Acute labyrinthitis

Types and causes

Clinical features

• Sudden-onset severe rotatory vertigo with nausea, vomiting, pallor and sweating.
• Tinnitus and hearing loss, which distinguish labyrinthitis from vestibular neuritis.
• In suppurative disease, tinnitus and dizziness progress rapidly to whirling vertigo. Otalgia, otorrhoea and fever reflect the underlying OM (Cummings).

• Spontaneous, direction-fixed horizontal-torsional nystagmus. It is irritative (beating toward the affected ear) early and becomes paralytic (beating toward the opposite ear) later.
• The nystagmus is suppressed by visual fixation. It is stronger when looking in the direction of the fast phase (Alexander's law).
• The patient falls or veers toward the affected side, and the Romberg and Fukuda tests are abnormal.
• Head impulse test (HIT) is positive, with a catch-up saccade toward the affected side (Rosen's Emergency Medicine).
• Otoscopy may show OM, a cholesteatoma or a perforation. A positive fistula sign suggests a canal fistula.
• Suppurative labyrinthitis is rare in the antibiotic era (K J Lee's).

• After the first several hours, vertigo and nystagmus abate. Central compensation over 2 to 3 weeks restores near-normal balance.
• In suppurative disease the tinnitus often fades, but hearing is lost permanently (Cummings).
• Recovery reflects contralateral compensation, not return of function in the affected ear (Scott-Brown's).

• Vestibular neuritis (no hearing loss).
• Posterior circulation stroke or cerebellar infarct.
• Meniere's disease.
• Perilymph fistula.
• Ramsay Hunt syndrome.
• Autoimmune inner ear disease.
• Vestibular migraine.
• Vestibular schwannoma.

• Meningitis.
• Labyrinthitis ossificans (fibrous or bony replacement of the labyrinth).
• Permanent unilateral SNHL.
• Chronic imbalance.

Investigations

1. Bedside examination. Check for nystagmus, HIT, and test of skew. In acute continuous vertigo with nystagmus, use HINTS (head impulse, nystagmus, test of skew) to exclude a central cause. These findings suggest a central lesion:
   • a normal HIT;
   • direction-changing or vertical nystagmus;
   • skew deviation.
   A peripheral picture shows an abnormal HIT, unidirectional nystagmus and no skew (Symptom to Diagnosis). Also look for new severe headache, neck pain, focal neurology, dysarthria, ataxia or inability to sit.
2. Pure tone audiometry. This confirms SNHL, which separates labyrinthitis from neuritis and is the baseline for steroid decisions. A conductive component may be present from middle ear fluid. Repeat audiometry to track recovery.
3. Culture. Swab any ear discharge or middle ear effusion. Take blood cultures if febrile. Consider lumbar puncture only if meningitis is suspected and imaging is safe.
4. Imaging.
   • No imaging is needed for a typical viral picture, or for a typical suppurative picture with OM and a predisposing condition (Cummings).
   • Imaging is indicated when complications, a central cause or an anatomical defect is suspected.
   • CT temporal bone shows mastoiditis, cholesteatoma, fistula and labyrinthine anomalies. It does not diagnose labyrinthitis itself (Scott-Brown's).
   • MRI with gadolinium shows enhancement within the labyrinth (vestibule and cochlea) in acute viral and bacterial labyrinthitis. This is thought to reflect breakdown of the labyrinth-blood barrier (Shambaugh). In suppurative cases MRI also looks for meningeal and intracranial extension.
   • In the ED, MRI is advised for bacterial labyrinthitis, and none is needed for viral cases (Tintinalli's).
5. Vestibular tests (caloric testing, vHIT, VEMPs) confirm a unilateral deficit but are not needed acutely.
6. Other tests when relevant. Syphilis serology, and autoimmune screening if hearing loss is progressive or bilateral.

Management

• Bed rest in the first 24 to 72 hours.
• Vestibular suppressants only short term (a few days) for severe vertigo and vomiting: prochlorperazine, cyclizine, or a benzodiazepine such as diazepam.
• IV fluids and antiemetics if vomiting is severe.
• Prolonged use delays central compensation.

• Start as soon as symptoms allow, within days.
• A 2024 meta-analysis of 12 RCTs (536 patients) found rehabilitation comparable to steroids for dizziness handicap in vestibular neuritis. Rehabilitation plus steroids was better than steroids alone (PMID 37339059). The evidence is for vestibular neuritis and is extrapolated to labyrinthitis.

• Viral labyrinthitis or sudden SNHL: a short oral prednisolone course, for example 1 mg/kg (about 60 mg) daily for 5 to 7 days, tapered. Intratympanic steroids are an option for salvage or when systemic steroids are contraindicated. Evidence for hearing recovery is limited, and the textbooks describe benefit as suggested but based on small studies (Scott-Brown's). Dosing here is from general practice, not the retrieved sources.
• Antivirals have no proven added benefit. Valacyclovir was not helpful in the Strupp trial.
• Serous labyrinthitis: treat the OM source. Oral antibiotics are typically effective, and steroids may improve hearing outcomes but data are lacking. Do a myringotomy if the infection does not resolve. Do a tympanomastoidectomy if cholesteatoma is present (K J Lee's). Adjuvant corticosteroids should be considered when labyrinthitis complicates acute OM (Shambaugh).

• Antibiotics. Give IV broad-spectrum antibiotics with good CSF penetration, such as a third-generation cephalosporin. Recommended duration is about 10 days. This does not reverse the labyrinthine damage but prevents meningeal spread (Cummings).
• Surgery. Manage the underlying OM or cholesteatoma. Options range from ventilation tube insertion to tympanomastoidectomy under antibiotic cover. Labyrinthectomy is unnecessary in labyrinthitis secondary to acute OM (Cummings).
• Steroids. Early steroids may help prevent labyrinthitis ossificans (Scott-Brown's).
• Monitoring. Watch for meningitis, facial palsy and intracranial extension.
• Long-term. The prognosis for recovery of hearing and vestibular function is poor. Unilateral vestibular loss is managed with physical therapy. Unilateral deafness can be managed with a bone-anchored or implantable hearing device (Shambaugh). Prompt CT or MRI is advised in children with deafness, because cochlear ossification can affect later cochlear implantation.

• Repeat audiometry at 2 to 4 weeks.
• Continue rehabilitation if imbalance persists.
• Refer if symptoms are recurrent or the hearing loss is fluctuating.

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Acute labyrinthitis

Types and causes

Clinical features

• Sudden severe rotatory vertigo with nausea, vomiting, pallor and sweating.
• Tinnitus and hearing loss.
• In suppurative disease, tinnitus and dizziness progress rapidly to whirling vertigo. Otalgia, otorrhoea and fever from the underlying OM are common (Cummings).

• Spontaneous, direction-fixed horizontal-torsional nystagmus.
  • It beats toward the affected ear early (irritative).
  • It beats toward the opposite ear later (paralytic).
  • Visual fixation suppresses it, and it is stronger on gaze in the direction of the fast phase.
• Gait is unsteady, with falling or veering toward the affected side.
• The head impulse test is abnormal, with a corrective saccade (Rosen's).
• Otoscopy may show OM, perforation or cholesteatoma. A positive fistula sign suggests a canal fistula.
• Fever, severe headache or neck stiffness suggests meningitis.

• Vertigo and nystagmus ease after the first several hours, and improvement continues over days.
• Central compensation over 2 to 3 weeks restores near-normal balance.
• In suppurative disease hearing is lost permanently (Cummings).
• Recovery reflects contralateral compensation, not return of function in the affected ear (Scott-Brown's).

• Vestibular neuritis (no hearing loss).
• Posterior circulation stroke or cerebellar infarct.
• Meniere's disease.
• Perilymph fistula.
• Ramsay Hunt syndrome.
• Vestibular migraine.
• Vestibular schwannoma.
• Autoimmune inner ear disease.

• Meningitis.
• Labyrinthitis ossificans (fibrous or bony replacement of the labyrinth).
• Permanent unilateral deafness.
• Chronic imbalance.
• Facial palsy.

Investigations

1. Rule out a central cause first. Use HINTS (head impulse, nystagmus, test of skew) in acute continuous vertigo with nystagmus.
   • Central signs: a normal head impulse test, direction-changing or vertical nystagmus, or skew deviation.
   • Peripheral picture: an abnormal head impulse test, unidirectional nystagmus and no skew (Symptom to Diagnosis).
   • Also look for focal neurology, severe headache, ataxia or inability to sit.
2. Pure tone audiometry. It confirms SNHL, which separates labyrinthitis from neuritis. It gives a baseline for steroid decisions and for follow-up. A conductive component may come from middle ear fluid.
3. Microbiology. Swab ear discharge or a middle ear effusion. Take blood cultures if febrile. Do a lumbar puncture only if meningitis is suspected and imaging shows it is safe.
4. Imaging, only when there is a reason for it.
   • A typical viral picture, or a typical suppurative picture in a patient with OM and a predisposing condition, needs no specific studies (Cummings).
   • CT temporal bone shows mastoiditis, cholesteatoma, fistula and labyrinthine malformation. It does not diagnose labyrinthitis itself (Scott-Brown's).
   • MRI with gadolinium shows enhancement within the labyrinth in acute viral and bacterial labyrinthitis. This reflects breakdown of the blood-labyrinth barrier (Shambaugh). Delayed post-contrast 3D-FLAIR is the most sensitive sequence. MRI is advised when bacterial labyrinthitis is suspected and none is needed for viral cases (Tintinalli's).

1. Vestibular function tests (calorics, video head impulse test, VEMPs) confirm a unilateral deficit but are not needed in the acute phase.
2. Other tests as indicated. Syphilis serology, Lyme serology, and an autoimmune screen if loss is progressive or bilateral.

Management

• Rest and fluids in the first 1 to 3 days. Give antiemetics, such as promethazine or prochlorperazine, if vomiting is severe.
• Use vestibular suppressants such as meclizine or diazepam for only 3 to 5 days (Textbook of Family Medicine). Longer use delays central compensation.
• Start early mobilisation and vestibular rehabilitation as symptoms allow. A 2024 meta-analysis of 12 RCTs in vestibular neuritis found rehabilitation comparable to steroids, and rehabilitation plus steroids better than steroids alone (PMID 37339059). A Cochrane review also supports vestibular rehabilitation for peripheral vestibular dysfunction. This evidence is from vestibular neuritis and mixed populations, and is extrapolated to labyrinthitis.

• Treat the hearing loss like sudden SNHL: a short oral corticosteroid course started early, for example prednisolone about 1 mg/kg daily (up to 60 mg) for 5 to 7 days, with or without a taper. Intratympanic steroid is an option if systemic steroids are contraindicated or as salvage. Dosing is from standard practice, not the retrieved textbooks.
• Evidence for benefit is limited. The textbooks describe steroid benefit in vestibular neuritis as suggested but based on small studies (Scott-Brown's).
• Antivirals have not shown added benefit. In the Strupp trial, valacyclovir added nothing to methylprednisolone.
• Repeat audiometry at 2 to 4 weeks.

• Treat the source. Oral antibiotics are typically effective for the OM (K J Lee's).
• Do a myringotomy with a ventilation tube if the infection persists.
• Do a tympanomastoidectomy if there is cholesteatoma.
• Consider steroids (oral or intratympanic). They may improve hearing outcomes, but data are lacking.
• Untreated, serous disease can progress to bacterial labyrinthitis (Swanson's Family Medicine Review).

• Admit the patient. Give IV broad-spectrum antibiotics with CSF penetration, adjusted to culture. Treatment is about 10 days. It will not reverse the damage but is needed to stop spread to the meninges (Cummings; K J Lee's).
• Treat the underlying ear disease, under antibiotic cover.
  • Use a ventilation tube if there is no cholesteatoma.
  • Use tympanomastoidectomy if there is cholesteatoma.
  • Labyrinthectomy is unnecessary when the cause is acute OM.
• Give steroids at diagnosis. They are associated with less labyrinthitis ossificans and, in meningitic labyrinthitis from H. influenzae and S. pneumoniae, better hearing outcomes (Cummings; K J Lee's). Evidence on preventing SNHL is still unclear.
• Watch for meningitis, facial palsy and intracranial extension.
• Follow with serial CT or MRI for labyrinthitis ossificans if SNHL persists. This matters in children who may need a cochlear implant.
• Long-term, unilateral vestibular loss is managed with physical therapy. Unilateral deafness can be managed with a bone-anchored or implantable hearing device (Shambaugh). Consider cochlear implant assessment for bilateral or profound loss.

• Audiometry at 2 to 4 weeks, then as needed.
• Continue rehabilitation until compensation is complete.
• Refer if hearing fluctuates, symptoms recur, or the loss is bilateral.

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