Otologic manifestations of relapsing polychondritis: Review of literature and report of nine cases

Objective Relapsing polychondritis (RP) is an episodic disease most likely of autoimmune etiology, characterized by recurrent inflammation of cartilaginous structures. Methods Retrospective case study at two tertiary referral centers with presentation of nine patients with otologic involvement of RP, review of the spectrum of otologic disorders seen, and treatment. Results The clinical course of otologic manifestations of RP was highly variable and ranged from mild to moderate. In 6/9 patients there was an association with other autoimmune disorders. In addition to recurrent auricular chondritis, which was present in 8/9 patients, our patients had otitis externa, chronic myringitis, Eustachian tube dysfunction, conductive hearing loss, sensorineural hearing loss, and tinnitus. All patients had their diagnosis of RP made on the basis of their otologic involvement and the response to systemic corticosteroids. Conclusion The diagnosis of RP is primarily clinical, but laboratory studies and biopsy may contribute as well. Once the diagnosis is suspected, the otolaryngologist should consider consultation with a rheumatologist to assist in the management of additional systemic manifestations