This case study presents a report of inflammatory pseudotumor of the inner ear in a child, discusses radiographic findings and clinical management, and reviews the current literature on this rare disease. A 2.5-year-old presented with otalgia, transient vertigo, and fluctuating facial palsy partially responsive to myringotomy with tube. Work-up for infectious and neoplastic processes was negative. Computed tomography (CT) of the temporal bone showed a very unusual, expansile, erosive appearance to the otic capsule, and magnetic resonance imaging of the temporal bone revealed soft tissue enhancement of the middle ear, mastoid, and inner ear. The patient's symptoms were transiently responsive to tympanomastoidectomy. Inner ear histopathology after labyrinthectomy revealed changes consistent with pseudotumor. Inflammatory pseudotumor of the inner ear is extremely rare but needs to be considered in cases of refractory otitis media with facial palsy, particularly when certain changes appear on CT of the temporal bone.

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