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Labyrinthitis
Counselor,

Labyrinthitis is an inflammation or dysfunction of the vestibular labyrinth (a system of intercommunicating cavities and canals in the inner ear). The syndrome is defined by the acute onset of vertigo, commonly associated with head or body movement. Vertigo is often accompanied by nausea, vomiting, or malaise.

Vertigo is the subjective sensation of environmental movement that may be experienced as a mild subjective instability of the surroundings or, in its most severe form, as a spinning sensation. The severity of vertigo may be experienced and described anywhere between these 2 extremes. Vertigo syndromes have many synonyms, including labyrinthitis, benign positional vertigo, cupulolithiasis, and vestibular neuronitis; but they are all different.

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Pathophysiology:

The pathophysiology of labyrinthitis is not completely understood. However, a dysfunction of the vestibular apparatus is clearly present when labyrinthitis occurs. Theories as to the cause of labyrinthitis involve lesions in the otolith organs, degeneration of the utricular macula, or lesions in the posterior semicircular canal. The cupulolithiasis theory suggests that otoconia, which are formed by a dysfunctional utricular macula, settle on the cupula of the posterior semicircular canal and cause aberrant stimulation of the vestibular system. A more popular theory is that of canalithiasis, outlined by Hall in 1979. This theory postulates that free-floating otoconial debris produces pressure in the semicircular canal, causing deflection of the cupula. This deflection results in a transient attack of vertigo.

Many cases of labyrinthitis are associated with systemic or virallike illnesses. Suppurative or bacterial labyrinthitis is rare, but it should be considered in patients with acute or chronic otitis media. Cases of labyrinthitis are reported in association with meningitis, but the presentation of the meningitis often overwhelms the vestibular symptoms.


Frequency:

In 1987, a large study of 240 patients with benign paroxysmal vertigo was published and revealed the following etiologies: Idiopathic - 49% (118 patients), Posttraumatic - 18% (43 patients), Viral neurolabyrinthitis - 15% (37 patients), Miscellaneous - 18% (42 patients). The miscellaneous etiologies in this study included vertebrobasilar ischemia, Ménière disease, postsurgical ototoxicity, luetic labyrinthitis, and chronic otomastoiditis. Benign paroxysmal vertigo and Ménière disease are the most frequent causes of peripheral vertigo. Brainstem tumors, although quite rare, can also cause vertigo.

Mortality/Morbidity:

In the vast majority of patients with labyrinthitis syndrome, the acute episode is self-limited and lasts anywhere from days to weeks. Spontaneous recovery is the rule; however, recurrences are common, especially in the first 5 years following the first episode. In rare cases, the episode can last months or even years. Death is not associated with the syndrome unless an underlying condition (e.g.: brainstem tumor, vertebrobasilar ischemia) is present. Loss of vestibular function and/or hearing as a result of bacterial labyrinthitis is typically severe and permanent.

History:

Although the vast majority of cases of labyrinthitis are self-limited, a minority of patients can have a more chronic and prolonged course. Patients express difficulties involving vertigo and hearing. Patients with the labyrinthitis syndrome typically develop brief (<1 min) episodes of vertigo that are associated with head and/or body position changes. Acute episodes of position-sensitive vertigo may have characteristics that include the following: nausea, vomiting, malaise, a brief change in hearing lasting no more than a few seconds, and tinnitus and a subjective change in hearing which are the most commonly reported symptoms.

Physical:

The importance of a neurologic exam cannot be overemphasized. All serious causes of vertigo must be ruled out before the diagnosis of labyrinthitis can be considered and confirmed. Cranial nerves should be evaluated and hearing tests should be performed. The type of nystagmus should be noted, as one or two types of nystagmus are associated more with central causes of vertigo (serious) rather than peripheral causes (benign). Maneuvers to fatigue the vertigo help to distinguish serious causes from benign causes. Benign vertigo generally fatigues and abates with positional changes.

Lab Studies and Imaging Studies:

Routine laboratory studies are not helpful. A workup for an infectious etiology based on clinical suspicion may be performed. This workup could include blood cultures, a urine culture, a chest radiograph, or a cerebrospinal fluid (CSF) examination. In most patients with the labyrinthitis syndrome, no imaging studies are required, however, if a central vestibular lesion is suggested by clinical findings, then an MRI should be performed.

For those patients with a chronic labyrinthitis syndrome course, more specialized tests can be performed. These tests should be performed under the direction of a neurologist and/or an otolaryngologist. They include: Electronystagmography, caloric testing, hearing tests, and forced voluntary hyperventilation for 1-3 minutes to see if symptoms are reproduced.


Treatment:

The patient should lie still with his/her eyes closed during an acute attack. Medication used in the treatment of labyrinthitis is primarily for relief of symptoms. Several medications have antivertiginous properties (eg, meclizine, scopolamine, ephedrine, dimenhydrinate, diazepam) and others are useful as antiemetics (e.g.: promethazine, prochlorperazine). Most acute episodes of labyrinthitis are short-lived and self-limited. For those with a possible condition such as vertebrobasilar ischemia or a brainstem tumor, admission to the hospital is appropriate under the direction of a neurologist and/or a neurosurgeon.

Medical/Legal Concerns:

Failure to diagnose an underlying condition (e.g.: vertebrobasilar ischemia, brainstem tumor, severe cervical spondylosis) that results in further neurological injury is the major factor leading to litigation. Failure to recognize the rare case of suppurative labyrinthitis or the unusual case associated with meningitis can also lead to litigation. The other medical-legal concern is complications from medication prescribed (e.g.: an acute dystonic reaction secondary to an antiemetic).

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Attorney & Physician Advisory Board
AMFS, Inc.
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