目的探讨大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患儿前庭水管开口口径及听力损失程度对声诱发短潜伏期负反应(acoustically evoked short latency negative response,ASNR)引出率的影响。方法回顾性分析26例(52耳...目的探讨大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患儿前庭水管开口口径及听力损失程度对声诱发短潜伏期负反应(acoustically evoked short latency negative response,ASNR)引出率的影响。方法回顾性分析26例(52耳)双侧单纯LVAS患儿的临床资料,在颞骨CT横断位上测量其前庭水管开口口径,根据气导短声听性脑干反应(ABR)反应阈将患者分为50~70 dB nHL组(18耳)、71~90 dB nHL组(15耳)和>90 dB nHL组(19耳),分析ASNR引出与未引出者前庭水管开口口径及不同听力损失程度组ASNR引出率的差异。结果26例(52耳)患者中ASNR总体引出率为48.07%(25/52耳);ASNR引出组(25耳)与未引出组(27耳)前庭水管开口口径的大小分别为6.9±1.4 mm和7.3±2.6 mm,差异无统计学意义(P>0.05);ABR反应阈为50~70 dB nHL组、71~90 dB nHL组和>90 dB nHL组的ASNR引出率分别为33.33%(6/181)、46.67%(12/19)及63.16%(12/19),差异无统计学意义(P>0.05)。结论本组LVAS患者ASNR引出率为48.07%,ASNR引出不受前庭水管开口口径及听力损失程度的影响。展开更多
目的探究大前庭水管综合征(LVAS)患者MRI不同内淋巴囊分型对声诱发短潜伏期负反应(ASNR)的影响。方法回顾性分析26例(52耳)经影像学及临床综合诊断为LVAS患者的MRI T2WI及ABR资料,根据MRI内淋巴囊高低信号分布差异,分为Ⅰ型组8耳,Ⅱ型...目的探究大前庭水管综合征(LVAS)患者MRI不同内淋巴囊分型对声诱发短潜伏期负反应(ASNR)的影响。方法回顾性分析26例(52耳)经影像学及临床综合诊断为LVAS患者的MRI T2WI及ABR资料,根据MRI内淋巴囊高低信号分布差异,分为Ⅰ型组8耳,Ⅱ型组12耳,Ⅲ型组32耳。并以经颞骨CT及内听道MRI确定无内耳畸形的重度-极重度感音神经性听力损失患者21例(42耳)作为对照组,比较各组ASNR引出率、反应阈及潜伏期。结果①LVAS组和对照组ASNR引出率分别为44.2%(23/52)和9.5%(4/42),差异有统计学意义(P<0.05);ASNR平均反应阈分别为91.57±7.25和89.25±6.99 dB nHL,差异无统计学意义(P>0.05);平均潜伏期分别为3.36±0.35和3.26±0.59 ms,差异无统计学意义(P>0.05)。②LVAS患者Ⅰ型组、Ⅱ型组和Ⅲ型组的ASNR引出率分别为50.0%(4/8)、33.3%(4/12)和46.7%(15/32),差异无统计学意义(P>0.05);ASNR平均反应阈分别为84.25±9.43、86.75±8.30和94.80±3.95 dB nHL,Ⅰ型组与Ⅲ型组之间差异有统计学意义(P<0.05);平均潜伏期分别为3.07±0.14、3.43±0.13和3.23±0.29 ms,差异无统计学意义(P>0.05)。结论LVAS患者ASNR引出率不受MRI内淋巴囊分型的影响,且较无内耳畸形的重度-极重度感音神经性聋患者更高。展开更多
Backgroud Large vestibular aqueduct syndrome （LVAS） is a major cause of hearing loss in childhood. This study aimed at measuring external aperture of enlargement of the vestibular aqueduct （EVA） and analyzing rela...Backgroud Large vestibular aqueduct syndrome （LVAS） is a major cause of hearing loss in childhood. This study aimed at measuring external aperture of enlargement of the vestibular aqueduct （EVA） and analyzing relationship between the size of external aperture and hearing loss. Methods Diagnostic criteria of LVAS were based on hearing loss and CT images. CT images of temporal bone of 100 LVAS patients were collected and 60 control subjects were reviewed retrospectively in the past 10 years. A battery of audiometric and vestibular function tests were performed. The width of the vestibular aqueduct （VA） was measured on axial CT images of the temporal bone. Results One hundred patients （65 men, 35 women） were diagnosed as having the isolated EVA. Hearing loss mostly occurred in early childhood. The diagnosis age of LVAS was 7.7 years on average. The causes of hearing loss could not be confirmed by initial consult. Typically, audiometric curve is the high-frequency down-sloping configuration. 92% of the cases had severe or profound sonsorineural hearing loss （SNHL）. The mean size of the external aperture was （7.5±1.2） mm in present LVAS. Statistical analysis showed that the degree of hearing loss is unrelated to the width of VA. Conclusions LVAS is a distinct clinical entity characterized by fluctuating, progressive SNHL. The degree of hearing loss is unrelated to the size of external aperture of VA. The protective management and hearing aid have become the main therapies. The cochlear implantation might be performed if the hearing loss affected learning at school.展开更多
To the Editor:Drug-induced immune hemolytic anemia (DIIHA)is a rare but severe condition.Ceftriaxone,a third-generation cephalosporin with a wide antibacterial spectrum and effective antibacterial action,is one of the...To the Editor:Drug-induced immune hemolytic anemia (DIIHA)is a rare but severe condition.Ceftriaxone,a third-generation cephalosporin with a wide antibacterial spectrum and effective antibacterial action,is one of the most common drugs causing DIIHA.^[1]Ceftriaxoneinduced immune hemolytic anemia (CIIHA)is a potentially fatal complication.^[2]Most of the patients were children.The reported fatality rate of CIIHA was as high as 40%.展开更多
文摘目的探讨大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患儿前庭水管开口口径及听力损失程度对声诱发短潜伏期负反应(acoustically evoked short latency negative response,ASNR)引出率的影响。方法回顾性分析26例(52耳)双侧单纯LVAS患儿的临床资料,在颞骨CT横断位上测量其前庭水管开口口径,根据气导短声听性脑干反应(ABR)反应阈将患者分为50~70 dB nHL组(18耳)、71~90 dB nHL组(15耳)和>90 dB nHL组(19耳),分析ASNR引出与未引出者前庭水管开口口径及不同听力损失程度组ASNR引出率的差异。结果26例(52耳)患者中ASNR总体引出率为48.07%(25/52耳);ASNR引出组(25耳)与未引出组(27耳)前庭水管开口口径的大小分别为6.9±1.4 mm和7.3±2.6 mm,差异无统计学意义(P>0.05);ABR反应阈为50~70 dB nHL组、71~90 dB nHL组和>90 dB nHL组的ASNR引出率分别为33.33%(6/181)、46.67%(12/19)及63.16%(12/19),差异无统计学意义(P>0.05)。结论本组LVAS患者ASNR引出率为48.07%,ASNR引出不受前庭水管开口口径及听力损失程度的影响。
文摘目的探究大前庭水管综合征(LVAS)患者MRI不同内淋巴囊分型对声诱发短潜伏期负反应(ASNR)的影响。方法回顾性分析26例(52耳)经影像学及临床综合诊断为LVAS患者的MRI T2WI及ABR资料,根据MRI内淋巴囊高低信号分布差异,分为Ⅰ型组8耳,Ⅱ型组12耳,Ⅲ型组32耳。并以经颞骨CT及内听道MRI确定无内耳畸形的重度-极重度感音神经性听力损失患者21例(42耳)作为对照组,比较各组ASNR引出率、反应阈及潜伏期。结果①LVAS组和对照组ASNR引出率分别为44.2%(23/52)和9.5%(4/42),差异有统计学意义(P<0.05);ASNR平均反应阈分别为91.57±7.25和89.25±6.99 dB nHL,差异无统计学意义(P>0.05);平均潜伏期分别为3.36±0.35和3.26±0.59 ms,差异无统计学意义(P>0.05)。②LVAS患者Ⅰ型组、Ⅱ型组和Ⅲ型组的ASNR引出率分别为50.0%(4/8)、33.3%(4/12)和46.7%(15/32),差异无统计学意义(P>0.05);ASNR平均反应阈分别为84.25±9.43、86.75±8.30和94.80±3.95 dB nHL,Ⅰ型组与Ⅲ型组之间差异有统计学意义(P<0.05);平均潜伏期分别为3.07±0.14、3.43±0.13和3.23±0.29 ms,差异无统计学意义(P>0.05)。结论LVAS患者ASNR引出率不受MRI内淋巴囊分型的影响,且较无内耳畸形的重度-极重度感音神经性聋患者更高。
文摘Backgroud Large vestibular aqueduct syndrome （LVAS） is a major cause of hearing loss in childhood. This study aimed at measuring external aperture of enlargement of the vestibular aqueduct （EVA） and analyzing relationship between the size of external aperture and hearing loss. Methods Diagnostic criteria of LVAS were based on hearing loss and CT images. CT images of temporal bone of 100 LVAS patients were collected and 60 control subjects were reviewed retrospectively in the past 10 years. A battery of audiometric and vestibular function tests were performed. The width of the vestibular aqueduct （VA） was measured on axial CT images of the temporal bone. Results One hundred patients （65 men, 35 women） were diagnosed as having the isolated EVA. Hearing loss mostly occurred in early childhood. The diagnosis age of LVAS was 7.7 years on average. The causes of hearing loss could not be confirmed by initial consult. Typically, audiometric curve is the high-frequency down-sloping configuration. 92% of the cases had severe or profound sonsorineural hearing loss （SNHL）. The mean size of the external aperture was （7.5±1.2） mm in present LVAS. Statistical analysis showed that the degree of hearing loss is unrelated to the width of VA. Conclusions LVAS is a distinct clinical entity characterized by fluctuating, progressive SNHL. The degree of hearing loss is unrelated to the size of external aperture of VA. The protective management and hearing aid have become the main therapies. The cochlear implantation might be performed if the hearing loss affected learning at school.
文摘To the Editor:Drug-induced immune hemolytic anemia (DIIHA)is a rare but severe condition.Ceftriaxone,a third-generation cephalosporin with a wide antibacterial spectrum and effective antibacterial action,is one of the most common drugs causing DIIHA.^[1]Ceftriaxoneinduced immune hemolytic anemia (CIIHA)is a potentially fatal complication.^[2]Most of the patients were children.The reported fatality rate of CIIHA was as high as 40%.
