Lordoglu Begüm, Acar Baran, Yazilitas Fatma, Ozlu Sare Gulfem, Senel Saliha
Department of Pediatrics, Dr. Sami Ulus Maternity and Children's Health and Diseases Training Hospital, Ankara, Turkey.
Department of ENT, Kecioren Training and Research Hospital, Ankara, Turkey.
Int J Pediatr Otorhinolaryngol. 2016 Aug;87:139-42. doi: 10.1016/j.ijporl.2016.06.015. Epub 2016 Jun 14.
To evaluate cochlear functions in patients with Familial Mediterranean Fever in relation to the disease severity score and treatment duration.
50 patients (4-18 years) who had been followed-up with the diagnosis of FMF and regularly receiving appropriate colchicine treatment and 39 healthy controls were included in the study. All the patients and controls were evaluated by audiologic evaluation, including high-frequency pure-tone audiometry and distortion product otoacoustic emission tests (DPOAE). The disease severity was determined by scoring system developed by Pras et al.
Fifty patients (52% female, 48% male; mean age12.2 ± 4.1 years) and 39 controls (58.9% female, 41.1% male, mean age 11.1 ± 3.4 years) were enrolled the study. The pure tone average of FMF patients was significantly higher than that of the control group at 500, 4000, and 8000 Hz frequencies. The patients' DPOAE signal values at 6 kHz, 8 kHz frequencies and SNR values at 8 kHz were significantly higher than control group. The patients' audiometry and DPOAE results were compared with the disease severity scores. Pure tone average was significantly higher in severe and moderate patient groups compared to the mild patient group at 2000 Hz frequency. DPOAE signal values showed statistically significant differences between the patient severity scores at 1.4 and 2.8 kHz frequencies. The mean colchicine treatment duration was found to be 5.1 ± 3.7 years. There were significant differences at 250 and 500 Hz frequencies when patients' audiometry results were compared with the treatment periods.
FMF affects cochlear functions particularly at high frequencies.
评估家族性地中海热患者的耳蜗功能与疾病严重程度评分及治疗持续时间的关系。
本研究纳入了50例（4至18岁）经诊断为家族性地中海热并接受定期适当秋水仙碱治疗的患者以及39名健康对照者。所有患者和对照者均通过听力学评估，包括高频纯音测听和畸变产物耳声发射测试（DPOAE）。疾病严重程度由Pras等人开发的评分系统确定。
50例患者（52%为女性，48%为男性；平均年龄12.2±4.1岁）和39名对照者（58.9%为女性，41.1%为男性，平均年龄11.1±3.4岁）纳入研究。家族性地中海热患者在500、4000和8000Hz频率下的纯音平均值显著高于对照组。患者在6kHz、8kHz频率下的DPOAE信号值以及在8kHz频率下的SNR值显著高于对照组。将患者的听力测试和DPOAE结果与疾病严重程度评分进行比较。在2000Hz频率下，重度和中度患者组的纯音平均值显著高于轻度患者组。DPOAE信号值在1.4和2.8kHz频率下的患者严重程度评分之间存在统计学显著差异。秋水仙碱的平均治疗持续时间为5.1±3.7年。当将患者的听力测试结果与治疗时间进行比较时，在250和500Hz频率下存在显著差异。
家族性地中海热尤其在高频影响耳蜗功能。
