Granulomatosis With Polyangiitis Presenting as Complete Hearing Loss

Area of Science:

• Rheumatology
• Nephrology
• Otolaryngology

Background:

• Granulomatosis with Polyangiitis (GPA) is a rare systemic autoimmune vasculitis affecting small to medium-sized vessels.
• GPA classically involves the upper respiratory tract, lungs, and kidneys, but can manifest with diverse and sequential symptoms.
• Delayed diagnosis of GPA is common due to its variable presentation, leading to potential irreversible organ damage.

Observation:

• A 29-year-old male with a history of hearing loss presented with hematemesis.
• The patient's initial symptoms were attributed to chronic otitis media.
• Further investigation led to the diagnosis of Granulomatosis with Polyangiitis.

Findings:

• The case highlights that GPA can present initially with seemingly unrelated symptoms such as hearing loss and gastrointestinal bleeding (hematemesis).
• This presentation underscores the importance of considering systemic vasculitis in patients with atypical or sequential symptoms.
• Diagnostic delays in GPA can be mitigated by recognizing a broader spectrum of initial clinical manifestations.

Implications:

• Recognizing the diverse and sometimes atypical presentations of GPA is critical for timely diagnosis and treatment.
• Early intervention in GPA can prevent severe complications and long-term sequelae, including renal failure and pulmonary compromise.
• This case emphasizes the need for a high index of suspicion for autoimmune vasculitis in patients presenting with unusual combinations of symptoms.