You Yeon Seok, Park Sang Woo, Yun Seok Kweon, Lee Eun Jung
Department of Otorhinolaryngology-Head and Neck Surgery.
Research Institute of Clinical Medicine of Chonbuk National University, Biomedical Research Institute of Chonbuk National University Hospital, Jeon-ju, Republic of Korea.
Medicine (Baltimore). 2020 Sep 4;99(36):e22127. doi: 10.1097/MD.0000000000022127.
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.
Sweet综合征(SS)是一种特发性自身免疫性疾病,与多种皮肤外表现有关。SS继发的耳科症状以双侧、进行性感音神经性听力损失为特征,这需要进行听觉康复,例如人工耳蜗植入。
一名43岁女性因双侧突发听力损失前往我校急诊科就诊。听力损失伴有突然发热,体温高达40°C,伴有呕吐,其他相关症状包括:耳鸣,听起来像机器嗡嗡声,轻度头晕,疼痛性皮疹(右上眼睑、胸部、背部、前臂和下肢),髋关节和膝关节疼痛,以及右眼视力丧失。该患者无自身免疫性疾病或手术史。
进行了纯音听力测定和皮肤病变活检。确诊为伴有双侧突发感音神经性听力损失的SS。
患者接受了静脉注射泼尼松龙和局部类固醇治疗。
治疗一周后,皮肤病变有所改善。治疗3个月后,听力测试显示完全恢复。
本病例强调了对于有耳科症状的SS患者,早期诊断和及时治疗对于听力恢复至关重要。