Abstract

Hearing loss has a broad differential diagnosis. Hearing impairment responding to corticosteroids is suggestive of an underlying autoimmune cause. We report a rare case of Cogan’s syndrome in a 28-year-old woman with no previous personal medical history. She had experienced two years of fluctuating hearing loss and bilateral keratitis that would respond to high-dose prednisone prescribed by her otorhinolaryngologist. We performed a complete work-up and ruled out most common causes of autoimmune inner ear diseases. Patient was diagnosed with Cogan’s syndrome based on clinical findings and prescribed corticosteroid-sparing immunosuppressive medication. During follow-up, she developed worsening fatigue, new onset anemia, and melena. Endoscopic and pathologic findings were diagnostic of Crohn’s disease. This case emphasizes the high prevalence of concomitant autoimmune diseases.

Highlights

  • Hearing loss has a broad differential diagnosis

  • A 28-year-old woman was referred to our internal medicine clinic by her otolaryngologist to pursue investigations for recurrent disabling episodes of hearing loss that would respond to systemic corticosteroids

  • The patient had no particular personal medical history and had a family history of sarcoidosis and Crohn’s disease in first-degree cousins. She experienced an acute episode of left ear sensorineural hearing loss in the high frequencies (>2000 Hz) with tinnitus and severe vertigo without a precipitating event

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Summary

Introduction

Hearing loss has a broad differential diagnosis. Hearing impairment responding to corticosteroids is suggestive of an underlying autoimmune cause. A 28-year-old woman was referred to our internal medicine clinic by her otolaryngologist to pursue investigations for recurrent disabling episodes of hearing loss that would respond to systemic corticosteroids. The patient had no particular personal medical history and had a family history of sarcoidosis and Crohn’s disease in first-degree cousins.

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Conclusion

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