糖皮质激素在大前庭水管综合征患儿不同频率听力损失中的临床效果观察

目的探讨糖皮质激素（glucocorticoid,GC）改善大前庭水管综合征（large vestibular aquduct syndrome,LVAS）不同频率听力的效果。方法 2008-01/2013-12月作者医院收治的22例（44耳）LVAS患儿,给予口服强的松、金纳多、维生素B1、B6、甲钴胺治疗;对比治疗前后听力损伤程度及各频率听阈。结果患儿治疗前中重度聋、重度聋、极重度聋分别占9.09%、25.00%、65.91%,治疗2周后中度聋、中重度聋、重度聋、极重度聋为2.27%、15.91%、36.36%、45.45%。治疗前后听力损失程度无明显差异（P〉0.05）。患儿治疗前0.25、0.5、1、2、4 kHz纯音听阈分别为（61.53±6.24）、（65.32±8.34）、（69.23±8.48）、（84.83±7.89）、（86.58±7.84）dB HL,治疗2周后0.25、0.5、1、2、4 kHz纯音听阈分别为（43.32±7.35）、（52.36±7.53）、（66.64±7.39）、（79.34±8.94）、（85.83±7.96）dB HL。患儿经治疗后对0.25、0.5 kHz纯音的听阈明显提高（P〈0.05）;而对1、2、4 kHz纯音的听阈则无明显改善（P〉0.05）。结论口服GC配合神经营养剂能够提高LVAS患儿的低频听力,但对其高频听力无改善效果。

Clinical Efficacy of Glucocorticoid on Patients with Large Vestibular Aqueduct Syndrome

Objective To investigate the clinical efficacy of glucocorticoid on hearing loss of different frequencies in patients with large vestibular aqueduct syndrome.Methods Twenty-two patients with large vestibular aqueduct syndrome in our hospital from Jan 2008 to Dec 2013 were treated with prednisone,ginaton,vitamin B1,vitamin B6,and mecobalamin.The degree of hearing loss and pure toneaudiometry of different frequencies were observed.Results Before treatment,there were 9.09% moderate-to-severe deafness,25.00% severe deafness and 65.91% profoundest deafress.After treatment of two weeks,there were 2.27% moderate deafness,15.91% moderate-to-severe deafness,36.36% severe deafness and 45.45% profoundest.There was no difference in degree of hearing loss before and after treatment（P〉0.05）.The patients’ pure toneaudiometry of 0.25,0.5,1,2,4 kHz were（61.53±6.24）、（65.32±8.34）、（69.23±8.48）、（84.83±7.89）、（86.58±7.84）dB HL before treatment and（43.32±7.35）、（52.36±7.53）、（66.64±7.39）、（79.34±8.94）、（85.83±7.96）dB HL respectively after treatment.The patients’ pure toneaudiometry of 0.25、0.5 kHz increased obviously after treatment（P〈0.05）;while pure toneaudiometry of 1,2,4 kHz did not improve（P〉0.05）.Conclusion Glucocorticoid can increase pure toneaudiometry of low frequency of patients with large vestibular aqueduct syndrome,but can’t improve hearing of high frequency.