Bilateral sudden sensorineural hearing loss with Sweet syndrome

Abstract

Introduction: 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. Patient concerns: 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. Diagnosis: Pure tone audiometry and biopsy on the skin lesion were performed. SS with bilateral sudden sensorineural hearing loss was confirmed. Interventions: The patient was treated with intravenous prednisolone and topical steroids. Outcomes: After a week of treatment, skin lesions had improved. And 3 months after treatment, the hearing test showed full recovery. Conclusion: This case emphasizes the point that early diagnosis and timely treatment are essential for hearing recovery in patients with SS who have otologic symptoms.

New Practical Aspects of Sweet Syndrome

“…Involvement of a variety of other extracutaneous sites has also been observed. Bilateral sensorineural hearing loss [96], laryngitis [97], myocarditis [98], and neutrophilic myositis leading to rhabdomyolysis [99] have been recently reported in literature. The gastrointestinal tract may also be involved, and may be accompanied by hepatic and/or renal involvement [2].…”

Hearing restoration and the stria vascularis: evidence for the role of the immune system in hearing restoration

“…Given the evidence for cytokine signalling within the adult mouse stria vascularis and in autoinflammatory and autoimmune disorders, we will now discuss therapeutic targeting of these signalling pathways. Given that treatment with steroids represents the first-line treatment of these disorders [34,42,64,65 ▪ ,66–73,74 ▪ ,75,76 ▪ ,77,78 ▪ ], understanding cytokine signalling in patients with steroid-responsive and steroid-unresponsive disease offers an opportunity to identify critical entry points for therapeutic immunomodulation. Corticosteroids repress IL-6 expression via inhibiting the interaction between NF-IL6 and the p65 subunit of NF- κ B [79].…”