Background Labyrinthitis is an inflammatory disorder of the inner ear or labyrinth.

Clinically, this condition produces disturbances of balance and hearing to varying degrees and may affect one or both ears. Bacteria or viruses can cause acute inflammation of the labyrinth in conjunction with either local or systemic infections. Autoimmune processes may also cause labyrinthitis. Vascular ischemia may result in acute labyrinthine dysfunction that mimics labyrinthitis. Pathophysiology The anatomic relationships of the labyrinth, middle ear, mastoid, and subarachnoid space are essential to understanding the pathophysiology of labyrinthitis. The labyrinth is composed of an outer osseous framework surrounding a delicate membranous network that contains the peripheral sensory organs for balance and hearing. See the image below. These sensory organs include the utricle, saccule, semicircular canals, and cochlea. Symptoms of labyrinthitis occur when infectious microorganisms or inflammatory mediators invade the membranous labyrinth and damage the vestibular and auditory end organs. The labyrinth lies within the petrous portion of the temporal bone adjacent to the mastoid cavity and connects with the middle ear at the oval and round windows. The labyrinth maintains connections with the central nervous system and subarachnoid space by way of the internal auditory canal and cochlear aqueduct. Bacteria may gain access to the membranous labyrinth by these pathways or through congenital or acquired defects of the bony labyrinth. Viruses may spread to labyrinthine structures hematogenously or by way of the aforementioned preformed pathways. Viral and bacterial labyrinthitis are sufficiently different to warrant discussing them as separate disease processes. Viral labyrinthitis Viral labyrinthitis is characterized by a sudden, unilateral loss of vestibular function and hearing. The acute onset of severe, often incapacitating, vertigo, frequently associated with nausea and vomiting, is characteristic of this disorder. The patient is often bedridden while the symptoms gradually subside. Vertigo eventually resolves after several days to weeks; however, unsteadiness and positional vertigo may persist for several months. Hearing loss is common and may be the primary presenting symptom in many patients. Physical examination findings include spontaneous nystagmus towards the unaffected side with diminished or absent caloric responses in the affected ear. The hearing loss is usually mild to moderate and typically evident in the higher frequencies (>2000 Hz), although any degree or type of hearing loss may be present. An upper respiratory tract infection precedes the onset of cochleovestibular symptoms in up to 50% of cases. Recurrent attacks are reported but are rare and may be confused with Mnire disease. Resolution of vertigo and dysequilibrium is common and is due to partial recovery of vestibular function with concurrent central compensation of the remaining unilateral vestibular deficit. Return of hearing usually mirrors the return of vestibular function. Viral labyrinthitis is often confused with vestibular neuritis, and the terms are occasionally used interchangeably in the literature. However, most authors agree that vestibular neuritis is a disorder of the vestibular nerve and is not associated with hearing loss.[1] Because the cochlea is affected in pan-labyrinthine inflammation, hearing loss is always present in persons with viral labyrinthitis. Vestibular neuritis typically manifests as sudden acute vertigo without hearing loss in an otherwise healthy patient. The condition is more common in the fourth and fifth decades of life and affects men and women equally. An upper respiratory tract infection often precedes the condition, and the disorder is more common in the spring and early summer. Histopathological nerve studies of patients with vestibular neuritis demonstrate axonal loss, endoneurial fibrosis, and atrophy. [1] These findings are consistent with a viral inflammatory etiology. The treatment of vestibular neuritis and viral labyrinthitis is similar. A unique form of viral labyrinthitis is herpes zoster oticus, or Ramsay-Hunt syndrome. The cause of this disorder is reactivation of a latent varicella-zoster virus infection occurring years after the primary infection. Evidence suggests that the virus may attack the spiral and vestibular ganglion in addition to the cochlear and vestibular nerves. [2] The initial symptoms are deep, burning, auricular pain followed a few days later by the eruption of a vesicular rash in the external auditory canal and concha. Vertigo, hearing loss, and facial weakness may follow singly or collectively. Symptoms typically improve over a few weeks; however, patients often suffer permanent hearing loss and persistent reduction of caloric responses. [3] Viral infections can cause both congenital and acquired hearing loss. Rubella and cytomegalovirus are the best-recognized viral causes of prenatal hearing loss. Virally induced hearing loss in the postnatal period is usually due to mumps or measles. Viral infections are also implicated in idiopathic sudden sensorineural hearing loss (SNHL). Experimental evidence suggests that inflammatory proteins play a critical role in the pathogenesis of cytomegalovirus-induced hearing loss. [4] More specific information regarding these viral diseases are covered in other articles. (See eMedicine Pediatric articles Measles, Mumps, Cytomegalovirus Infection, and Rubella.) Bacterial labyrinthitis Bacterial labyrinthitis is a potential consequence of meningitis or otitis mediaand may occur by either direct bacterial invasion (suppurative labyrinthitis) or through the passage of bacterial toxins and other inflammatory mediators into the inner ear (serous labyrinthitis). Meningitis typically affects both ears, whereas otogenic infections typically cause unilateral symptoms.

Bacteria can spread from the cerebrospinal fluid to the membranous labyrinth by way of the internal auditory canal or cochlear aqueduct. Bacterial infections of the middle ear or mastoid most commonly spread to the labyrinth through a dehiscent horizontal semicircular canal.[5] Usually, the dehiscence is the result of erosion by a cholesteatoma. Suppurative labyrinthitis resulting from otitis media is uncommon in the postantibiotic era. When suppurative labyrinthitis occurs, it is almost always associated with cholesteatoma. Profound hearing loss, severe vertigo, ataxia, and nausea and vomiting are common symptoms of bacterial labyrinthitis. Treatment of suppurative labyrinthitis is aimed at eradicating the underlying infection, providing supportive care to the patient, draining middle ear effusions or mastoid infections, and preventing the spread of infection. Labyrinthitis ossificans often follows suppurative labyrinthitis; therefore, decisions regarding cochlear implantation must be made early. Meningitis also may result in progressive hearing loss secondary to necrosis and fibrosis of the membranous cochlea and labyrinth. [6] Serous labyrinthitis occurs when bacterial toxins and host inflammatory mediators, such as cytokines, enzymes, and complement, cross the round window membrane, causing inflammation of the labyrinth in the absence of direct bacterial contamination.[7] This condition is associated with acute or chronic middle ear disease and is believed to be one of the most common complications of otitis media. Toxins, enzymes, and other inflammatory products infiltrate the scala tympani, forming a fine precipitate just medial to the round window membrane. Penetration of the inflammatory agents into the endolymph at the basilar turn of the cochlea results in a mild-to-moderate high-frequency SNHL. Audiography testing reveals a mixed hearing loss when a middle ear effusion is present. Vestibular symptoms may occur but are less common. Treatment is aimed at eliminating the underlying infection and clearing the middle ear space of effusion. The hearing loss is usually transient but may persist if the otitis is left untreated. Autoimmune labyrinthitis is an uncommon cause of sensorineural hearing loss and may occur as a local, inner ear process or as part of a systemic autoimmune disease such as Wegener granulomatosis or polyarteritis nodosa. Hearing loss in autoimmune inner ear disease is typically bilateral and progressive over weeks to months and vestibular complaints may occur in up to 80% of patients with autoimmune inner ear disease. [8, 9] Currently, no accurate or reliable autoimmune test is commercially available and the diagnosis of autoimmune labyrinthitis rests on a positive clinical response to steroid therapy.
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Epidemiology Frequency United States Although definitive epidemiological data are lacking, viral labyrinthitis is the most common form of labyrinthitis observed in clinical practice. The prevalence of sudden SNHL is estimated at 1 case in 10,000 persons, with up to 40% of these patients complaining of vertigo or dysequilibrium.[11] One study reported that 37 of 240 patients presenting with positional vertigo had viral labyrinthitis.[12] Auditory and vestibular symptoms develop in approximately 25% of patients with herpes oticus, in addition to the facial paralysis and vesicular rash that characterize the disease. [3] Bacterial labyrinthitis is rare in the postantibiotic era, although bacterial meningitis remains a significant cause of hearing loss. Auditory symptoms, vestibular symptoms, or both may be present in as many as 20% of children with meningitis.[13] Mortality/Morbidity Deaths associated with labyrinthitis are not reported except in cases of meningitis or overwhelming sepsis. The morbidity of labyrinthitis, especially bacterial labyrinthitis, is significant. Bacterial labyrinthitis, regardless of etiology, accounts for one third of all cases of acquired hearing loss. In the pediatric population, the risk of hearing loss secondary to meningitis is estimated to be 10-20%. [14, 13] One study reported dizziness following pneumococcal meningitis in 23% of patients.[15] S treptococcus pneumoniae appears to be the causative agent, most likely associated with meningitis-associated hearing loss.
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Mnire disease may follow an episode of suppurative or serous labyrinthitis and is most likely due to fibrosis of the endolymphatic sac and altered Na+/K+ transport. Age Viral labyrinthitis is usually observed in adults aged 30-60 years and is rarely observed in children. Meningogenic suppurative labyrinthitis is usually observed in children younger than 2 years, which is the population most at risk for meningitis. Otogenic suppurative labyrinthitis can be observed in persons of any age in the presence of cholesteatoma or as a complication of untreated acute otitis media.[7] Serous labyrinthitis is more common in the pediatric age group, in which the vast majority of both acute and chronic otitis media cases are observed. History A thorough medical history, including symptoms, past medical history, and medications, is essential to diagnosing labyrinthitis as the cause of the patient's vertigo or hearing loss.

Symptoms Vertigo (timing and duration, association with movement, head position, and other characteristics) Hearing loss (unilateral or bilateral, mild or profound, duration, and other characteristics) Aural fullness Tinnitus Otorrhea Otalgia Nausea or vomiting Fever Facial weakness or asymmetry Neck pain/stiffness Upper respiratory tract infection symptoms (preceding or concurrent) Visual changes Past medical history Episodes of dizziness or hearing loss Infections Sick contacts Ear surgery Hypertension/hypotension Diabetes Stroke Migraine Trauma (head or cervical spine) Family history of hearing loss or ear disease Medications Aminoglycosides and other ototoxic medications Beta-blockers and other antihypertensives Tranquilizers, including benzodiazepines Antiepileptics Alcohol Illicit drugs Physical Include in the physical examination a complete head and neck examination with emphasis on the otologic, ocular, and cranial nerve portions of the examination. A brief neurologic examination is also necessary. Seek the presence of meningeal signs if meningitis is a consideration. Otologic examination Perform an external inspection for signs of mastoiditis, cellulitis, or prior ear surgery. Inspect the ear canal for otitis externa, otorrhea, or vesicles. Inspect the tympanic membrane and middle ear for the presence of perforation, cholesteatoma, middle ear effusion, or acute otitis media. Ocular examination Inspect the ocular range of motion and pupillary response. Perform a funduscopic examination to assess for papilledema. Observe for nystagmus (spontaneous, gaze-evoked, and positional). Perform a Dix-Hallpike test if the patient can tolerate it. If visual changes are suggested, consult an ophthalmologist. Neurologic examination Perform a complete cranial nerve examination. Assess for balance using the Romberg test and tandem gait. Assess cerebellar function by performing finger-to-nose and heel-to-shin tests. Causes Little direct evidence suggests a viral cause for labyrinthitis; however, a wealth of epidemiologic evidence implicates a number of viruses as potentially causing inflammation of the labyrinth. Viral labyrinthitis is often preceded by an upper respiratory tract infection and occurs in epidemics. The histologic finding of axonal degeneration in the vestibular nerve suggests a viral etiology for vestibular neuritis. [1] The bacteria that cause labyrinthitis are the same bacteria responsible for meningitis and otitis. Gram-negative organisms are found more commonly when cholesteatoma is the inciting factor. Potential viral causes Cytomegalovirus Mumps virus Varicella-zoster virus Rubeola virus Influenza virus Parainfluenza virus Rubella virus

Herpes simplex virus 1 Adenovirus Coxsackievirus Respiratory syncytial virus Potential bacterial causes S pneumoniae Haemophilus influenzae Moraxella catarrhalis N meningitidis Streptococcus species Staphylococcus species Proteus species Bacteroides species Escherichia coli Mycobacterium tuberculosis Laboratory Studies No specific laboratory studies are available for labyrinthitis. Routine serology testing often fails to reveal an infectious organism, and when results are positive, methods to determine if the same organism caused the damage to the membranous labyrinth are not available. Obtain appropriate tests to help exclude other possible etiologies in the differential diagnosis. Examine cerebrospinal fluid if meningitis is suggested. If a systemic infection is considered, a CBC count and blood cultures are indicated. Perform culture and sensitivity testing of middle ear effusions if present, and select appropriate antibiotic therapy accordingly. Imaging Studies CT scan Consider a CT scan prior to lumbar puncture in cases of possible meningitis. A CT scan is also useful to help rule out mastoiditis as a potential cause. A temporal bone CT scan may aid in the management of patients with cholesteatoma and labyrinthitis. A noncontrast CT scan is best for visualizing fibrosis and calcification of the membranous labyrinth in persons with chronic labyrinthitis or labyrinthitis ossificans. MRI MRI can be used to help rule out acoustic neuroma, stroke, brain abscess, or epidural hematoma as potential causes of vertigo and hearing loss. The cochlea, vestibule, and semicircular canals enhance on T1-weighted postcontrast images in persons with acute and subacute labyrinthitis.[17] This finding is highly specific and correlates with objective and subjective patient assessment. Recent improvements in MRI techniques may make this the study of choice for suspected labyrinthitis. Other Tests Audiography Obtain an audiogram in all patients who may have labyrinthitis. Evaluate critically ill and severely vertiginous patients when stable and able to tolerate the test. The audiogram may show different findings depending on the etiology of the labyrinthine inflammation. Persons with viral labyrinthitis have mild-to-moderate high-frequency SNHL in the affected ear, although any frequency spectrum may be affected. Suppurative (bacterial) labyrinthitis typically results in severe-to-profound unilateral hearing loss. In cases of meningitis, the loss is often bilateral. Persons with serous (bacterial) labyrinthitis have unilateral high-frequency hearing loss in the affected ear. A conductive loss in the same ear may occur secondary to effusion. Vestibular testing Caloric testing and an electronystagmogram may help in diagnosing difficult cases and establishing a prognosis for recovery. Recent evidence suggests that careful evaluation of the vestibuloocular reflexmay help establish the etiology of the labyrinthitis.[18] Persons with viral labyrinthitis have nystagmus with unilateral caloric vestibular paresis/hypofunction. Persons with suppurative (bacterial) labyrinthitis have nystagmus and an absent caloric response on the affected side. Persons with serous (bacterial) labyrinthitis usually have normal electronystagmogram results, but they may have a decreased caloric response in the affected ear. However, the presence of a middle ear effusion can attenuate the caloric response and cause a false-positive finding. Medical Care The initial treatment of viral labyrinthitis consists of bed rest and hydration. Most patients can be treated on an outpatient basis. However, they should be cautioned to seek further medical care for worsening symptoms, especially neurologic symptoms (eg, diplopia, slurred speech, gait disturbances, localized weakness or numbness). Patients with severe nausea and vomiting may benefit from intravenous fluid and antiemetic medications. Diazepam or other benzodiazepines are occasionally helpful as a vestibular suppressant. A short course of oral corticosteroids may be helpful. Currently, the role of antiviral therapy is not established.

Steroids (methylprednisolone) were found to be more effective than antiviral agents (valacyclovir) for recovery of peripheral vestibular function in patients with vestibular neuritis in a randomized controlled trial by Strupp et al. [19]This may also apply to the treatment of viral labyrinthitis. The antiviral drugs acyclovir, famciclovir, and valacyclovir shorten the duration of viral shedding in persons with herpes zoster oticus and may prevent some auditory and vestibular damage if started early in the clinical course. Administer corticosteroids to reduce inflammation and edema in the facial canal and labyrinth. For bacterial labyrinthitis, antibiotic treatment is selected based on culture and sensitivity results. Antibiotic treatment should consist of a broad-spectrum antibiotic or combination therapy with CNS penetration until culture results are available. Treat the vertigo symptomatically as indicated. The use of steroids in meningogenic hearing loss is controversial. Recent studies have shown that antioxidant therapy and cochlear microperfusion may be useful adjuvant treatments. [20, 21] Surgical Care In cases of labyrinthitis resulting from otitis media, perform a myringotomy and evacuate the effusion. A ventilation tube also may be indicated. Middle ear effusion should be sent for microscopic evaluation as well as culture and sensitivity. Mastoiditis and cholesteatoma are handled best with surgical drainage and debridement by way of a mastoidectomy. Medication Summary Medications may be indicated in persons with viral labyrinthitis to treat the symptoms of vertigo and nausea/vomiting. These medications include benzodiazepines and antiemetics and are typically used for a few days, until symptoms are relieved. Corticosteroids should, in theory, reduce labyrinthine inflammation and prevent the sequelae of labyrinthitis due to infectious or inflammatory causes. Definitive evidence is lacking, however, for the efficacy of corticosteroids in the treatment of labyrinthitis and sudden sensorineural hearing loss.[22]Intratympanic steroids may be more effective than systemic steroids in the treatment of sudden hearing loss, either alone or in combination with systemic steroids. [23, 24] Antiviral agents may play a role in the treatment of labyrinthitis due to presumed viral infections. However, recent studies have not shown improvement in treatment outcomes when antivirals are combined with systemic steroids in the treatment of labyrinthitis.[25] Antibiotic therapy for bacterial causes of labyrinthitis must be directed at the most likely causative organisms. A complete discussion of all the antibiotics available for the treatment of suppurative or toxic bacterial labyrinthitis is beyond the scope of this article. Benzodiazepines Class Summary These agents are used for the symptomatic treatment of vertigo. Diazepam (Valium) Depresses all levels of CNS (eg, limbic and reticular formation), possibly by increasing activity of GABA. Individualize dosage and increase cautiously to avoid adverse effects. Lorazepam (Ativan) By increasing action of GABA, which is a major inhibitory neurotransmitter in the brain, may depress all levels of CNS, including limbic and reticular formation. Antiemetics Class Summary These agents are used for relief of nausea and vomiting. Prochlorperazine (Compazine) May relieve nausea and vomiting by blocking postsynaptic mesolimbic dopamine receptors through anticholinergic effects and depressing reticular activating system. In addition to antiemetic effects, it has the advantage of augmenting hypoxic ventilatory response, acting as a respiratory stimulant at high altitude. Antiviral drugs Class Summary Nucleoside analogs are initially phosphorylated by viral thymidine kinase to eventually form a nucleoside triphosphate. These molecules inhibit HSV polymerase with 30-50 times the potency of human alpha-DNA polymerase. Famciclovir (Famvir) Prodrug that, when biotransformed into active metabolite (penciclovir), may inhibit viral DNA synthesis/replication.

Valacyclovir (Valtrex)

Prodrug rapidly converted to the active drug acyclovir. More expensive but has a more convenient dosing regimen than acyclovir. Acyclovir (Zovirax) Has affinity for viral thymidine kinase and, once phosphorylated, causes DNA chain termination when acted upon by DNA polymerase. Compliance problem; requires 5 daily doses. Corticosteroids Class Summary Have anti-inflammatory properties and cause profound and varied metabolic effects. Modify the body's immune response to diverse stimuli. Prednisone (Deltasone, Orasone, Meticorten) Standard agents administered in cases of sudden hearing loss and may play a role in the treatment of viral labyrinthitis. Their role in treatment of bacterial labyrinthitis and meningogenic hearing loss is controversial.