Vestibular Neuritis and Labyrinthitis
Timothy C. Hain, MD
Reviewed 10/2008
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Figure 1:
Cutaway of the inner ear. Movement of the head is detected by the semicircular canals, and transmitted to the brain via the vestibular nerve. Vestibular neuritis may affect the nerve itself or the vestibular ganglion (Scarpa's ganglion). |
What is Vestibular Neuritis and Labyrinthitis?
Vestibular neuritis causes dizziness due to a viral infection of the vestibular nerve (see Figure 1). The vestibular nerve carries information from the inner ear about head movement. When one of the two vestibular nerves is infected, there is an imbalance between the two sides, and vertigo appears. Vestibular neuronitis is another term that is used for the same clinical syndrome. The various terms for the same clinical syndrome probably reflect our lack of ability to localize the site of lesion.
While there are several different definitions for vestibular neuritis in the literature, with variable amounts of vertigo and hearing symptoms, we will use the definition of Silvoniemi (1988) who stated that the syndrome is confined to the vestibular system. Hearing is unaffected.
Labyrinthitis is a similar syndrome to vestibular neuritis, but with the addition of hearing symptoms (sensory type hearing loss or tinnitus).
The symptoms of both vestibular neuritis and labyrinthitis typically include dizziness or vertigo, disequilibrium or imbalance, and nausea. Acutely, the dizziness is constant. After a few days, symptoms are often only precipitated by sudden movements. A sudden turn of the head is the most common "problem" motion. While patients with these disorders can be sensitive to head position, it is generally not related to the side of the head which is down (as in BPPV), but rather just whether the patient is lying down or sitting up.
About 5% of all dizziness (and perhaps 15% of all vertigo) is due to vestibular neuritis or labyrinthitis. It occurs in all age groups, but cases are rare in children.
Recurrent vestibular neuritis -- Benign recurrent vertigo
Fortunately, in the great majority of cases (at least 95%) vestibular neuritis it is a one-time experience. A recent study of 101 patients found only 2 experienced a recurrence, both within 3 years of initial diagnosis (Huppert, 2006). Rarely the syndrome is recurrent, coming back year after year. When it is recurrent, the symptom complex often goes under other names. These include benign paroxysmal vertigo in children (Basser, 1964), benign recurrent vertigo (Slater 1979, Moretti et al, 1980), or Ménière's disease (Rassekh and Harker, 1992). Many authors attribute this syndrome to migraine associated vertigo. There is often a familial pattern (Oh et al, 2001)
What Causes Vestibular Neuritis and Labyrinthitis?
In vestibular neuritis, the virus that causes the infection is thought to be usually a member of the herpes family, the same group that causes cold sores in the mouth as well as a variety of other disorders (Arbusow et al, 2000). This is not the same herpes virus involved in genital herpes. It is also thought that a similar syndrome, indistinguishable from vestibular neuritis, can be caused by loss of blood flow to the inner ear (Fischer, 1967). However, present thought is that inflammation, presumably viral, is much more common than loss of blood flow.
In labyrinthitis, it is also thought that generally viruses cause the infection, but rarely labyrinthitis can be the result of a bacterial middle ear infection. In labyrinthitis, hearing may be reduced or distorted in tandem with vertigo. Both vestibular neuritis and labyrinthitis are rarely painful -- when there is pain it is particularly important to get treatment rapidly as there may be a treatable bacterial infection or herpes infection.
There are several possible locations for the damage to the vestibular system that manifests as vestibular neuritis. There is good evidence for occasional lesions in the nerve itself, as this can be seen lighting up on MRI scan. There is also reasonable evidence that vestibular neuritis often spares part of the vestibular nerve, the inferior-division (Fetter and Dichgans, 1996; Goebel et al, 2001). Because the inferior division supplies the posterior semicircular canal and saccule, even a "complete" loss on vestibular testing may be associated with some retained canal function. Furthermore, it is common to have another dizziness syndrome, BPPV, follow vestibular neuritis. Presumably this happens because the utricle is damaged (supplied by the superior vestibular nerve), and deposits loose otoconia into the preserved posterior canal.
There is also neuropathological evidence for loss of vestibular ganglion lesions. For example, pathologic study of a single patient documented findings compatible with an isolated viral infection of Scarpa's ganglion (the vestibular ganglion). There was loss of hair cells, epithelialization of the utricular maculae and semicircular canal cristae on the deafferented side, and reduced synaptic density in the ipsilateral vestibular nucleus (Baloh et al, 1996).
Finaly, there is also some evidence for viral damage to the brainstem vestibular nucleus (Arbusow et al, 2000). Since the vestibular neurons are distinct from cochlear neurons in the brainstem, a brainstem localization as well as the vestibular ganglion makes more sense than the nerve lesions in persons with no hearing symptoms. Nevertheless, if the nerve were involved after it separates from the cochlear nerve, neuritis would still be a reasonable mechanism. Prior to death and autopsy there is no way to make a clear distinction.
How is Vestibular Neuritis and Labyrinthitis Diagnosed?
Acutely, in uncomplicated cases, while a thorough examination is necessary, no additional testing is usually required. Certain types of specialists, namely otologists, neurotologist, and otoneurologists, are especially good at making these diagnoses and seeing one of these doctors early on may make it possible to avoid unnecessary testing. In large part, the process involves ascertaining that the entire situation can be explained by a lesion in one or the other vestibular nerve. It is not possible on clinical examination to be absolutely certain that the picture of vestibular neuritis is not actually caused by a brainstem or cerebellar stroke, so mistakes are possible. Nevertheless, this happens so rarely that it is not necessary to perform magnetic resonance imaging (MRI) scans or the like very often.
Signs of vestibular neuritis include spontaneous nystagmus, and unsteadiness. Occasionally other ocular disturbances will occur such as skew deviation (Safran et al, 1994) and asymmetric gaze evoked nystagmus.
However, if symptoms persist beyond one month, reoccur periodically, or evolve with time (see following), testing may be proposed. In this situation, nearly all patients will be asked to undergo an audiogram and anElectronystagmography (ENG). An audiogram is a hearing test needed to distinguish between vestibular neuritis and other possible diagnoses such as Ménière's disease and Migraine. The ENG test is essential to document the characteristic reduced responses to motion of one ear. An MRI scan will be performed if there is any reasonable possibility of a stroke or brain tumor. Occasionally one can visualize the inflammation of the vestibular nerve. In most instances, it is most cost effective to see a neurologist prior to obtaining an MRI. Blood tests for diabetes, thyroid disorders, Lyme disease, collagen vascular disease and syphilis are sometimes performed, looking for these treatable illnesses. However, it is rare that these are ever positive.
How is Vestibular Neuritis and Labyrinthitis Treated?
Acutely, vestibular neuritis is treated symptomatically, meaning that medications are given for nausea (anti-emetics) and to reduce dizziness (vestibular suppressants). Typical medications used are Antivert (meclizine), Ativan (lorazepam) , Phenergan, Compazine, and Valium (diazepam) . When a herpes virus infection is strongly suspected, a medication called acyclovir or a relative may be used. Steroids (prednisone, methylprednisolone or decadron) are also used for some cases. A recent study found methylpredisolone to significantly improve recovery in most patients (Strupp, 2004). Acute labyrinthitis is treated with the same medications as as vestibular neuritis, plus an antibiotic such as amoxicillin if there is evidence for a middle ear infection (otitis media), such as ear pain and an abnormal ear examination suggesting fluid, redness or pus behind the ear drum. Occasionally, especially for persons whose nausea and vomiting cannot be controlled, an admission to the hospital is made to treat dehydration with intravenous fluids. Generally, admission is brief, just long enough to rehydrate the patient and start them on an effective medication to prevent vomiting.
Because the symptoms associated with labyrinthitis and vestibular neuritis are associated with acute inflammation, laboratory trials have evaluated the use of the potent anti-inflammatory agent etanercept (TNF-alpha) and found it no more helpful than steroids (Lobo, 2006).
It usually takes three weeks to recover from vestibular neuritis or labyrinthitis. Recovery happens due to a combination of the body fighting off the infection, and the brain getting used to the vestibular imbalance (compensation). Some persons experience persistent vertigo or discomfort on head motion even after three weeks have gone by. After two to three months, testing (that is,an ENG, audiogram and others) is indicated to be certain that this is indeed the correct diagnosis. A vestibular rehabilitation program, may help speed full recovery via compensation.
How Might Vestibular Neuritis and Labyrinthitis Affect My Life?
You will probably be unable to work for one or two weeks. You may be left with some minor sensitivity to head motion which will persist for several years, and may reduce your ability to perform athletic activities such as racquetball or volleyball. After the acute phase is over, for a moderate deficit, falls are no more likely than in persons of your age without vestibular deficit (Herdman et al, 2000).
Research Studies in Vestibular Neuritis and Labyrinthitis
Vestibular neuritis and labyrinthis are well recognized clinical syndromes. In our view, research is needed to quickly sort out the cause of this syndrome. We also hope that eventually better treatment, such as antivirals may be found. At the American Hearing Research Foundation (AHRF), we have funded research on similar conditions in the past. If you would you would like more information about contributing to the AHRF's efforts, click here.
Acknowledgments
Figure 1 is courtesy of Northwestern University.
References
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