What autoimmune diseases are associated with vestibular problems?

Autoimmune Diseases Associated with Vestibular Problems

Several autoimmune diseases are definitively associated with vestibular dysfunction, with the most important being autoimmune inner ear disease, Cogan syndrome, systemic lupus erythematosus, sarcoidosis, and multiple sclerosis.

Primary Autoimmune Vestibulocochlear Disorders

Autoimmune Inner Ear Disease (AIED)

• AIED presents with progressive fluctuating bilateral hearing loss that is typically steroid-responsive, with vertigo occurring in some cases 1.
• The fluctuation of hearing is a key distinguishing feature that should raise suspicion for this diagnosis 1.
• This condition may present with bilateral sudden hearing loss or progressive audiovestibular symptoms 1.

Cogan Syndrome

• Cogan syndrome is characterized by the triad of nonsyphilitic interstitial keratitis of the cornea, hearing loss, and vertigo 1.
• Look specifically for concurrent eye symptoms including pain, redness, lacrimation, and photophobia in patients presenting with audiovestibular complaints 1.
• This is a critical diagnosis not to miss as it requires specific immunosuppressive therapy 1.

Systemic Autoimmune Diseases with Vestibular Involvement

Systemic Lupus Erythematosus (SLE)

• SLE patients have a 24% prevalence of sensorineural hearing loss or episodic vertigo, with 9% experiencing episodic vertigo specifically 2.
• Histopathologic studies demonstrate that type I vestibular hair cells are significantly reduced in density in all five vestibular sensory epithelia (saccular macula, utricular macula, and all three semicircular canal cristae) in SLE patients 3.
• The association between sensorineural hearing loss and episodic vertigo is statistically significant in SLE patients, suggesting a common audiovestibular dysfunction mechanism 2.
• SLE may present with progressive fluctuating bilateral hearing loss and can mimic other autoimmune inner ear conditions 1.
• Vision, skin, and joint problems may accompany the audiovestibular symptoms 1.

Multiple Sclerosis

• Multiple sclerosis can present with sudden hearing loss and vestibular symptoms when involving the brainstem or cerebellar peduncles 1.
• Look for additional neurologic features including unilateral weakness or numbness, visual loss, diplopia, or paraparesis 1.
• MRI typically shows white matter signal abnormalities, particularly on FLAIR sequences 1.
• Approximately 4% of acute vestibular syndrome cases are due to MS involving brainstem or cerebellar structures 1.

Sarcoidosis

• Sarcoidosis causes bilateral vestibular loss along with pulmonary symptoms 1.
• Diagnostic clues include elevated serum angiotensin-converting enzyme levels or abnormal Gallium scan 1.
• This should be considered in patients with known pulmonary sarcoidosis presenting with new vestibular complaints 1.

Additional Autoimmune Conditions with Vestibular Manifestations

Behçet's Disease

• Behçet's disease is associated with cochleovestibular involvement as part of its multisystem inflammatory presentation 4.

Vogt-Koyanagi-Harada Syndrome

• This syndrome presents with audiovestibular disorders along with uveitis and neurologic manifestations 4.

Relapsing Polychondritis

• Relapsing polychondritis can cause audiovestibular symptoms through inflammation of cartilaginous structures 4.

Antiphospholipid Syndrome

• Antiphospholipid syndrome may cause vestibular dysfunction, particularly when occurring concomitantly with SLE 2.

IgG4-Related Disease

• IgG4-related disease has been recognized as causing audiovestibular disorders 4.

ANCA-Associated Vasculitides

• ANCA-associated vasculitides (including granulomatosis with polyangiitis) can present with cochleovestibular involvement 4.

Clinical Approach and Key Diagnostic Considerations

Red Flags Requiring Immediate Workup

• Bilateral sudden hearing loss should prompt evaluation for autoimmune, infectious, ototoxic, or vascular causes 5, 6.
• Progressive fluctuating hearing loss, especially bilateral, strongly suggests autoimmune etiology 1.
• Concurrent eye symptoms (pain, redness, photophobia) with audiovestibular complaints mandate evaluation for Cogan syndrome 1.

Essential History Elements

• Specifically ask about fluctuation of hearing, as this distinguishes autoimmune causes from other etiologies 1.
• Document timing and pattern of vertigo episodes (episodic versus continuous) 1.
• Inquire about systemic symptoms including joint pain, skin rashes, pulmonary symptoms, and visual changes 1, 4.
• Obtain detailed medication history to exclude ototoxic causes 1, 6.

Physical Examination Priorities

• Perform complete neurologic examination to exclude central pathology, particularly if dizziness or imbalance is present 5.
• Examine for focal neurologic deficits including weakness, numbness, dysarthria, hemiataxia, and facial weakness 1.
• Look for downbeating or gaze-evoked nystagmus suggesting central involvement 1.
• Examine eyes for keratitis, uveitis, or other inflammatory changes 1, 4.

Diagnostic Testing Strategy

• Audiometry is mandatory if any hearing loss is suspected or if tinnitus or aural fullness accompanies vestibular symptoms 5.
• Brain MRI is indicated when central pathology is suspected or when peripheral causes have been excluded 1.
• Consider autoimmune serologies including ANA, antiphospholipid antibodies, and disease-specific markers based on clinical presentation 2.
• Serum angiotensin-converting enzyme level or Gallium scan if sarcoidosis is suspected 1.

Common Pitfalls to Avoid

• Do not dismiss bilateral audiovestibular symptoms as benign without considering autoimmune etiologies, as early treatment with immunosuppression may prevent permanent damage 1.
• Fluctuating symptoms are often mistakenly attributed to Ménière's disease when they may represent autoimmune inner ear disease or systemic autoimmune conditions 1.
• Absence of systemic symptoms does not exclude autoimmune disease, as audiovestibular manifestations may precede other organ involvement 4, 2.
• In patients with known autoimmune disease presenting with vestibular symptoms, do not assume symptoms are unrelated—there is a well-established pathophysiologic connection 2, 3.