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Causes
Common causes
Benign paroxysmal positional vertigo (BPPV)
Vestibular neuronitis
Infection
Middle ear infections eg. Otitis media, mastoiditis
Labyrinthitis
Herpes zoster oticus (Ramsay Hunt synd)
Structural disorder
Cholesteatoma
Perilymphatic fistula
otosclerosis
Trauma
Temporal bone fracture
Labyrinthine concussion
Dix-Hallpike Maneuver:
Gold standard
Head movement should be fairly quick WITH EYES OPEN
Head turned 45o while patient upright and neck extended 20o
Latency 5 -20s
Crescendo-decrescendo nystagmus up to 60 sec.
Dix-Hallpike Manuer
Caloric Test
Electro/videonystagmography (ENG)
Evaluation of vertigo
The Dix-Hallpike positional test (also known as the Nylen-Brny maneuver) is performed.
[20]
A positive test result may be indicates coexisting benign positional vertigo. The Dix
Hallpike test is performed as described below.
The patient is positioned in the middle of the table so that the head extends past the head of
the bed when he or she is supine. The patient is then rapidly moved backward so that the head
hangs over the edge, and the eyes are observed for evidence of nystagmus. If no nystagmus is
observed over a 20-second period, the patient is returned to the upright position. The next
step is to bring the patient rapidly to the head-right supine position, again looking for
nystagmus. The maneuver is then repeated for the head-left supine position.
Any nystagmus or symptoms, as well as the position the head was in when these symptoms
or signs were elicited, should be noted. Nystagmus typically has a latency of 2-5 seconds.
Nystagmus and vertigo associated with peripheral causes such as Mnire disease should be
fatigable. Central lesions should have no latency and do not fatigue.
If classic findings of benign paroxysmal positional vertigo are found during Dix-Hallpike
testing, then canalith-repositioning procedures may be performed.[21] Care should be taken to
ask about preexisting spine issues and/or neck issues before performing the Dix Hallpike test
in order to prevent injury.
The Romberg test generally shows significant instability and worsening during acute attacks
when the eyes are closed.
The Hennebert sign is nystagmus caused by positive and negative pressure in the external
auditory canal.[9]
The Tullio phenomenon is sound-induced vertigo, nystagmus, or both.[22] It is historically
associated with syphilis but has been described in Mnire disease.
Approach Considerations
Laboratory tests, though not specific for Mnire disease, should be directed at differentiating
the disease from other causes on the basis of associated symptoms. More extensive testing is
typically reserved for outpatient or inpatient workup and is not performed in the emergency
department (ED), including the following otologic tests:
Audiometry
Electrocochleography (ECOG)
Otoscopy
A patient with a history classic for Mnire disease normally does not need imaging studies
performed. If there is concern about the presence of other intracranial disease processes, then
magnetic resonance imaging (MRI) or computed tomography (CT) can be obtained.
Laboratory Studies
No blood test is specific for Mnire disease. However, the following studies may be ordered
to exclude obvious metabolic disturbances, infections, or hormonal imbalances:
Erythrocyte sedimentation rate (ESR) and antinuclear antibody (ANA) test to rule out
autoimmune disorders
Urinalysis to rule out proteinuria and hematuria and indicators of otorenal syndrome
Audiometry is particularly helpful for documenting present hearing acuity and detecting
future change. In any given patient, the audiogram may have a broad spectrum of results that
ranges from normal hearing to profound hearing loss, reflecting the fluctuating nature of the
impairment. From patient to patient, there is also a wide range of different types of hearing
loss.
The patient may not notice a loss at specific frequencies. Low-frequency or mixed low- and
high-frequency insufficiency may be observed. Typically, however, the lower frequencies are
affected more severely. This is due to preferential sensitivity of the apex to the hydrops.
Multiple hearing tests, which document fluctuating hearing loss, are helpful in diagnosing
Mnire.
Electrocochleography
ECOG is an electrophysiological test that reflects elevation of inner ear pressure.
Specifically, it detects distention of the basilar membrane of the inner ear. This distortion is
presumably due to elevated endolymph pressure associated with hydrops. The pressure may
cause the membranes to tear and the inner ear to misfire, causing vertigo.
ECOG measures the ratio of the summating potential (probably from the movement of the
basilar membrane) and the nerve action potential in response to auditory stimuli. Hydrops
(elevated pressure) is suggested when this ratio is greater than 35%. The test is most accurate
when Mnire disease is active.
Electronystagmography
ENG is a test of inner ear function (particularly the horizontal semicircular canals). The
test determines inner ear responsiveness to movement and caloric stimulation. It tests central
and peripheral function and can help localize the site of the lesion.
Administer the test when the patient has an empty stomach and after discontinuing meclizine
(Antivert), antihistamines, and sedatives for 2 weeks. These drugs may alter test results.
The caloric portion of the test is performed by irrigating the ear with warm air and then
cold air, with the patient in a supine position. The temperature differential causes the fluid
within the horizontal semicircular canal to move, triggering a nystagmus response. The
response on one side is compared with the opposite side. As with any reflex, one would
expect equal reactions on each side. Usually, anything that would cause a weakened
response would be considered pathological.
Principles of Medical Management
Medical treatment of Mnires disease is aimed at symptomatic relief. In an acutely
vertiginous patient, management is directed toward vertigo control.[25] Intravenous (IV) or
intramuscular (IM) diazepam provides excellent vestibular suppression and antinausea
effects. Steroids can be given for anti-inflammatory effects in the inner ear. IV fluid support
can help prevent dehydration and replaces electrolytes.
Typically, vestibulosuppressants and antinausea medications (eg, meclizine,
prochlorperazine) are prescribed for prn use. Note that frequent and long-term use of these
Pharmacologic Therapy
Vestibulosuppressants
Steroids have also been helpful in treating endolymphatic hydrops because of their antiinflammatory properties. Steroids can reverse vertigo, tinnitus, and hearing loss, probably by
reducing endolymphatic pressure. Steroids can be given orally, intramuscularly, or even
transtympanically. A trial of IM steroid injection followed by a tapering dose of oral steroids
has been recommended.[21] No trials evaluating the efficacy of systemic steroids for the
treatment of Mnire disease have been done.[26]
Although the transtympanic route is controversial, it is gaining wider acceptance throughout
the otologic community. Transtympanic steroid injection has been shown to be beneficial in
controlling loss of hearing and the number of vertigo attacks.[27]
Aminoglycosides
Aminoglycosides are reserved for end-stage intractable Mnire disease. They are a class of
antibiotics that were serendipitously discovered to be preferentially toxic to the vestibular
(balance) end organ. Destruction of the vestibular end organ renders the brain insensitive to
fluctuations in inner ear pressure brought on by Mnire disease.
When given systemically, aminoglycosides affect both ears. Although aminoglycosides can
be used to treat extremely severe bilateral Mnire disease, such treatment leaves the patient
with little or no balance function. The resulting complete loss of inner ear function (ie, Dandy
syndrome) can be debilitating.
Histamine agonists such as betahistine (Serc) are widely used in Europe and South America
for the treatment of Mnire disease. Betahistines mechanism of action has not been
established with certainty, but it is thought to act by increasing circulatory flow to the
cochlear stria vascularis[28] or through inhibition of vestibular nuclei activity.[29]
Many have reported success with the use of betahistine to mitigate symptoms of Mnire
disease. Unfortunately, because this agent has not been approved by the US Food and Drug
Administration (FDA), it is not discussed much in the United States.
Interventional management:
Labyrinthectomy
disease, much as for systemic hypertension, the goal is to reduce the total body fluid volume.
This, in turn, may reduce the inner ear fluid volume.
Because sodium seems to play a major role in fluid retention within the inner ear, avoiding
foods with high sodium content (eg, pizza, preserved foods, smoked fish) is paramount. Note
that many preserved and smoked foods contain sodium nitrite, which can contribute to high
sodium content. Consult with a nutritionist to establish a rigid salt-restricted diet (1.5 g
sodium/d).
Activity restriction
Vestibular Rehabilitation
Vestibular therapy is a physical therapy and occupational therapy modality that helps
habituate patients to their vestibular loss. It helps recalibrate a patient's balance by helping
them compensate for the effects of the inner ear disorder. It is performed by repetitive balance
exercises.
Because of the fluctuating nature of Mnire disease, vestibular therapy is not particularly
useful as a primary treatment. However, it is useful in the rehabilitation of patients who have
undergone vestibular ablation. In fact, vestibular rehabilitation is strongly recommended in
those who have undergone aminoglycoside perfusion, labyrinthectomy, or vestibular nerve
section. It can be helpful in teaching patients to cope with vertigo and imbalance.
Prevention
Quality evidence is lacking regarding deterrence and prevention of acute attacks of Mnire
disease; however, a salt restricted diet, as described elsewhere (see Diet and Activity), is often
suggested. In addition, avoidance of trigger substances may help prevent acute episodes. The
following substances should be avoided:
Caffeine
Nicotine
Tobacco
Finally, it is often suggested that patients try to avoid loud noises and to make use of stressreduction techniques.