Bilateral sudden sensorineural hearing loss with Sweet syndrome: A case report

Sweet syndrome (SS) is an idiopathic autoimmune disease which has been associated with various extracutaneous manifestations. Otologic symptoms secondary to SS are characterized by bilateral, progressive, sensorineural hearing loss, which requires auditory rehabilitation with, for example, cochlear implantation.

A 43-year-old woman complaining of bilateral sudden hearing loss visited the Emergency Department of our University. Abrupt onset of fever peaking up to 40°C and vomiting accompanied the hearing loss and other associated symptoms were: tinnitus that sounded like a machine humming, mild dizziness, a painful rash (on the right upper eyelid, chest, back, forearms, and lower extremities), arthralgia in both the hip and knee joints, and vision loss in the right eye. The patient had no history of autoimmune diseases or surgery.

Pure tone audiometry and biopsy on the skin lesion were performed. SS with bilateral sudden sensorineural hearing loss was confirmed.

The patient was treated with intravenous prednisolone and topical steroids.

After a week of treatment, skin lesions had improved. And 3 months after treatment, the hearing test showed full recovery.

This case emphasizes the point that early diagnosis and timely treatment are essential for hearing recovery in patients with SS who have otologic symptoms.