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Vogt-Koyanagi-Harada Disease Mimicking Idiopathic Intracranial Hypertension

A dispatch from PubMed — filed

Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune disorder characterized by an inflammatory response targeting melanocyte-associated antigens in tissues such as the uveal tract, inner ear, meninges, and the skin. The classic presentation is bilateral pan-uveitis (choroiditis and multifocal serous retinal detachment) in association with extra-ocular features such signs of meningeal irritation (headache,...

Clinical Takeaway

Audiologists and otologists should consider VKH disease in patients presenting with audiovestibular symptoms alongside eye inflammation or skin/hair changes, as misdiagnosis can delay sight- and hearing-preserving immunotherapy.

Why It Matters

Raising awareness that VKH disease can mimic common neurological diagnoses is critical for preventing irreversible hearing and vision loss through timely immunosuppressive treatment.

Key Points
  1. 01VKH disease, a rare autoimmune disorder targeting pigment (melanocyte) cells, can present with hearing and balance symptoms.
  2. 02This case mimicked idiopathic intracranial hypertension (raised brain pressure without a known cause), risking misdiagnosis.
  3. 03Delayed diagnosis of VKH can lead to permanent hearing loss, vision loss, or both.
  4. 04Audiovestibular manifestations are a recognized but often overlooked feature of VKH disease.
  5. 05Case highlights the need for multidisciplinary evaluation when audiovestibular symptoms co-occur with eye or skin findings.
Claims & Evidence

VKH disease can clinically mimic idiopathic intracranial hypertension, including audiovestibular manifestations.

studysupported

VKH disease targets melanocyte antigens as part of its autoimmune mechanism.

studysupported
Research metadata
PMID
42131163
DOI
10.1080/01658107.2025.2507403.
Journal
Acta Oto-Laryngologica
Publication type
case_report
Evidence level
4
Sample size
1
Population
Individual patient with Vogt-Koyanagi-Harada disease presenting with audiovestibular symptoms mimicking idiopathic intracranial hypertension
Intervention
Clinical evaluation and diagnosis of VKH disease with audiovestibular manifestations

Primary outcomes

Characterization of audiovestibular features of VKH disease; Differential diagnosis from idiopathic intracranial hypertension

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