Lin Chun-Yu, Lin Szu-Lan, Kao Chun-Chu, Wu Jiunn-Liang
Department of Otolaryngology, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Auris Nasus Larynx. 2005 Jun;32(2):99-105. doi: 10.1016/j.anl.2004.11.001. Epub 2005 Jan 15.
Based on imaging findings, large vestibular aqueduct syndrome (LVAS) in early childhood is the most common cause of sensorineural hearing loss. Children with LVAS are at a high risk of suffering sudden deteriorations in hearing. This study was to review treatment of sudden hearing deterioration in children with LVAS who underwent corticosteroid therapy. We conducted a retrospective study of patients presenting to an academic tertiary medical center. Sixteen children of LVAS were evaluated. Corticosteroid therapy (prednisolone 1-2 mg/(kg day) or equal titer's dexamethasone) was administered as soon as sudden hearing loss developed. The pure tone audiometric result improved more than 10dB at two or more consecutive frequencies and was regarded as a significant response to corticosteroid therapy. Sixteen cases comprising 12 boys and 4 girls were retrospectively analyzed in this study. The mean age at which LVAS was diagnosed was 2.3 years. Mean follow-up for the 16 cases from the first clinic visit to November 2003 was 4.2 years. The initial audiograms varied from down-sloping, valve or rising patterns. In addition, bilateral enlargement of the vestibular aqueduct was found to all children and the mean diameter of right and left ears were 7.23 and 6.83 mm, respectively. Seven children had totally experienced 13 episodes of sudden hearing deterioration. After receiving corticosteroid therapy in time, 11 of 13 episodes had indicated significant responses to treatment, a response rate of 85%. Early detection of LVAS and the timing of treatment are crucial for preventing the residual hearing from deteriorating. As soon as the hearing deterioration of a child with LVAS is recognized, aggressive intervention such as corticosteroid therapy should be performed in no time.
根据影像学检查结果，儿童期大前庭导水管综合征（LVAS）是感音神经性听力损失最常见的病因。患有LVAS的儿童听力突然恶化的风险很高。本研究旨在回顾接受皮质类固醇治疗的LVAS患儿突发听力恶化的治疗情况。我们对一家学术性三级医疗中心的患者进行了回顾性研究。对16例LVAS患儿进行了评估。一旦出现突发听力损失，即给予皮质类固醇治疗（泼尼松龙1-2mg/（kg·天）或等效效价的地塞米松）。纯音听力测定结果在两个或更多连续频率上改善超过10dB被视为对皮质类固醇治疗有显著反应。本研究对16例病例进行了回顾性分析，其中包括12名男孩和4名女孩。LVAS确诊时的平均年龄为2.3岁。16例患者从首次就诊至2003年11月的平均随访时间为4.2年。初始听力图呈下坡型、谷型或上升型。此外，所有患儿均发现双侧前庭导水管扩大，右耳和左耳的平均直径分别为7.23mm和6.83mm。7名儿童共经历了13次突发听力恶化。及时接受皮质类固醇治疗后，13次发作中有11次显示对治疗有显著反应，有效率为85%。早期发现LVAS以及治疗时机对于防止残余听力恶化至关重要。一旦识别出LVAS患儿的听力恶化，应立即进行积极干预，如皮质类固醇治疗。
