Download Meniere`s Disease

Meniere’s Disease
5 Minute Clinical Consult overview (LOE = 5, expert opinion)
Basic information
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.
Systems affected:
 Nervous
Incidence/prevalence in US
 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 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)
 Male child: 10/100,000
 Female child: 10/100,000
 Male adult: 560/100,000
 Female adult: 560"/100,000
Predominant age
Usual age of onset 20-60
Predominant gender
Male = Female
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
Differential diagnosis
 Acoustic tumor
 Syphilis
 Perilymph fistula
 Multiple sclerosis
 Viral labyrinthitis
 Vertebrobasilar disease
 Other labyrinthine disorders producing same symptoms (Cogan's syndrome, benign
positional vertigo, temporal bone trauma)
Associated conditions
 Cochlear hydrops (hearing problem only)
 Vestibular hydrops (balance problem only)
 Drop attacks
Associated conditions
 Cochlear hydrops (hearing problem only)
 Vestibular hydrops (balance problem only)
 Drop attacks
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
o Pallor
o Sweating
o Nausea and vomiting
o Falling
o Prostration
o All symptoms aggravated by motion
o Between attacks may experience motion-related imbalance without vertigo
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 can alter labs
Any medication that produces a significant degree of sedation is likely to affect vestibular testing
and invalidate it
Conditions that can alter labs
Many conditions may produce auditory and vestibular findings identical to those associated with
Ménière 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
o Hearing test (audiometry using pure tone and speech) to show low frequency
sensorineural [nerve] loss and impaired speech discrimination)
o Tuning fork test (Weber and Rinne) to confirm validity of audiometry
o Auditory Brainstem Response audiometry (ABR) to rule out acoustic neuroma
o Electrocochleography (ECOG) may be useful to confirm etiology
 Vestibular
o Spontaneous nystagmus (rapid rhythmic eye motion) seen visually. Must avoid
eye fixation by having patient use 40 diopter glasses for test.
o 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 diagnosis, but is not diagnostic.
Imaging studies
MRI to rule out acoustic tumor, which can produce identical symptoms and findings
Treatment
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 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.
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.
Activity
 Limit activity during attacks
 Between attacks patient may be fully active, but this may be limited by (1) fear of
impending attack, (2) unsteadiness following attacks, (3) ear fullness or tinnitus, or (4)
hearing loss in involved ear that may severely limit the patient's ability to perform work
duties or to participate in social life
Possible complications
 Failure to diagnose acoustic neuroma
 Loss of hearing
 Injury during attack
 Inability to work
Medications
Drugs of choice
 Acute attack. For severe episode, one of the following may be used. Adult doses are
indicated
o Atropine 0.2-0.4 mg IV
o Diazepam (Valium) 5-10 mg IV slowly
o Transdermal scopolamine, 1 patch, or smaller segment of patch, applied to skin
surface and not replaced sooner than 3 days
 Maintenance. Adult doses are indicated (frequently must be reduced to avoid sedating
effects)
o Meclizine (Antivert, Bonine) 25-100 mg orally, either at bedtime or in divided
doses
o Ergotamine-belladonna-phenobarbital (Bellergal Spacetabs), one q 12 hr
o Diazepam (Valium), 2 mg (or less) tid
Contraindications

Atropine - cardiac disease, especially supraventricular tachycardia and other
arrhythmias
 Scopolamine - children and elderly
Precautions
 Sedating drugs should be used with caution, particularly in elderly people. The need to
reduce the dosage is common. Patients should be cautioned not to operate motor
vehicles when they are sedated.
 Atropine, scopolamine, and Bellergal should be used with particular caution. If not
prescribed frequently, refer to manufacturer's literature.
Interactions
 Bellergal - oral anticoagulants, tricyclic antidepressants, phenothiazines, narcotics, beta
blockers, estrogens, and others
 Transdermal scopolamine - anticholinergics, belladonna products, antihistamines,
tricyclic antidepressants, and others
Alternative drugs
 Acute attack
o Droperidol, 1.5-2.5 mg IV slowly (in hospital setting)
o Promethazine (Phenergan) 12.5-25 mg IV slowly
o Diphenhydramine (Benadryl) 50 mg IV slowly
o Carbogen (5% carbon dioxide and 95% oxygen) by mask from tank
 Maintenance
o Dimenhydrinate (Dramamine) 50 mg q4-6h po
o Promethazine (Phenergan) 12.5-25 mg q4-6h po
o Diphenidol (Vontrol) 25-50 mg tid po
o Diphenhydramine (Benadryl), 25-50 mg q6-8h po. Maximum, 100 mg/24 hours
o Chlorothiazide (Diuril) 500 mg daily po with potassium supplement
Follow-up
Expected course
 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 510% of patients require surgery for incapacitating vertigo.
 Very important not to overlook acoustic tumor, which produces an identical clinical
picture
Patient monitoring
The most common complaint by Ménière patients regarding prior treatment is that the primary
care physician did not take the condition seriously and that he or she did not seem interested in
providing ongoing care. Because of the emotional impact alone, these patients need close
followup care. It is important to monitor the status of their hearing, since it is at risk, and to
continue to consider the possibility of a more serious underlying problem such as an acoustic
tumor.
Prevention and 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)
Patient education
Many otolaryngologists keep booklets on Ménière disease as handouts. Ask your
otolaryngology consultant for a supply.
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.
Other ages
Usual onset age 20-60
Pregnancy
Not a common problem, but difficult to treat because of risk of producing fetal abnormalities with
medication
 Ménière syndrome
 Endolymphatic hydrops
ICD9 codes
 386.00: Meniere disease, unspecified
Other notes
 Hearing loss
References
 Lucente FE, Gady HE. Essentials of Otolaryngology. 4th ed. Philadelphia, Lippincott
Williams & Wilkins, 1999:116-125
Web sites
 http://www.vestibular.org>Vestibular Disorders Association
 http://oto.wustl.edu/men>Washington University - The Ménière's Page
 http://www.geocities.com/hotsprings/spa/3143>Meniere&rtsinglequote;s Disease Home
Page
Author(s)
 Gale Gardner, MD
Axial computed tomographic scan of the normal ear
In an axial CT scan, several anatomic landmarks can be identified. These
structures are important because their obliteration or alteration provides
important clues in the diagnosis of otitic infections. A, superior CT section
shows the head of the malleus and body of the incus, as well as the lateral
semicircular canal (LSCC) and vestibule. The mastoid air cell system is well
developed, with abundant pneumatization that emanates from the antrum.
Middle ear infections often spread into the mastoid via the antrum. B, A CT
section taken from a slightly more inferior position shows the turns of the
cochlea (arrow). Acute infection of the inner ear structures can lead to
permanent loss of vestibular function and profound deafness in the affected
ear. Acute otitis media does not usually cause ossicular destruction.
However, acute necrotizing otitis media, caused by -hemolytic
streptococci, can lead to destruction of the blood supply to the ossicles and
subsequent bony loss.