Labyrinthitis

oPatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.

Labyrinthitis and vestibular neuritis are sometimes used interchangeably. However, experts in the field now recommend that vestibular neuritis be confined to cases in which the vestibular nerve only is involved, whereas labyrinthitis should be used in cases in which the vestibular nerve and the labyrinth are affected.

Labyrinthitis is one of many causes of vertigo. Typically it produces disturbances of balance and hearing to varying degrees and may affect one or both ears.

  • The labyrinth incorporates the peripheral sensory organs for balance and hearing, in a delicate membranous network (incorporating the utricle, saccule, semicircular canals and cochlea).
  • Symptoms of labyrinthitis occur when there is inflammation of the membranous labyrinth and when there is damage to the vestibular and auditory end organs.
  • It is possible for a variety of organisms and inflammatory mediators through both local and systemic disease to produce labyrinthitis. Bacteria or viruses can cause labyrinthitis but are sufficiently distinct to be considered as separate disease processes:
    • Bacteria may gain access to the membranous labyrinth through anatomical connections:
      • between the central nervous system and subarachnoid space via the internal auditory canal and cochlear aqueduct; or
      • through congenital or acquired defects of the bony labyrinth.
    • Viruses typically spread to labyrinthine structures haematogenously or via the anatomical connections above.
  • Many cases of labyrinthitis appear to be viral in origin or associated with systemic disease. Many cases of benign positional vertigo formerly thought to be idiopathic are known to be associated with osteoporosis or osteopenia.[2] Many factors can be associated with cochlear trauma, including vertebrobasilar ischaemia, meningitis, Ménière’s disease and medication (eg, aminoglycoside).[3][4] 

An upper respiratory tract infection precedes the onset of symptoms in about half of cases of viral labyrinthitis. Viral labyrinthitis is often confused with vestibular neuritis. It is generally accepted that vestibular neuritis is a disorder of the vestibular nerve and is not associated with hearing loss. The cochlea is affected in labyrinthine inflammation and therefore hearing loss is always present in people with viral labyrinthitis.

Herpes zoster oticus, or Ramsay Hunt syndrome, is a unique variety of viral labyrinthitis. It is caused by reactivation of varicella-zoster viral infection years after the primary infection.

It is worth noting that viral infections cause both congenital and acquired hearing loss (rubella and cytomegalovirus are viral causes of prenatal hearing loss). Postnatally, viral-induced hearing loss is usually due to mumps or measles. Viral infections are also implicated in idiopathic sudden sensorineural hearing loss (SNHL).

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  • The epidemiology of diseases causing vertigo has been described as underdeveloped.[5] 
  • Viral labyrinthitis is the most common form of labyrinthitis. It is usually observed in adults aged 30-60 years and is rarely observed in children. It is most common in the fourth decade with women outnumbering men by about 2:1.
  • Bacterial labyrinthitis is rare in the post-antibiotic era. Meningogenic suppurative labyrinthitis is most often seen in young children (under the age of 2 years), when children are at most risk of meningitis. Otogenic suppurative labyrinthitis can be observed in all ages (with cholesteatoma and in untreated acute otitis media).

History

This should be thorough and particularly include details of symptoms, past medical history and current medication.

The history in labyrinthitis[1][6] 
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).
  • Tinnitus.
  • Otorrhoea.
  • Otalgia.
  • Nausea or vomiting.
  • Fever.
  • Facial weakness or asymmetry.
  • Neck pain/stiffness.
  • Upper respiratory tract infection symptoms (preceding or concurrent).
  • Visual changes.
Past medical conditions:
  • 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.
All current medication including illicit drugs. Ask particularly about:
  • Aminoglycosides and other ototoxic medications.
  • Beta-blockers and other antihypertensives.
  • Tranquilisers, including benzodiazepines.
  • Anti-epileptics.

Viral labyrinthitis is characterised by:

  • Sudden unilateral loss of vestibular function and hearing (can be with tinnitus).
  • Sudden, severe and often incapacitating vertigo.
  • Nausea and vomiting - these are often associated with it.
  • Hearing loss, which may be the primary presenting symptom in many patients. Some degree of hearing loss is always present with viral labyrinthitis.[7] People with labyrinthitis do not usually have the sense of fullness in the ear that people with Ménière's disease have.[8]
  • The patient often being confined to bed while the symptoms abate. This takes several days to weeks in the case of vertigo but unsteadiness and positional vertigo may last for months.
  • Usually being a self-limiting condition but some cases can be chronic or recurrent.

It is very important to ask about hearing loss, as it does not occur with benign paroxysmal vertigo or vestibular neuronitis but it does occur with infectious labyrinthitis and Ménière's disease. It is also frequently found in ototoxicity and autoimmune labyrinthitis. Autoimmune disease seems to cause sensorineural hearing loss rather than labyrinthitis.[9] 

Examination[10]

  • Perform a coarse examination of hearing using a 256 Hz (middle C) tuning fork or 512 Hz (top C). Hearing tests do not have to be prolonged or intricate for this purpose.
  • Inspect the ear and tympanic membrane using an auriscope.
  • Note any marked loss of hearing or asymmetry. Weber's test involves placing a vibrating tuning fork on the forehead and asking if it is heard louder in either ear. There should be no difference but, in nerve deafness, it is quieter in the affected ear whilst, in conductive deafness, the sound transmitted through the skull is louder in the affected ear.
  • Perform a neurological examination with particular reference to the cranial nerves. If any cranial nerves except the VIII are abnormal, this is of great significance.
  • If it was not possible to demonstrate the vertigo, the Hallpike manoeuvre may be employed.
The Hallpike manoeuvre and interpretation

The patient sits on the couch with the head end flat. They should keep both eyes open all the time and should lie back from sitting to supine three times:

  • The first time, the head faces forwards with the neck slightly extended.The second time, the head is turned 45° to one side and the third time, to the other side.
  • Each time check for signs of nystagmus and ask about feelings of vertigo.
  • If the results are clear this is helpful but often they are inconclusive.
It is important to distinguish between peripheral and central dysfunction, as the implications are often much more serious for the latter than for the former:
  • Peripheral vestibular dysfunction has a short latency between the change in position and the vertigo, nausea and malaise. There are just several seconds between the change in position and the onset of nystagmus. Repetitive testing produces a reduced response.
  • Central vestibular lesions produce immediate onset of nystagmus without any latency and minimal symptoms of vertigo, nausea, or malaise. The nystagmus does not fatigue or habituate with repetitive testing.
  • Routine blood tests are not helpful; neither are viral antibody tests. However, if a systemic infection is suspected, FBC and blood cultures are indicated.
  • Examine cerebrospinal fluid if meningitis is suggested.
  • Perform culture and sensitivity testing of middle ear effusions if present (and select appropriate antibiotic therapy).
  • Most patients do not require imaging. However:
    • Consider a CT scan prior to lumbar puncture in cases of possible meningitis.
    • A CT scan can help rule out mastoiditis.
    • A temporal bone CT scan may help in patients with cholesteatoma and labyrinthitis.
    • If a sinister cause is suspected, MRI scan can be helpful. Compared with CT, it provides much better pictures of the posterior fossa and VIII nerve course.
    • MRI scanning can be used to help rule out acoustic neuroma, stroke, brain abscess, or epidural haematoma as potential causes of vertigo and hearing loss.
  • Obtain an audiogram in all patients who may have labyrinthitis. This is important with unilateral hearing loss or asymmetric hearing loss.
  • Vestibular testing:
    • Caloric testing and an electronystagmogram may help in diagnosing difficult cases and in determining the prognosis for recovery.
    • Patients with viral labyrinthitis have nystagmus and unilateral caloric vestibular paresis/hypofunction.
    • Vestibular dysfunction represents underactivity in labyrinthitis. New tests involving vestibular-evoked myalgic potentials have been developed to assess vestibular activity.[12] 
  • Ramsay Hunt syndrome is a particular variety of viral labyrinthitis:
    • Initial symptoms are deep, burning, auricular pain.
    • The eruption of a vesicular rash in the external auditory canal and concha follows after a few days.
    • Vertigo, hearing loss and facial weakness may follow (together or independently).
    • Symptoms typically improve over a few weeks (some patients can suffer permanent hearing loss and persistent reduction of caloric responses).
  • Suppurative labyrinthitis (following otitis is uncommon):
    • It is almost always associated with cholesteatoma.
    • Profound hearing loss, severe vertigo, ataxia and nausea and vomiting are common symptoms.
  • Serous labyrinthitis:
    • Is associated with acute or chronic middle ear disease and a common complication of otitis media.
    • An audiogram reveals mixed hearing loss when a middle ear effusion is present.
    • Vestibular symptoms may occur but are less common.
    • The hearing loss is usually transient but may persist if the otitis is left untreated.
    • Treatment is aimed at the underlying infection and clearing the middle ear effusion.
    • The hearing loss is usually transient but may persist if the otitis is left untreated.
  • Vestibular neuronitis or neuritis:
    • Typically presents in a previously healthy person as sudden acute vertigo without hearing loss. It is more common in the fourth and fifth decades of life. It 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.
    • The treatment of vestibular neuritis and viral labyrinthitis is similar.
  • Benign positional vertigo (BPV):[13] 
    • The characteristic nystagmus and vertigo are associated with changes of position.
    • Onset is sudden and severity very variable.
    • People who have BPV do not usually feel dizzy all the time. Severe attacks of dizziness are triggered by head movements. At rest between episodes, patients usually have few or no symptoms.

Other diagnoses to consider are:

  • If the patient presents with sudden-onset unilateral deafness, admission to hospital or urgent assessment by an ear, nose and throat specialist should be considered. The reasoning for this is that such deafness can be indicative of acute ischaemia of the labyrinth or brainstem. Emergency treatment in such cases can restore the patient's hearing.
  • Otherwise, the patient can usually be managed at home. During an acute attack the patient should lie still with eyes closed.
  • Patients should be advised to seek further medical care for worsening symptoms - especially neurological symptoms (such as diplopia, slurred speech, gait disturbances, localised weakness or numbness).[14] 
  • Vertigo, nausea and vomiting may be helped by prochlorperazine, promethazine and domperidone. In an acute attack, gastric emptying will be slowed or even reversed and so a buccal version of prochlorperazine may be preferable.
  • Diazepam or other benzodiazepines are occasionally helpful as a vestibular suppressant.[15] 
  • A short course of oral corticosteroids may be helpful.[16] 
  • Currently, the role of antiviral therapy is controversial.[1] 
  • Always advise patients not to drive or operate machinery when experiencing symptoms or taking medication, as appropriate.
  • Surgical treatment may be necessary - eg, myringotomy and evacuation of effusion in labyrinthitis secondary to otitis media and mastoidectomy for mastoiditis or cholesteatoma.

Although the labyrinthitis is usually considered benign and self-limiting, chronic or recurrent cases merit referral to exclude sinister aetiology. Also the morbidity of labyrinthitis, especially bacterial labyrinthitis, is more significant than is often appreciated. In the paediatric population the risk of hearing loss is significant.

  • The acute symptoms of vertigo and nausea and vomiting resolve after several days to weeks in all the different types of labyrinthitis.
  • Recovery of hearing loss is more variable:
    • Suppurative labyrinthitis usually leaves permanent and profound hearing loss.
    • Hearing loss associated with viral labyrinthitis may recover. Disequilibrium and or positional vertigo also may be present long-term following resolution of the acute infection.
    • Permanent hearing loss is a recognised complication of children with meningitis. Dexamethasone may have some protective effect against this complication but the evidence is equivocal.[17] 
    • Permanent hearing loss occurs in about 10% of patients with herpes zoster oticus.[18] 

It is important:

  • Not to miss a potentially life-threatening condition, such as meningitis, cerebrovascular ischaemia, or brainstem tumour. Chronic or recurrent cases should be referred.
  • To counsel patients not to drive or operate machinery when suffering from vertigo or taking medication for symptoms.

Further reading & references

  • Swartz R, Longwell P; Treatment of vertigo. Am Fam Physician. 2005 Mar 15;71(6):1115-22.
  • Hilton M, Pinder D; The Epley (canalith repositioning) manoeuvre for benign paroxysmal positional vertigo. Cochrane Database Syst Rev. 2004;(2):CD003162.
  • L M Luxon; Evaluation and management of the dizzy patient; Journal of Neurology, Neurosurgery & Psychiatry 2004;75:iv45-iv52
  1. Thompson TL, Amedee R; Vertigo: a review of common peripheral and central vestibular disorders. Ochsner J. 2009 Spring;9(1):20-6.
  2. Parham K, Leonard G, Feinn RS, et al; Prospective clinical investigation of the relationship between idiopathic benign paroxysmal positional vertigo and bone turnover: A pilot study. Laryngoscope. 2013 May 14. doi: 10.1002/lary.24162.
  3. Abi-Hachem RN, Zine A, Van De Water TR; The injured cochlea as a target for inflammatory processes, initiation of cell death pathways and application of related otoprotectives strategies. Recent Pat CNS Drug Discov. 2010 Jun;5(2):147-63.
  4. Lee AT; Diagnosing the cause of vertigo: a practical approach. Hong Kong Med J. 2012 Aug;18(4):327-32.
  5. Neuhauser HK; Epidemiology of vertigo. Curr Opin Neurol. 2007 Feb;20(1):40-6.
  6. Paludetti G, Conti G, DI Nardo W, et al; Infant hearing loss: from diagnosis to therapy Official Report of XXI Conference of Italian Society of Pediatric Otorhinolaryngology. Acta Otorhinolaryngol Ital. 2012 Dec;32(6):347-70.
  7. Merchant SN, Durand ML, Adams JC; Sudden deafness: is it viral? ORL J Otorhinolaryngol Relat Spec. 2008;70(1):52-60; discussion 60-2. doi: 10.1159/000111048. Epub 2008 Feb 1.
  8. Vertigo; NICE CKS, April 2010
  9. Agrup C; Immune-mediated audiovestibular disorders in the paediatric population: a review. Int J Audiol. 2008 Sep;47(9):560-5. doi: 10.1080/14992020802282268.
  10. Labuguen RH; Initial evaluation of vertigo. Am Fam Physician. 2006 Jan 15;73(2):244-51.
  11. Cunningham S et al; Infections of the Labyrinth, University of Texas Medical Branch, 2004
  12. Agrawal Y, Bremova T, Kremmyda O, et al; Semicircular canal, saccular and utricular function in patients with bilateral vestibulopathy: analysis based on etiology. J Neurol. 2013 Mar;260(3):876-83. doi: 10.1007/s00415-012-6724-y. Epub 2012 Oct 27.
  13. Bromwich M; Why do some people get dizzy in the examination chair? J Can Dent Assoc. 2010;76:a62.
  14. Seemungal BM, Bronstein AM; A practical approach to acute vertigo. Pract Neurol. 2008 Aug;8(4):211-21. doi: 10.1136/jnnp.2008.154799.
  15. Swartz R, Longwell P; Treatment of vertigo. Am Fam Physician. 2005 Mar 15;71(6):1115-22.
  16. Jang CH, Park SY, Wang PC; A case of tympanogenic labyrinthitis complicated by acute otitis media. Yonsei Med J. 2005 Feb 28;46(1):161-5.
  17. Manolidis S, Johnson R; Do corticosteroids prevent hearing loss in pediatric bacterial meningitis? An analysis of the evidence. Ear Nose Throat J. 2006 Sep;85(9):586-92.
  18. Gondivkar S, Parikh V, Parikh R; Herpes zoster oticus: A rare clinical entity. Contemp Clin Dent. 2010 Apr;1(2):127-9. doi: 10.4103/0976-237X.68588.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.

Original Author:
Dr Richard Draper
Current Version:
Peer Reviewer:
Dr Helen Huins
Document ID:
2367 (v23)
Last Checked:
04/11/2013
Next Review:
03/11/2018