Labyrinthitis

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"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.
Meni ¨re 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 " 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, 2nd most common cause of dizziness due to persistent peripheral vestibular hypofunction (9%); benign positional vertigo (40%) is most common. More than ’ ; 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 erythematous, polyarteritis nodosa, Beh §et 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
Meni ¨re 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 Meni ¨re 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 (6)[B],(7)[C].
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 " 50 mg PO q4 " 6h PRN
Antiemetics
- Ondansetron (Zofran) 4 to 8 mg PO TID PRN or granisetron (Kytril) 1 mg PO TID PRN
- Meclizine (Antivert, Bonine) 12.5 to 25 mg PO q4h PRN
- Promethazine (Phenergan) 12.5 to 25 mg PO/PR QID PRN or prochlorperazine (Compazine) 25 mg PR BID PRN
- Metoclopramide (Reglan) 10 mg PO TID PRN
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, then tapered to 10 mg PO daily over 3 weeks
- Pediatrics: prednisone 1 mg/kg PO daily 3 times per week, 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

Dimenhydrinate, diphenhydramine, ondansetron, granisetron, and metoclopramide are pregnancy Category B.

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.

INPATIENT CONSIDERATIONS

Admission Criteria/Initial Stabilization

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 year (8)[C].

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

11 Post RE, Dickerson LM. Dizziness: a diagnostic approach. Am Fam Physician. 2010;82(4):361 " 368.22 Jahn K, Langhagen T, Schroeder AS, et al. Vertigo and dizziness in childhood " update on diagnosis and treatment. Neuropediatrics. 2011;42(4):129 " 134.33 Neuhauser HK, Lempert T. Vertigo: epidemiologic aspects. Semin Neurol. 2009;29(5):473 " 481.44 Wipperman J. Dizziness and vertigo. Prim Care. 2014;41(1):115 " 131.55 Hillier SL, McDonnell M. Vestibular rehabilitation for unilateral peripheral vestibular dysfunction. Cochrane Database Syst Rev. 2011;(2):CD005397.66 Sokolova L, Hoerr R, Mischenko 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;2014:682439.77 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 " 193.88 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.

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 light-headedness, vertigo, disequilibrium, or imbalance.
Benzodiazepines are better vestibular suppressant and are preferred over antihistamine/anticholinergics such as meclizine.
Episodic vertigo tends to be caused by BPPV or Meni ¨re disease, whereas persistent vertigo is more consistent with labyrinthitis.

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