eMedicine Specialties > Neurology > Neuro-otology

Inner Ear, Labyrinthitis

Mark E Boston, MD, Chairman, Department of Otolaryngology-Head and Neck Surgery, Wilford Hall Medical Center, Lackland Air Force Base
Barry Strasnick, MD, FACS, Chairman, Professor, Department of Otolaryngology - Head and Neck Surgery, Eastern Virginia Medical School

Updated: Sep 9, 2009

Introduction

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 Media file 1.) 

Anatomy of the labyrinth.

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 Ménière 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 media and 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.¹⁰  

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.¹⁶  
• Ménière 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.

Clinical

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

Differential Diagnoses

Other Problems to Be Considered

Vertebrobasilar insufficiency
Presyncope dizziness
Cerebellar infarct
Dysequilibrium of aging
Drug-induced vertigo and/or hearing loss

Workup

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 reflex may 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.

Treatment

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.

Consultations

• Consult a neurosurgeon in the event of suppurative intracranial complications.
• Consultation with an infectious disease specialist may be warranted in the presence of systemic infection or unusual or atypical infections.

Medication

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

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.

Dosing

Adult

2-10 mg PO tid or 5-10 mg IV/IM in an acute setting

Pediatric

1-2.5 mg PO tid

Interactions

Increases toxicity of benzodiazepines in CNS with coadministration of phenothiazines, barbiturates, alcohols, and MAOIs

Contraindications

Documented hypersensitivity; narrow-angle glaucoma; history of addiction, alcohol intoxication, myasthenia gravis

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Caution with other CNS depressants, low albumin levels, or hepatic disease (may increase toxicity)

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.

Dosing

Adult

1-2 mg PO/IV/IM tid

Pediatric

Not established

Interactions

CNS toxicity of benzodiazepines increases when used concurrently with alcohol, phenothiazines, barbiturates, and MAOIs

Contraindications

Documented hypersensitivity; preexisting CNS depression, hypotension, and narrow-angle glaucoma

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Caution in renal or hepatic impairment, myasthenia gravis, organic brain syndrome, or Parkinson disease

Antiemetics

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.

Dosing

Adult

PO: 10 mg q6h
IV: 2.5-10 mg slow push q6h
PR: 25 mg q12h

Pediatric

<2 years: Not established
2-12 years: 2.5 mg PO bid/tid
>12 years: Administer as in adults

Interactions

Coadministration with other CNS depressants or anticonvulsants may cause additive effects; with epinephrine, may cause hypotension

Contraindications

Documented hypersensitivity; bone marrow suppression, narrow-angle glaucoma, and severe liver or cardiac disease

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Drug-induced Parkinson syndrome or pseudoparkinsonism occurs quite frequently; akathisia is most common extrapyramidal reaction in elderly persons; lowers seizure threshold; caution with history of seizures

Antiviral drugs

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.

Dosing

Adult

500 mg PO tid for 7 d

Pediatric

Not established

Interactions

Coadministration with probenecid or cimetidine may increase toxicity; coadministration increases bioavailability of digoxin

Contraindications

Documented hypersensitivity; impaired renal function

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Caution in renal failure or coadministration of nephrotoxic drugs

Valacyclovir (Valtrex)

Prodrug rapidly converted to the active drug acyclovir. More expensive but has a more convenient dosing regimen than acyclovir.

Dosing

Adult

1000 mg PO tid for 7 d

Pediatric

Not established

Interactions

Probenecid, zidovudine, or cimetidine coadministration prolongs half-life and increases CNS toxicity

Contraindications

Documented hypersensitivity; renal transplantation or renal failure

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Caution in renal failure and coadministration of nephrotoxic drugs; associated with onset of hemolytic uremic syndrome

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.

Dosing

Adult

800 mg PO 5 times/d for 7 d or 15 mg/kg IV divided tid for 5-10 d

Pediatric

Not established

Interactions

Concomitant use of probenecid or zidovudine prolongs half-life and increases CNS toxicity

Contraindications

Documented hypersensitivity; renal failure or impairment

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Caution in renal failure or when using nephrotoxic drugs

Corticosteroids

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.

Dosing

Adult

40-60 mg PO qd for 5 d, then taper over 5 d

Pediatric

Not established

Interactions

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur

Follow-up

Further Inpatient Care

• Most patients with labyrinthitis can be evaluated and treated in the emergency department and then discharged.
• Some patients with intractable vertigo and vomiting may require admission.
• For those with a possible severe underlying condition (eg, vertebrobasilar ischemia, brainstem tumor), admission to the hospital may be appropriate under the direction of a neurologist, a neurosurgeon, or both.

Further Outpatient Care

Patients with persistent vestibular symptoms may be candidates for vestibular rehabilitation. For many patients with chronic vertigo due to a peripheral vestibular etiology, a simple home program of vestibular habituation head movement exercises reduces symptoms of imbalance during stance and gait.²⁶

A follow-up audiogram should be performed in all patients with hearing loss and in patients who were not tested at presentation. An auditory brainstem response test is indicated for younger children.

Prognosis

The acute symptoms of vertigo and nausea and vomiting resolve after several days to weeks in all forms of labyrinthitis; however, hearing loss is more variable.

• Suppurative labyrinthitis nearly always results in permanent and profound hearing loss, whereas hearing loss associated with viral labyrinthitis may recover. Dysequilibrium and/or positional vertigo also may be present long-term following resolution of the acute infection.
• Permanent hearing loss occurs in 10-20% of children with meningitis.^14,13
• Permanent SNHL occurs in approximately 6% of patients with herpes zoster oticus who present with hearing loss.⁵

Patient Education

For excellent patient education resources, visit eMedicine's Ear, Nose, and Throat Center. Also, see eMedicine's patient education articles Labyrinthitis and Vertigo.

Miscellaneous

Medicolegal Pitfalls

• Failure to diagnose a potentially life-threatening condition, such as meningitis, cerebrovascular ischemia, or a brainstem tumor. A 2009 case report suggests that an early anterior inferior cerebellar artery infarction should be considered in patients presenting with acute hearing loss and vertigo.²⁷
• Failure to counsel patients regarding the potential for injury to themselves or others if they operate heavy machinery or drive a vehicle while vertiginous or while taking certain medications to control symptoms

Special Concerns

• Noninfectious labyrinthitis is very rare in children; therefore, seek an alternative diagnosis in patients this age. Labyrinthitis resulting from otitis media or meningitis is not uncommon in children.
• Avoid scopolamine (or use with extreme caution) in elderly patients.

Multimedia

Media file 1: Anatomy of the labyrinth.

References

1. Schuknecht HF, Kitamura K. Second Louis H. Clerf Lecture. Vestibular neuritis. Ann Otol Rhinol Laryngol Suppl. Jan-Feb 1981;90(1 Pt 2):1-19. [Medline].

2. Kuhweide R, Van de Steene V, Vlaminck S, Casselman JW. Ramsay Hunt syndrome: pathophysiology of cochleovestibular symptoms. J Laryngol Otol. Oct 2002;116(10):844-8. [Medline].

3. Hato N, Kisaki H, Honda N, Gyo K, Murakami S, Yanagihara N. Ramsay Hunt syndrome in children. Ann Neurol. Aug 2000;48(2):254-6. [Medline].

4. Schraff SA, Schleiss MR, Brown DK, Meinzen-Derr J, Choi KY, Greinwald JH, et al. Macrophage inflammatory proteins in cytomegalovirus-related inner ear injury. Otolaryngol Head Neck Surg. Oct 2007;137(4):612-8. [Medline].

5. Gulya AJ. Infections of the labyrinth. In: Bailey BJ, Johnson JT, Pillsbury HC, Tardy ME, Kohut RI, eds. Head and Neck Surgery-Otolaryngology. Vol 2. Philadelphia, Pa: JB Lippincott; 1993:1769-81.

6. Berlow SJ, Caldarelli DD, Matz GJ, Meyer DH, Harsch GG. Bacterial meningitis and sensorineural hearing loss: a prospective investigation. Laryngoscope. Sep 1980;90(9):1445-52. [Medline].

7. Jang CH, Park SY, Wang PC. A case of tympanogenic labyrinthitis complicated by acute otitis media. Yonsei Med J. Feb 28 2005;46(1):161-5. [Medline].

8. Harris JP, Ryan AF. Fundamental immune mechanisms of the brain and inner ear. Otolaryngol Head Neck Surg. Jun 1995;112(6):639-53. [Medline].

9. Broughton SS, Meyerhoff WE, Cohen SB. Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum. Oct 2004;34(2):544-8. [Medline].

10. Bovo R, Ciorba A, Martini A. The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls. Eur Arch Otorhinolaryngol. Jan 2009;266(1):37-40. [Medline].

11. Byl FM. Seventy-six cases of presumed sudden hearing loss occurring in 1973: prognosis and incidence. Laryngoscope. May 1977;87(5 Pt 1):817-25. [Medline].

12. Baloh RW, Honrubia V, Jacobson K. Benign positional vertigo: clinical and oculographic features in 240 cases. Neurology. Mar 1987;37(3):371-8. [Medline].

13. Nadol JB Jr. Hearing loss as a sequela of meningitis. Laryngoscope. May 1978;88(5):739-55. [Medline].

14. Woolley AL, Kirk KA, Neumann AM Jr, McWilliams SM, Murray J, Freind D. Risk factors for hearing loss from meningitis in children: the Children's Hospital experience. Arch Otolaryngol Head Neck Surg. May 1999;125(5):509-14. [Medline].

15. Bohr V, Paulson OB, Rasmussen N. Pneumococcal meningitis. Late neurologic sequelae and features of prognostic impact. Arch Neurol. Oct 1984;41(10):1045-9. [Medline].

16. Kutz JW, Simon LM, Chennupati SK, Giannoni CM, Manolidis S. Clinical predictors for hearing loss in children with bacterial meningitis. Arch Otolaryngol Head Neck Surg. Sep 2006;132(9):941-5. [Medline].

17. Mark AS, Seltzer S, Nelson-Drake J, Chapman JC, Fitzgerald DC, Gulya AJ. Labyrinthine enhancement on gadolinium-enhanced magnetic resonance imaging in sudden deafness and vertigo: correlation with audiologic and electronystagmographic studies. Ann Otol Rhinol Laryngol. Jun 1992;101(6):459-64. [Medline].

18. Maire R, van Melle G. Diagnostic value of vestibulo-ocular reflex parameters in the detection and characterization of labyrinthine lesions. Otol Neurotol. Jun 2006;27(4):535-41. [Medline].

19. Strupp M, Zingler VC, Arbusow V, Niklas D, Maag KP, Dieterich M, et al. Methylprednisolone, valacyclovir, or the combination for vestibular neuritis. N Engl J Med. Jul 22 2004;351(4):354-61. [Medline].

20. Barkdull GC, Vu C, Keithley EM, Harris JP. Cochlear microperfusion: experimental evaluation of a potential new therapy for severe hearing loss caused by inflammation. Otol Neurotol. Jan 2005;26(1):19-26. [Medline].

21. Klein M, Koedel U, Pfister HW, Kastenbauer S. Meningitis-associated hearing loss: protection by adjunctive antioxidant therapy. Ann Neurol. Oct 2003;54(4):451-8. [Medline].

22. Wei BP, Mubiru S, O'Leary S. Steroids for idiopathic sudden sensorineural hearing loss. Cochrane Database Syst Rev. Jan 25 2006;CD003998. [Medline].

23. Battaglia A, Burchette R, Cueva R. Combination therapy (intratympanic dexamethasone + high-dose prednisone taper) for the treatment of idiopathic sudden sensorineural hearing loss. Otol Neurotol. Jun 2008;29(4):453-60. [Medline].

24. Plontke SK, Löwenheim H, Mertens J, Engel C, Meisner C, Weidner A, et al. Randomized, double blind, placebo controlled trial on the safety and efficacy of continuous intratympanic dexamethasone delivered via a round window catheter for severe to profound sudden idiopathic sensorineural hearing loss after failure of systemic therapy. Laryngoscope. Feb 2009;119(2):359-69. [Medline].

25. Westerlaken BO, Stokroos RJ, Dhooge IJ, Wit HP, Albers FW. Treatment of idiopathic sudden sensorineural hearing loss with antiviral therapy: a prospective, randomized, double-blind clinical trial. Ann Otol Rhinol Laryngol. Nov 2003;112(11):993-1000. [Medline].

26. Cohen HS, Kimball KT. Decreased ataxia and improved balance after vestibular rehabilitation. Otolaryngol Head Neck Surg. Apr 2004;130(4):418-25. [Medline].

27. Lee H, Kim HJ, Koo JW, Kim JS. Progression of acute cochleovestibulopathy into anterior inferior cerebellar artery infarction. J Neurol Sci. Mar 15 2009;278(1-2):119-22. [Medline].

Keywords

labyrinthitis of the inner ear, labyrinthitis, viral labyrinthitis, serous labyrinthitis, bacterial labyrinthitis, suppurative labyrinthitis, sudden sensorineural hearing loss, neurolabyrinthitis, vestibulocochleitis, vestibulocochlearis, sudden hearing loss, ear infection, inner ear infection, ear labyrinth infection, hearing disorder, hearing disturbance, balance disorder, balance disturbance, vertigo, dysequilibrium, hearing loss, vestibular neuritis, herpes zoster oticus, Ramsay-Hunt syndrome, varicella-zoster virus, varicella reactivation, zoster reactivation, rubella, cytomegalovirus, CMV, mumps, measles, SNHL, herpes oticus, labyrinthine inflammation, labyrinthine disease, labyrinthine disorder, labyrinthine infection

Contributor Information and Disclosures

Author

Mark E Boston, MD, Chairman, Department of Otolaryngology-Head and Neck Surgery, Wilford Hall Medical Center, Lackland Air Force Base
Mark E Boston, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Barry Strasnick, MD, FACS, Chairman, Professor, Department of Otolaryngology - Head and Neck Surgery, Eastern Virginia Medical School
Barry Strasnick, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American College of Surgeons, American Medical Association, American Tinnitus Association, Ear Foundation Alumni Society, Norfolk Academy of Medicine, North American Skull Base Society, Society of University Otolaryngologists-Head and Neck Surgeons, Vestibular Disorders Association, and Virginia Society of Otolaryngology-Head and Neck Surgery
Disclosure: Nothing to disclose.

Medical Editor

Michael E Hoffer, MD, Director, Spatial Orientation Center, Department of Otolaryngology, Naval Medical Center of San Diego
Michael E Hoffer, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery
Disclosure: American biloogical group Royalty Other

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Gerard J Gianoli, MD, Clinical Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Tulane University School of Medicine; Vice President, The Ear and Balance Institute; Chief Executive Officer, Ponchartrain Surgery Center
Gerard J Gianoli, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Robert A Egan, MD, Director of Neuro-Ophthalmology, St Helena Hospital
Robert A Egan, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, North American Neuro-Ophthalmology Society, and Oregon Medical Association
Disclosure: Nothing to disclose.

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)