Avoid significant noise exposure, or use ear protectors
Avoid use of ototoxic medications (aspirin, quinine, kanamycin, and many others)

POSSIBLE COMPLICATIONS

Failure to diagnose acoustic neuroma
Loss of hearing
Injury during attack
Inability to work

EXPECTED COURSE AND PROGNOSIS

Alternating attacks and remission
Over time the balance problem tends to resolve, but the hearing worsens
The great majority of patients can be managed successfully with medication. About 5-10% of patients require surgery for incapacitating vertigo
Very important not to overlook acoustic tumor, which produces an identical clinical picture

MISCELLANEOUS

ASSOCIATED CONDITIONS

Cochlear hydrops (hearing problem only)
Vestibular hydrops (balance problem only)
Drop attacks

AGE-RELATED FACTORS

Pediatric: Unusual, but occasional. Dizziness in children likely to be on basis of significant central nervous system disease.
Geriatric: Less likely to occur in elderly. Patients exposed to loud noise levels over many years are more susceptible.
Others: Usual onset age 20-60

PREGNANCY

Not a common problem, but difficult to treat because of risk of producing fetal abnormalities with medication

OTHER NOTES

Hearing loss

ABBREVIATIONS

N/A

Clinical Investigations

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