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Clinical Problem Solving: Radiology
June 2005

Radiology Quiz Case 1—Diagnosis

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2005;131(6):536-537. doi:10.1001/archotol.131.6.536

Although McCabe1 first reported AIED in adults in 1979, little is known about the diagnosis and management of AIED in the pediatric population. The differential diagnosis of our patient’s hearing loss included juvenile rheumatoid arthritis with inner ear involvement, atypical Cogan syndrome, systemic lupus erythematosus, relapsing polychondritis, Behçet syndrome, Wegener granulomatosis, polyarteritis nodosa, and the more rare Vogt-Koyanagi-Harada syndrome (uveoencephalitis that includes decreased visual acuity, meningitis signs, SNHL, and poliosis or alopecia).2 In the present case, because of the patient’s age, most of these causes of SNHL were unlikely. When AIED is being considered as a possible cause, the main features include rapidly progressive bilateral hearing loss due to either systemic or organ-specific disease when there is reactivity against a self-antigen found in the inner ear.3 Ndiaye et al2 reported 3 cases of pediatric Cogan syndrome that share some features with our case, such as ophthalmologic and rheumatologic involvement with SNHL. Veldman et al4 described a 14-year-old girl with sudden-onset bilateral SNHL without vestibular symptoms. She, like our patient, complained of fatigue, arthralgia, and malaise and was found by an indirect granulocyte phagocytosis test and a C1q binding assay to have circulating immune complexes that later disappeared after response to steroid treatment.