Labyrinthitis

Mary S. Lindholm, MD Reviewed 06/2017
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Subject: Labyrinthitis

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BASICS

DESCRIPTION

  • The sudden and persistent onset of vertigo, often accompanied by hearing loss, caused by acute inflammation or infection of the labyrinth

  • Labyrinthitis is a clinical diagnosis in absence of neurologic deficits.

  • Typically presents with false sense of motion or room-spinning vertigo lasting for hours or days and often sudden unilateral hearing loss

  • Often associated with vestibular hypofunction of the involved ear. Peripheral vertigo improves over time with central compensation.

  • System(s) affected: nervous, special sensory (auditory and vestibular)

  • Synonym(s): acute peripheral vestibulopathy; vestibular neuronitis (vertigo/dizziness only); vestibular neuritis (vertigo/dizziness only)

ALERT
  • “Vertigo” and “dizziness” are commonly used terms. Clarify symptoms by giving options of alternative descriptions such as light-headedness, disequilibrium, room-spinning vertigo, or imbalance.

  • Hearing loss and duration of symptoms can help narrow the differential diagnosis in patients with vertigo.

  • Benign paroxysmal positional vertigo (BPPV) is the most common cause of vertigo. Unlike labyrinthitis, BPPV is episodic, with severe symptoms lasting ≪1 minute. BPPV is diagnosed using the Dix-Hallpike maneuver. Unlike labyrinthitis, it is not associated with hearing loss.

  • Ménière disease presents with the classic triad of episodic vertigo, tinnitus, and sensorineural hearing loss that is often fluctuant 1[C].

 

Geriatric Considerations

  • Elderly are less likely to compensate fully and may report symptoms of disequilibrium lasting weeks to months after resolution of the acute vertigo.

  • Avoid excessive use of scopolamine, meclizine, and other vestibular suppressants following the initial event, as this will delay central compensation.

  • Benzodiazepines are preferred vestibular suppressant treatment but do increase the risk of falls in older persons.

Pediatric Considerations

Less common in children, incidence of vestibular vertigo in 10-year-olds estimated to be 5.7% 2[C]. 

EPIDEMIOLOGY

  • 10% of all patients seen for dizziness

  • Most common in 30 to 50 years of age 3

  • Predominant sex: female = male

Incidence

  • Viral labyrinthitis is the most common etiology.

  • Suppurative or serous labyrinthitis secondary to otitis media is increasingly rare.

Prevalence

In the United States, second most common cause of dizziness due to persistent peripheral vestibular hypofunction (9%); benign positional vertigo (40%) is most common. More than 1/3 of adults see a health care provider for vertigo in their lifetimes 4

ETIOLOGY AND PATHOPHYSIOLOGY

  • Acute inflammation or damage to the inner ear, involving both branches of the vestibulocochlear nerve

  • Viruses pass via hematogenous spread into the labyrinth or directly from the middle ear to labyrinth via the round/oval window.

  • Bacterial toxins and host inflammatory mediators from a middle ear infection may reach the inner ear.

  • Ischemia: Ischemic or thromboembolic events involving the labyrinthine artery can cause symptoms that mimic acute labyrinthitis; often presents with associated neurologic symptoms

  • Autoimmune: Local or systemic inflammatory processes may affect the inner ear via autoantibodies vasculitis of the labyrinthine artery.

    • ▪ Wegener granulomatosis, Cogan syndrome, systemic lupus erythematosus, polyarteritis nodosa, Behçet disease
  • Infections

    • ▪ Common viral: cytomegalovirus, mumps, varicella zoster, rubeola, influenza, parainfluenza, herpes simplex, adenovirus, coxsackievirus, respiratory syncytial virus, HIV
    • ▪ Common bacterial: Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, Neisseria meningitidis, Streptococcus spp., Staphylococcus spp., Borrelia burgdorferi
    • ▪ Treponemal: Treponema pallidum
  • Ototoxic drugs (e.g., aspirin, aminoglycosides, loop diuretics, cisplatin)

Genetics

No known genetic link 

RISK FACTORS

  • Viral upper respiratory infection

  • Otitis media

  • Vestibulotoxic/ototoxic medications

  • Head trauma

  • History of allergies

  • Meningitis

  • Cerebrovascular disease

  • Other risk factors include autoimmune disease, herpes zoster infection, excessive alcohol consumption, and smoking.

GENERAL PREVENTION

  • Scheduled immunizations (to prevent common viral pathogens)

  • Prevent maternal transmission of pathogens, including syphilis and HIV.

COMMONLY ASSOCIATED CONDITIONS

  • Viral upper respiratory infection

  • Allergies

  • Otitis media

  • Cholesteatoma

  • Head injury

DIAGNOSIS

HISTORY

  • Vertigo AND (often) hearing loss in one ear

  • Vertigo is acute in onset and lasts days to weeks.

  • Nausea and vomiting are common.

  • Fullness of affected ear

  • Tinnitus of affected ear (roaring, ringing)

  • Upper respiratory tract infection symptoms

  • Otorrhea or otalgia (not common with viral causes)

  • Severe headache, fever, and nuchal rigidity in the setting of meningitis

  • Recurrent symptoms should raise suspicion for autoimmune causes.

  • Profound imbalance or associated focal neurologic signs are not typical and should prompt imaging.

PHYSICAL EXAM

  • Nystagmus

    • ▪ Fast-beating nystagmus toward affected ear (acutely)
    • ▪ Fast-beating nystagmus away from affected ear (chronically)
  • Symptoms abate in supine position and with eyes closed or with visual fixation.

  • Otologic exam may be unremarkable in the setting of viral labyrinthitis.

  • Serous/purulent effusion may be present in the middle ear.

  • Retraction of the tympanic membrane and keratinaceous debris may be present with cholesteatoma.

DIFFERENTIAL DIAGNOSIS

  • Vestibular neuritis/neuronitis (vertigo without hearing loss)

  • BPPV: episodic, vertigo lasting seconds/minutes, worse when lying down or looking up

  • Ménière disease: episodic vertigo lasting minutes to hours, associated with the triad of episodic vertigo, tinnitus, and hearing loss

  • Vestibular migraine

  • Autoimmune inner ear disease

  • Postconcussive syndrome

  • Acute otitis media

  • Ototoxicity

  • Cardiovascular accident (CVA)/brainstem infarct

  • Cerebellopontine-angle tumors (e.g., vestibular schwannoma)

  • Less common etiologies: parainfectious encephalomyelitis or cranial polyneuritis, Ramsay Hunt syndrome, HIV infection, syphilis, temporal lobe epilepsy, perilymphatic fistula, superior canal dehiscence, idiopathic sudden single-sided deafness, multiple sclerosis, vasculitis (cerebral or systemic)

DIAGNOSTIC TESTS & INTERPRETATION

  • Routine lab studies are not helpful in making the diagnosis unless an autoimmune cause is highly suspected.

  • Consider culture of otorrhea or middle ear fluid to direct antibiotic choice.

  • Consider lumbar puncture only if meningitis is suspected.

  • Consider screening for syphilis or HIV when clinically indicated by risk factors or clinical history.

  • Imaging is not required for the diagnosis of acute labyrinthitis.

  • If associated neurologic symptoms or sensorineural hearing loss are present, an MRI and MR angiography of brain and brainstem are recommended.

  • Vertigo usually spontaneously resolves, and there is a low risk of developing Ménière disease or migraines.

Follow-Up Tests & Special Considerations

Labyrinthitis ossificans is fibrosis of the internal auditory canal following bacterial meningitis and is thought to occur due to a suppurative labyrinthitis. This can occur rapidly, especially after S. pneumoniae meningitis. 

Diagnostic Procedures/Other

  • Audiogram should be obtained.

  • Vestibular tests are not typically indicated in the acute setting. If vertigo and dizziness persist after expected resolution of symptoms, electronystagmography should be used.

Test Interpretation

  • Audiogram may show varying degrees of both hearing loss and discrimination.

  • Caloric testing may show relative weakness of the horizontal semicircular canal of the affected side. Sensitivity and specificity of this test are variable within literature.

TREATMENT

  • Symptom management and reassurance

  • Vestibular rehabilitation is the mainstay of treatment and has been shown to be safe and effective management for unilateral peripheral vestibular dysfunction 5[A].

  • Patients should begin exercises as soon as the acute phase resolves and movement is tolerable, generally within 2 to 3 days of onset.

  • Vestibular suppressants as needed (see “Medication”) for severe acute attacks of vertigo only. Patients should be advised NOT to use these medications as scheduled medications or for prophylaxis without symptoms.

  • Sudden single-sided hearing loss (onset within 2 weeks) should be managed with high-dose oral steroids as soon as possible.

  • Auricular acupuncture and Ginkgo biloba may be emerging adjunctive therapies to reduce vertiginous symptoms, although research is limited.

  • For suppurative labyrinthitis, appropriate antibiotics to eradicate infection

GENERAL MEASURES

Vestibular exercises for prolonged symptoms and unilateral vestibular loss have been shown to alleviate postural control. 

MEDICATION

Use of the following drugs should be on a PRN basis. Benzodiazepines can also assist with the anxiety associated with vertigo. No patient should take vestibular suppressants as a chronic medication, as they can block central compensation. 
  • Vestibular suppressants

    • Lorazepam (Ativan): 0.5 to 2 mg sublingual (SL)/PO BID PRN or diazepam (Valium) 2 to 5 mg QID PO PRN
    • Meclizine (Antivert, Bonine, Zentrip [dissolvable]) 12.5 to 25 mg PO BID-TID PRN
    • Dimenhydrinate (Dramamine) 25 to 50 mg PO q4-6h PRN
  • Antiemetics

  • Antivirals

    • Acyclovir 800 mg PO 5 times per day for 7 days can be used in cases associated with herpes.
  • Steroids

    • ▪ Adults: methylprednisolone initially 100 mg PO daily and then tapered to 10 mg PO daily over 3 weeks
    • ▪ Pediatrics: prednisone 1 mg/kg PO daily 3 times per week and then taper over 3 weeks
    • ▪ Given early in the setting of bacterial meningitis, may decrease the otologic sequelae, specifically labyrinthitis ossificans
    • ▪ Used in treatment of labyrinthitis for associated sudden sensorineural hearing loss

Pregnancy Considerations

First Line

  • Benzodiazepines, which are better vestibular suppressants, are preferred over antihistamine/anticholinergics such as meclizine. Sublingual benzodiazepines are very effective for vertigo and should be considered first-line therapy.

  • Urgent steroid treatment in acute setting

ISSUES FOR REFERRAL

  • Consider neuro-otology referral for other peripheral causes of vertigo or unremitting vertigo.

  • Consider neurology referral for suspected central causes of vertigo or dizziness.

  • Consider otolaryngology referral for progressive bilateral hearing loss and vertigo after preliminary laboratory workup excluding rheumatologic causes.

ADMISSION, INPATIENT, AND NURSING CONSIDERATIONS

  • Patients with systemic infection, young age, or intractable vertigo with nausea and vomiting may need to be hospitalized for intravenous fluids and medications.

  • Usually outpatient management

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Follow hearing loss weekly with audiograms until hearing stabilizes. Acute vertiginous symptoms may last up to 6 weeks. Residual symptoms have been documented to last months or years. 

DIET

Avoid alcohol, as this may exacerbate symptoms. 

PATIENT EDUCATION

Lie still with eyes closed in a darkened room during acute attacks. Otherwise, encourage activity as tolerated. Minimize rapid head movement until symptoms resolve. 

PROGNOSIS

Prognosis depends on cause of labyrinthitis. 

COMPLICATIONS

Permanent hearing loss, more common with bacterial causes, and chronic impairment of balance 

REFERENCES

Post  RE, Dickerson  LM. Dizziness: a diagnostic approach. Am Fam Physician.  2010;82(4):361–368.  [View Abstract]
Jahn  K, Langhagen  T, Schroeder  AS, et al. Vertigo and dizziness in childhood—update on diagnosis and treatment. Neuropediatrics.  2011;42(4):129–134.  [View Abstract]
Neuhauser  HK, Lempert  T. Vertigo: epidemiologic aspects. Semin Neurol.  2009;29(5):473–481.  [View Abstract]
Wipperman  J. Dizziness and vertigo. Prim Care.  2014;41(1):115–131.  [View Abstract]
Hillier  SL, McDonnell  M. Vestibular rehabilitation for unilateral peripheral vestibular dysfunction. Cochrane Database Syst Rev.  2011;(2):CD005397.  [View Abstract]

ADDITIONAL READING

  • Lee HK, Ahn SK, Jeon SY, et al. Clinical characteristics and natural course of recurrent vestibulopathy: a long-term follow-up study. Laryngoscope.  2012;122(4):883–886.

  • Romoli M, Allais G, Airola G, et al. Ear acupuncture and fMRI: a pilot study for assessing the specificity of auricular points. Neurol Sci.  2014;35(Suppl 1):189–193.

  • Sokolova L, Hoerr R, Mishchenko T. Treatment of vertigo: a randomized, double-blind trial comparing efficacy and safety of Ginkgo biloba extract EGb 761 and betahistine. Int J Otolaryngol.  2014;2014:682439.

SEE ALSO

Ménière Disease; Postconcussion Syndrome (Mild Traumatic Brain Injury); Tinnitus 

CODES

ICD10

  • H83.09 Labyrinthitis, unspecified ear

  • H83.01 Labyrinthitis, right ear

  • H83.02 Labyrinthitis, left ear

  • H83.03 Labyrinthitis, bilateral

ICD9

  • 386.30 Labyrinthitis, unspecified

  • 386.31 Serous labyrinthitis

  • 386.33 Suppurative labyrinthitis

  • 386.35 Viral labyrinthitis

  • 386.34 Toxic labyrinthitis

  • 386.32 Circumscribed labyrinthitis

SNOMED

  • 23919004 Labyrinthitis (disorder)

  • 41674001 Serous labyrinthitis

  • 24817009 Suppurative labyrinthitis

  • 409711008 Viral labyrinthitis

  • 61794006 Circumscribed labyrinthitis

  • 3344003 Toxic labyrinthitis

CLINICAL PEARLS

  • Ask patients to describe symptoms in their own words; alternative symptoms include lightheadedness, vertigo, disequilibrium, or imbalance.

  • Benzodiazepines are better vestibular suppressants and are preferred over antihistamine/anticholinergics such as meclizine.

  • Episodic vertigo tends to be caused by BPPV or Ménière disease, whereas persistent vertigo is more consistent with labyrinthitis.

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