How does systemic lupus erythematosus (SLE) affect the vestibular system and what is the recommended management?

Vestibular Involvement in Systemic Lupus Erythematosus

Overview

Cranial neuropathy affecting the eighth cranial nerve (vestibulocochlear) is a recognized neuropsychiatric manifestation of SLE, though relatively uncommon, occurring in 1-5% of patients. 1 The vestibular system can be affected through inflammatory mechanisms, vascular injury, or immune-mediated damage to the inner ear structures.

Clinical Presentation

Audiovestibular Symptoms

• Episodic vertigo occurs in approximately 9-24% of SLE patients, with some meeting criteria for Meniere's disease-like presentations 2
• Sensorineural hearing loss (SNHL) is found in 21-24% of patients and is significantly associated with episodic vertigo 3, 2
• Patients may present with severe vertigo, unsteady gait, and nystagmus (including vertical nystagmus patterns) 4
• Tinnitus can accompany vestibular symptoms 2

Key Clinical Associations

• Audiovestibular disturbances are more prevalent than historically recognized, with up to 67% of SLE patients reporting symptoms 3
• SNHL and episodic vertigo are associated conditions in SLE, suggesting common audiovestibular dysfunction rather than isolated pathology 2
• Migraine is comorbid in approximately 29% of SLE patients with audiovestibular symptoms but is not directly associated with SNHL or vertigo 2

Diagnostic Evaluation

Initial Assessment

The diagnostic work-up should parallel evaluation of non-SLE patients with similar manifestations, then secondarily attribute findings to lupus activity. 1

Essential Testing

• Pure-tone audiometry to document sensorineural hearing loss 3
• Electronystagmography (ENG) shows abnormalities in approximately 50% of symptomatic SLE patients 3
• Impedance audiometry for baseline evaluation 3
• Video-oculography with oculomotor and caloric testing for comprehensive vestibular assessment 2

Neuroimaging

• Brain MRI (T1/T2, FLAIR, diffusion-weighted imaging, gadolinium-enhanced T1) to exclude brainstem stroke, meningitis, or structural lesions 1
• MRI may show focal abnormal signal intensity in the medulla oblongata in cases with central vertigo 4
• Note that MRI may be normal despite clinical audiovestibular dysfunction 3

Laboratory Evaluation

• CSF analysis to exclude CNS infection, particularly when fever or systemic symptoms are present 1
• Standard SLE serologies: anti-dsDNA, complement levels (C3, C4), antiphospholipid antibodies 1
• Complete blood count to assess for cytopenias 1

Differential Diagnosis Considerations

Other neurological conditions must be excluded before attributing symptoms to SLE:

• Brainstem stroke (particularly in antiphospholipid antibody-positive patients) 1
• CNS infection (bacterial, viral, fungal meningitis) 1
• Meniere's disease (which has increased prevalence in SLE patients) 2
• Medication-related vestibular toxicity 1

Management Algorithm

When to Treat with Immunosuppression

Glucocorticoids and immunosuppressive therapy are indicated when cranial neuropathy (including eighth nerve involvement) is thought to reflect an inflammatory process, particularly in the presence of concurrent generalized lupus activity. 1

First-Line Immunosuppressive Therapy

• Pulse intravenous methylprednisolone in combination with intravenous cyclophosphamide is the recommended regimen for severe cranial neuropathy 1
• This combination has demonstrated effectiveness in other severe NPSLE manifestations with response rates of 60-75% 1
• Treatment should be initiated promptly, as delay beyond 2 weeks is associated with worse neurological outcomes 1

Maintenance Therapy

• Chronic immunosuppressive therapy is necessary given the 50-60% relapse rate during corticosteroid dose reduction 1
• Options include azathioprine, mycophenolate mofetil, or continued cyclophosphamide 1

When to Use Anticoagulation

Antiplatelet or anticoagulation therapy should be considered in antiphospholipid antibody-positive patients, particularly when vestibular symptoms are associated with thrombotic mechanisms or ischemic injury. 1

Anticoagulation Strategy

• For first venous thrombotic event: target INR 2.0-3.0 1
• For arterial thrombosis or recurrent events: target INR 3.0-4.0 1
• Low-dose aspirin may be considered for primary prevention in antiphospholipid antibody-positive patients 1

Symptomatic Management

• Anti-epileptic drugs or vestibular suppressants may be used for symptomatic control while addressing underlying inflammatory process 1
• Dopamine antagonists can be effective for movement-related symptoms 1

Monitoring and Follow-Up

Routine Surveillance

Audiologic research should include routine pure-tone audiometry and ENG assessment for all SLE patients to enable early detection and crucial treatment. 3

• Baseline audiovestibular testing at SLE diagnosis
• Repeat testing when new symptoms develop or with disease flares
• Serial audiometry for patients on potentially ototoxic medications

Response Assessment

• Clinical improvement should occur within days to 3 weeks with appropriate immunosuppressive therapy 1
• Neurological response should parallel improvement in systemic disease activity 1
• Brain SPECT imaging may help monitor treatment response in selected cases 1

Important Caveats

Prognostic Factors

• Extensive MRI lesions, reduced muscle strength, sphincter dysfunction, antiphospholipid antibodies, and delayed treatment initiation (>2 weeks) are associated with severe neurological deficits 1
• Visual outcome in SLE-related optic neuropathy is poor, with only 30% maintaining visual acuity >20/25, highlighting the importance of early aggressive treatment for cranial neuropathies 1

Clinical Pitfalls

• Do not dismiss audiovestibular symptoms as non-specific; they may represent true eighth nerve involvement requiring immunosuppression 3, 2
• Absence of MRI abnormalities does not exclude vestibular involvement 3
• Normal CSF does not rule out NPSLE, as mild abnormalities are common but non-specific 1
• Consider that symptoms may precede other organ involvement, necessitating vigilant monitoring for systemic disease activity 1