Neurology · PANCE / PANRE

Vestibular Neuritis and Labyrinthitis

Acute peripheral vestibulopathy from inflammation of the vestibular nerve (neuritis) or whole inner ear (labyrinthitis), producing prolonged vertigo.

Also known as: vestibular neuritis, labyrinthitis, vestibular neuronitis, acute peripheral vestibulopathy, AUVS

Overview

Acute Vestibular Syndrome (AVS) characterized by rapid-onset, prolonged (days), continuous vertigo, nausea/vomiting, gait instability, and spontaneous nystagmus, lasting more than 24 hours. Vestibular neuritis spares hearing; labyrinthitis adds sensorineural hearing loss and/or tinnitus by involving the cochlea.

Epidemiology

Annual incidence 3-15 per 100,000. Peak age 30-60. No clear sex predilection. Vestibular neuritis is the third most common cause of peripheral vertigo after BPPV and Ménière disease.

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Question 1NeurologyMedium
A 44-year-old man presents with the sudden onset of severe, constant spinning vertigo that began 18 hours ago and has not let up, along with nausea, vomiting, and inability to walk without veering to the left. He reports a head cold about 10 days ago but denies any hearing change, tinnitus, ear pain, or facial weakness. On exam he has spontaneous left-beating horizontal-torsional nystagmus that worsens when fixation is removed, an abnormal head impulse test with a catch-up saccade to the left, and no skew deviation. There is no dysarthria, limb dysmetria, or focal weakness, and hearing is normal bilaterally. Which of the following is the most likely diagnosis?
  • ABrainstem infarction
  • BCerebellar infarction
  • CVestibular neuritis
  • DVestibular migraine
Reveal answer & full explanation
Correct answer: C — Vestibular neuritis
  • ABrainstem infarction
  • BCerebellar infarction
  • CVestibular neuritis
  • DVestibular migraine

Why Vestibular neuritis is correct

  • This is an acute vestibular syndrome: rapid-onset, continuous vertigo lasting more than 24 hours with nausea, vomiting, and gait instability, classically preceded by a viral URI 1-2 weeks earlier (attributed to HSV-1 reactivation in the vestibular ganglion).
  • The HINTS exam shows a peripheral pattern: an ABNORMAL head impulse with a catch-up saccade, UNIDIRECTIONAL (left-beating) nystagmus suppressed by fixation, and NO skew. Preserved hearing makes this vestibular neuritis rather than labyrinthitis.
  • Falls toward the affected side and the absence of any brainstem or cerebellar signs further support a peripheral lesion.

Why the others are wrong

  • Cerebellar infarction — the critical central mimic, but it produces a central HINTS pattern (NORMAL head impulse, direction-changing nystagmus, or skew deviation) and is suggested by vascular risk factors and gait failure out of proportion to the nystagmus; this patient has a reassuring peripheral pattern and no cerebellar signs.
  • Vestibular migraine — causes recurrent episodes of vertigo with migraine features such as headache, photophobia, or aura, not a single first prolonged event after a recent URI with a peripheral HINTS pattern.
  • Brainstem infarction — would be expected to show central HINTS findings plus brainstem signs such as dysarthria, diplopia, or skew deviation, none of which are present here.
Question 2NeurologyMedium
A 52-year-old man presents to the emergency department with 18 hours of continuous, ongoing severe spinning vertigo, nausea, and vomiting that began abruptly. He reports a head cold about 10 days ago. He has no hearing loss or tinnitus. He has hypertension and a 20-pack-year smoking history. On exam he has spontaneous left-beating horizontal nystagmus and falls to the right when walking; there is no dysarthria, limb weakness, or dysmetria. Which of the following is the most appropriate next diagnostic test to distinguish a peripheral cause from a posterior circulation stroke?
  • ABrain MRI with DWI sequences
  • BPure-tone audiometry test
  • CBedside HINTS examination
  • DDix-Hallpike maneuver test
Reveal answer & full explanation
Correct answer: C — Bedside HINTS examination
  • ABrain MRI with DWI sequences
  • BPure-tone audiometry test
  • CBedside HINTS examination
  • DDix-Hallpike maneuver test

Why Bedside HINTS examination is correct

  • This patient has acute vestibular syndrome (AVS): abrupt-onset, continuous vertigo with spontaneous nystagmus and gait instability that is still ongoing, the syndrome in which vestibular neuritis must be separated from a posterior circulation (PICA/AICA) stroke.
  • In AVS, the three-step HINTS battery (Head Impulse, Nystagmus direction, Test of Skew) is more sensitive than early MRI for detecting a central cause. A peripheral pattern is an abnormal head impulse (catch-up saccade), unidirectional nystagmus, and no skew deviation; a central pattern is a normal head impulse, direction-changing nystagmus, or skew deviation.
  • His vascular risk factors (hypertension, smoking, age) make stroke exclusion essential, and HINTS provides that answer immediately at the bedside.

Why the others are wrong

  • Brain MRI with DWI sequences is appropriate when HINTS shows a central pattern or central features persist, but DWI can be falsely negative in the first 24-48 h after a posterior-fossa stroke, so it is not the best first discriminating test.
  • Pure-tone audiometry test characterizes the sensorineural hearing loss of labyrinthitis, but this patient has no hearing loss and it does nothing to exclude stroke.
  • Dix-Hallpike maneuver test screens for BPPV, which causes brief, positional vertigo lasting seconds rather than the continuous prolonged vertigo of AVS, and it cannot distinguish a central from a peripheral cause.
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Risk factors

  • Preceding upper respiratory viral illness (1-2 weeks prior in many cases)
  • Reactivation of latent HSV-1 within Scarpa's ganglion (leading hypothesis)
  • Recent ear infection (bacterial labyrinthitis)
  • Cholesteatoma or chronic otitis media (suppurative labyrinthitis)
  • Autoimmune disorders
  • Vascular risk factors (raise suspicion for stroke mimics)

Pathophysiology

Vestibular neuritis is most often attributed to viral (HSV-1) reactivation within the vestibular ganglion or post-infectious immune-mediated demyelination of the superior vestibular nerve (which supplies the horizontal and anterior semicircular canals and utricle). Labyrinthitis involves both vestibular and cochlear structures and may be viral (post-URI) or bacterial (from acute otitis media, meningitis, or cholesteatoma — a true emergency).

Clinical presentation

Symptoms

  • Sudden severe constant vertigo lasting hours to days, often peaking on day 1-2
  • Nausea, vomiting, profuse diaphoresis
  • Gait instability and falls toward the affected side
  • Hearing loss and tinnitus only in labyrinthitis (NOT in neuritis)
  • Often preceded by viral URI symptoms 1-2 weeks earlier
  • Symptoms gradually improve over 1-2 weeks; chronic mild imbalance may persist for months

Signs / physical exam

  • Spontaneous unidirectional horizontal-torsional nystagmus with fast phase AWAY from the affected ear (peripheral pattern)
  • Nystagmus suppressed by visual fixation, intensifies with fixation removed (Frenzel goggles)
  • HINTS exam (Head Impulse, Nystagmus, Test of Skew) — peripheral pattern: abnormal/catch-up saccade on head impulse, unidirectional nystagmus, no skew
  • Positive Romberg, falls toward affected ear
  • Hearing preserved (neuritis) or unilateral SNHL (labyrinthitis)
  • No focal weakness, dysarthria, dysmetria, or other brainstem/cerebellar findings

Classic findings

AVS with peripheral HINTS pattern + recent URI + unidirectional nystagmus suppressed by fixation.

Differential diagnosis

  • Posterior circulation stroke (especially PICA/AICA) — CRITICAL mimic — central HINTS pattern (normal head impulse, direction-changing nystagmus, skew deviation), other brainstem/cerebellar signs, vascular risk factors; MRI with DWI to exclude
  • Ménière disease — Recurrent episodes 20 min-12 h with low-frequency hearing loss, tinnitus, aural fullness; not a single prolonged event
  • BPPV — Brief (seconds) positional vertigo; Dix-Hallpike positive
  • Vestibular migraine — Recurrent episodes with migraine features; vertigo duration variable
  • Multiple sclerosis — Younger patients, recurrent or progressive course, other CNS findings, demyelinating MRI lesions
  • Acoustic neuroma — Progressive unilateral SNHL, gradual imbalance, MRI confirms
  • Ramsay Hunt syndrome (herpes zoster oticus) — Vertigo + hearing loss + ipsilateral facial palsy + vesicular ear-canal rash

Diagnostic workup

Diagnostic criteria

Bárány Society 2022: acute or subacute onset of spinning vertigo, lasting at least 24 h, with spontaneous horizontal nystagmus and unilateral vestibular hypofunction (head impulse or caloric); no acute central neurologic signs; vestibular neuritis if hearing spared, labyrinthitis if cochlear involvement.

Labs

  • Generally clinical diagnosis; labs as needed to exclude metabolic mimics
  • CBC, BMP for hydration assessment

Imaging

  • HINTS exam at bedside is more sensitive than early MRI for posterior stroke in AVS
  • MRI brain with DWI within 24-72 h if any central features, vascular risk factors, age >60, severe imbalance disproportionate to nystagmus, or any HINTS findings suggesting central etiology (INFARCT mnemonic — Impulse Normal, Fast-phase Alternating, Refixation on Cover Test)
  • Audiometry to characterize hearing loss in labyrinthitis
  • Caloric testing or video head impulse test (vHIT) — unilateral vestibular weakness in neuritis
  • Temporal bone CT if cholesteatoma or suppurative labyrinthitis suspected

Treatment

First-line

  • Acute symptomatic relief (24-72 h only, then taper): meclizine 25-50 mg q6-8h, dimenhydrinate, promethazine, lorazepam, ondansetron
  • Hydration — IV fluids if vomiting precludes oral intake
  • Methylprednisolone 100 mg/day PO with taper over 3 weeks — shortens recovery and improves vestibular function recovery in vestibular neuritis (per Strupp NEJM 2004)
  • Vestibular rehabilitation therapy starting within 48-72 h — improves central compensation; the single most important long-term intervention
  • Antivirals (valacyclovir) — not shown to improve outcomes; not routinely recommended

Viral labyrinthitis

  • Same supportive care as vestibular neuritis
  • Audiology evaluation for hearing loss; consider intratympanic or oral steroids for sudden SNHL
  • Hearing aid or cochlear implant for permanent severe loss

Bacterial / suppurative labyrinthitis

  • Otolaryngology consultation urgently
  • IV antibiotics covering AOM/meningitis pathogens (ceftriaxone ± vancomycin)
  • Surgical drainage if cholesteatoma or mastoiditis
  • Steroids to reduce inflammation
  • Meningitis precautions

Ramsay Hunt syndrome

  • Valacyclovir or acyclovir + prednisone
  • Eye protection if facial palsy

Second-line / adjunct

  • Vestibular rehabilitation (gaze stabilization, balance exercises) — outpatient or home program
  • Address driving and fall risk during recovery
  • Counseling — anxiety often complicates recovery

Complications

  • Persistent unilateral vestibular hypofunction with chronic imbalance
  • Permanent hearing loss (labyrinthitis)
  • Secondary BPPV (debris dislodged into posterior canal during acute event) — Dix-Hallpike at follow-up
  • Persistent postural-perceptual dizziness (PPPD) — chronic functional dizziness after acute event
  • Falls and injury
  • Anxiety and avoidance behaviors
  • Suppurative labyrinthitis: meningitis, intracranial extension

PANCE pearls

  • The most important task in AVS is to distinguish vestibular neuritis from a posterior circulation stroke — use HINTS (head impulse, nystagmus direction, test of skew), not MRI alone (MRI DWI may be falsely negative in the first 24-48 h for posterior strokes).
  • Peripheral pattern (consistent with neuritis): ABNORMAL head impulse (catch-up saccade toward affected side), UNIDIRECTIONAL nystagmus, NO skew deviation.
  • Central pattern (consistent with stroke): normal head impulse, direction-CHANGING nystagmus, or skew deviation — refer urgently for stroke evaluation.
  • Limit vestibular suppressants to ~3 days — chronic use impairs central compensation.
  • Steroids accelerate recovery in vestibular neuritis (per Strupp NEJM 2004); antivirals do not.
  • Pure hearing loss without vertigo is NOT labyrinthitis — consider sudden sensorineural hearing loss (SSNHL), an emergency requiring high-dose steroids.

References

  • Bárány Society 2022 — Strupp M et al. Acute unilateral vestibulopathy/vestibular neuritis: Diagnostic criteria. J Vestib Res 2022;32:389-406.
  • Strupp 2004 — Strupp M et al. Methylprednisolone, valacyclovir, or the combination for vestibular neuritis. NEJM 2004;351:354-361.
  • HINTS — Kattah JC et al. HINTS to diagnose stroke in the acute vestibular syndrome: three-step bedside oculomotor examination more sensitive than early MRI DWI. Stroke 2009;40:3504-3510.

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