Vestibular Neuritis and Labyrinthitis

Timothy C. Hain, MD Page last modified: September 6, 2009 Go to Spanish version In vestibular neuritis, dizziness is attributed 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. The term "neuritis" implies damage to the nerve, and "neuronitis', damage to the sensory neurons of the vestibular ganglion. There is actually evidence for both. There is also some evidence for viral damage to the brainstem vestibular nucleus (Arbusow et al, 2000), a second potential "neuronitis". As the vestibular neurons are distinct from cochlear neurons in the brainstem, this localization (as well as the vestibular ganglion) makes more sense than the nerve 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, and the present favored term is "neuritis". Labyrinthitis, is defined as the combination of the symptoms of vestibular neuritis, with the addition of hearing symptoms. It may be due to a process that affects the inner ear as a whole, or due to a process that affects the 8th nerve as a whole. Labyrinthitis is also always attributed to an infection.

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). 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). There is some controversy about this idea however, as there is little direct evidence for herpes infection (Matsuo, 1986). The varacella zoster virus (the cause of Ramsey Hunt) is also thought to be a common source of vestibular neuritis. It is also thought that a similar syndrome indistinguishable from vestibular neuritis can be caused by loss of blood flow to the vestibular system (Fischer, 1967). However, present thought is that inflammation, presumably viral, is much more common than loss of blood flow. There are still some that disagree (Fattori et al. 2003) . In labyrinthitis, it is thought that generally viruses cause the infection, but rarely labyrinthitis can be the result of a bacterial middle ear infection. While there are several different definitions of 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 of vestibular neuritis is confined to the vestibular system. In vestibular neuritis,

by definition, hearing is unaffected. In labyrinthitis, hearing may be reduced or distorted in tandem with vertigo. These definitions are flawed -- they depend on clinical findings and imply anatomic localization that may not always be true. Recently evidence has been put forth that some patients with the clinical syndrome of "vestibular neuritis", anatomically may have lesions in the labyrinth (Murofushi et al, 2003). Although anatomic data is rarely available, if diagnostic technology improves in the future, we may need to change the definition of "vestibular neuritis". 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. 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. 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). In spite of the limited pathology that would suggest involvement of the entire vestibular nerve, there is reasonable evidence that vestibular neuritis often spares part of the vestibular nerve, the inferior-division (e.g. Fetter and Dichgans, 1996; Goebel et al, 2001) as well as spare the superior division (Aw et al, 2001) although not all agree (Lu et al, 2003). Because the inferior division supplies the posterior semicircular canal and saccule, even a "complete" loss on vestibular testing (associated with a superior canal lesion) may be associated with some retained canal function. Similarly, an inferior division vestibular neuritis might be associated with a normal ENG test but an abnormal VEMP test. 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 (supplied by the inferior vestibular nerve).

How Common is Vestibular Neuritis ?

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.

How is the diagnosis of Vestibular Neuritis and Labyrinthitis made?

Acutely, in uncomplicated cases, while a thorough examination is necessary, no additional testing is usually required. Certain types of specialists, "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 or medication. 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, or an inner ear tumor, so mistakes are possible (Lee et al, 2003). Nevertheless, this happens so rarely that it is not necessary to perform MRI scans or the like very often. In severe or complex situations , we will sometimes (not always) order the following tests

Audiogram (to distinguish labyrinthitis from neuritis, and to detect Meniere's disease) VEMP (to detect vestibular neuritis) MRI of brain and IAC, with and without contrast (to be sure there are no tumors, inflammation of nerve or cochlea). Blood testing (fasting blood sugar, Lyme, FTA, CRP, HSV-1 titer)

Differential diagnosis (common considerations)

Vestibular neuritis Labyrinthitis Inner ear "stroke" or "TIA". Inner ear tumor Meniere's disease Migraine associated vertigo

There are many medical conditions that can create roughly the same constellation of findings and symptoms as vestibular neuritis and labyrinthitis. Sorting these out usually is done by a physician who can combine clinical knowledge and experience with results of inner ear testing. A "classic" case of VN mainly relies on ascertaining that findings consist of a subacute (over hours) pure dizziness.

If there are hearing symptoms with dizziness, then labyrinthitis would be the first consideration. Menieres disease is also a very reasonable consideration. Some persons have a"stuttering" course -- a series of abrupt onset/stopping of symptoms (usually attributed to circulation problems). A constellation of accompanying signs that would be unusual in ear disease -- such as weakness, numbness, unusually prominent unsteadiness, or skew deviation can also help make this diagnosis. A slowly progressive dizziness is usually attributed to a slowly growing or worsening process, such as an acoustic tumor.

When there are hearing symptoms with spells, Meniere's disease would be considered more seriously. When there are headaches, prominent visual symptoms, and light sensitivity -- migraine.

Movie of nystagmus of vestibular neuritis. Signs of vestibular neuritis include spontaneous nystagmus, and unsteadiness. One may notice that vision is disturbed or jumpy on looking to a particular side. This usually means that the opposite ear is affected -- it is called "Alexander's Law" and is due to asymmetric gaze evoked nystagmus. Occasionally other ocular disturbances will occur such as vertical double vision -- skew deviation (Safran et al, 1994). 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 an ENG. An audiogram is a hearing test needed to distinguish between vestibular neuritis and other possible diagnoses such as Meniere's disease and Migraine. The ENG test is essential to document the characteristic reduced responses to motion of one ear. An example of this is shown on the caloric test page. A test called a VEMP may be helpful in determing the extent of damage (Lu et al, 2003). Also, VEMP can be helpful in confirming the diagnosis of vestibular neuritis as opposed to another process that has damaged the nerve as most persons with vestibular neuritis will have reduced ENG function but a present (abeit perhaps reduced) VEMP. VEMP's recover more quickly than other tests do in vestibular neuritis (Kim et al, 2008). An MRI scan will be performed if there is any reasonable possibility of a stroke or brain tumor. In most instances, it is most cost effective to see a neurologist prior to obtaining an MRI. As illustrated in the central vertigo cases here, sometimes these can be very difficult to detect at the bedside. Occasionally with an MRI one can visualize the inflammation of the vestibular nerve or of the labyrinth. A case of cochlear inflammation is shown here. 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.

See this page for all of the tests done on an example patient with vestibular neuritis. See this page for all of the tests done on an example patient with labyrinthitis. See this page for case of labyrinthitis with cochlear enhancement on MRI

How is Vestibular Neuritis and Labyrinthitis Treated?

Acutely, vestibular neuritis is usually 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. When a

circulation disturbance is suspected, an agent that reduces the likelihood of stroke may be used. Steroids (prednisone, methylprednisolone or decadron) are also used for some cases. Strupp and others (2004) recently reported that steroids (methylprednisolone for 3 weeks) significantly improved the recovery of peripheral vestibular function in patients with vestibular neuritis, while valacyclovir did not. All patients were also given an anticholinergic medication (pirenzepine). Side effects were encountered including a gastric ulcer in one patient and induction of diabetes in two others. Strupp and coauthors did not indicate a mechanism for the positive effect of steroids. If real, the effect may be via reduction of nerve swelling. 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. It usually takes 3 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 3 weeks have gone by. After two-three months, testing (i.e. an ENG, audiogram, VEMP, and others) is indicated to be certain that this is indeed the correct diagnosis and a referral to a vestibular rehabilitation program, may help speed full recovery via compensation.

How might vestibular neuritis 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, volleyball and similar activities. 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). Persons in certain occupations, such as pilots, may have a greater long term impact (Shupak et al, 2003). You may also have mild problems with your thinking. Even in persons who are well compensated, sensory integration seems to require more attention in persons with vestibular lesions than normal subjects (Redfern et al, 2003).

Recurrent vestibular neuritis -- Benign recurrent vertigo (BRV)

Fortunately, in the great majority of cases (at least 95%) vestibular neuritis it is a one-time experience. Rarely (5%) the syndrome is recurrent, coming back at least once, and sometimes 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 vestibular Meniere's syndrome (Rassekh and Harker, 1992). Many authors attribute this syndrome to migraine associated vertigo. There is often a familial pattern (Oh et al, 2001), and it may instead be an entity by itself (Lee et al, 2006) but lacking any clear diagnostic findings that distinguish it from recurrent vestibular neuritis or acephalgic migraine.

Recurrent labyrinthitis
When labyrinthitis recurs (i.e. there is hearing and dizziness that recurs), the diagnosis is often changed from labyrinthitis to "Meniere's disease". The reason for this is that the diagnostic criteria for Meniere's are essentially those of recurrent labyrinthitis. It is the author's impression that this "conversion" process occurs far more commonly than there is recurrent vestibular neuritis.

Quick spins
Another recurrence pattern in vestibular neuritis is the "quick spin" pattern - -people complain of brief spells lasting seconds to minutes in which the entire world rotates at high speed, then stops, without any hearing symptoms. This may occur as often as 50 times/day. This pattern of dizziness often responds to anticonvulsants such as carbamazepine or oxcarbamazine, and in these situations, may reasonably be attributed to vestibualar paroxysmia. . In this disorder, one can often recognize the patient the video-frenzel goggles. There is a paretic type spontaneous nystagmus and vibration induced nystagmus, which reverses with hyperventilation for 30 seconds. Case example of quick spins: A middle aged administrator complained of multiple spells of spinning vertigo with nausea unaccompanied by hearing symptoms. The spells lasted 1020 minutes, were accompanied by sweating and nausea. He has had times in which he has had three or four episodes per day. There appear to be no consistent triggers. On examination, a right-beating spontaneous nystagmus was observed. This reversed direction with hyperventilation. Hearing testing was normal as was ENG testing and MRI scan. After being started on oxcarbamazine, gradually increasing to 600 mg twice/day, his spells decreased to less than once/two weeks, and were minimal in intensity. Acknowledgment: The graphic of figure 1 is courtesy of was originally funded by NIH.

REFERENCES:

Arbusow V and others. Detection of herpes simplex virus type 1 in human vestibular nuclei. Neurology 2000:55:880-882. Aw and others, Individual semicircular canal function in superior and inferior vestibular neuritis, Neurology 2001;57:768-774 Baloh RW, Ishyama A, Wackym PA, Honrubia V. Vestibular neuritis: clinicalpathologic correlation. Otolaryngology HNS 114(4):586-92, 1996

Basser L. Benign paroxysmal vertigo of childhood: a variety of vestibular neuritis. Brain 87:141-152 Coats AC. Vestibular neuronitis. Acta Otolaryngol (stockh) (suppl) 251:1-32, 1969 Fattori B, Ursino F, Cristofani R, Galetta F and Nacci A (2003). "Relevance of plasma D-dimer measurement in patients with acute peripheral vertigo." J Laryngol Otol 117(6): 467-72. Fetter M, Dichgans J. Vestibular neuritis spares the inferior division of the vestibular nerve. Brain 119:755-763, 1996 Fischer CM. Vertigo in cerebrovascular disease. Arch Otolaryngol 85:529-534, 1967 Goebel JA, O'Mara W, Gianoli G. Anatomic considerations in vestibular neuritis. Otol Neurotol 22:512-518, 2001 Hart CW. Vestibular paralysis of sudden onset and probably viral etiology. Ann ORL 74:33-47, 1965 Herdman SJ, Blatt P, Schubert MC, Tusa RJ. Falls in patients with vestibular deficits. Am J. Otol 21:847-851, 2000 Hotson JR, Baloh RW. Acute vestibular syndrome. NEJM 9/3/1998, 680-685 Kim HA and others. Otolith dysfunction in vestibular neuritis. Recovery pattern and a predictor of symptom recovery. Neurology 2008:70:449-453 Lee H and others. Nodulus infarction mimicking acute peripheral vestibulopathy. Neurology 2003:60:1700-02 Lee H and others. A genome-wide linkage scan of familial benign recurrent vertigo: linkage to 22q12 with evidence of heterogeneity. Human Mol Gen 2006, 15, 2, 2006 Lu YC, Young YH. Vertigo from herpes zoster oticus: superior or inferior vestibular nerve origin? Laryngoscope 2003; 113: 307-11 Matsuo, T. (1986). "Vestibular neuronitis--serum and CSF virus antibody titer." Auris Nasus Larynx 13(1): 11-34. Moretti G, Manzoni G, Caffara P, Parma M. Benign recurrent vertigo and its connection with migraine. Headache 20:344-346, 1980 Murofushi T and others. The site of lesion in "vestibular neuritis". Study by galvanic VEMP. Neurology 2003, 61:p417. Oh AK and others. Familial recurrent vertigo. Am J. Med Gen 100:287-291, 2001 Rasekh CH, Harker LA. The prevalence of migraine in Meniere disease. Laryngoscope 102:135-138, 1992 Redfern MS and others. Cognitive influences in postural control of patients with unilateral vestibular loss. Gait and Posture 00 (2003), 1-11. Safran AB, Vibert D, Issoua D, Hausler R. Am J. Ophthalm. 118(2):238-45, 1994 Silvoniemi P. Vestibular neuronitis: an otoneurological evaluation. Acta Otolaryngol (Stockh) (Suppl) 453:1-72, 1988 Schuknecht HF, Kitamura K. Vestibular Neuritis. Ann ORL 79:1-19, 1981 SHUPAK A, Nachum Z, Stern Y, Tal D, Gil A, Gordon CR. Vestibular neuronitis in pilots: follow-up results and implications for flight safety. Laryngoscope 2003; 113: 316-21 Slater R. Benign recurrent vertigo. J. NNNP 42:363-367, 1979 Strupp M and others. Methylprednisolone, valacyclovir or the combination for vestibular neuritis. NEJM 35:4, July 22, 2004. 354-361

Zajtchuk J, Matz G, Lindsay J. Temporal bone pathology in herpes oticus. Ann ORL 81:331-338, 1972

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