[Cogan syndrome: sudden, bilateral high-grade hearing loss]

Area of Science:

• Ophthalmology and Otolaryngology
• Autoimmune Diseases

Background:

• Cogan's syndrome is a rare autoimmune disorder.
• It affects the eyes and ears, potentially leading to significant sensory loss.

Observation:

• Two female patients presented with varied symptoms.
• One had classic interstitial keratitis and hearing loss.
• The other experienced progressive deafness, vestibular dysfunction, conjunctivitis, and otitis externa.

Findings:

• The second patient later developed typical Cogan's syndrome with interstitial keratitis.
• Hearing impairment is primarily linked to endolymphatic hydrops.
• The condition is driven by an autoimmune response.

Implications:

• Early recognition and treatment are crucial for managing Cogan's syndrome.
• Corticosteroids are the primary therapeutic intervention.
• Understanding the autoimmune basis aids in patient management and research.