听神经病谱系障碍患者临床听力学特点分析.PDF

CHIN ARCH OTOLARYNGOL HEAD NECK SURG / December 2015, Vol.22, No.12 611 DOI: 10.16066/j.1672-7002.2015.06.001 听神经病谱系障碍患者临床听力学特点分析 董瑞娟，王硕，刘博，刘冬鑫，王媛，陈静 首都医科大学附属北京同仁医院耳鼻咽喉头颈外科中心，北京市耳鼻咽喉科研究所，耳鼻咽喉头颈科学教育部重点实验室（首 都医科大学），北京　100730 [ 摘　要]　目的　总结听神经病谱系障碍（aud it or y characteristics and auditory test results of patients with neuropathy spectrum disorder ，ANSD ）患者临床特点和听 auditory neuropathy spectrum disorder and to analyse the 力学测试结果，分析其听力学特点。 　回顾性分析60 METHODS 方法 audiological characteristics. 　The onset age, 例听神经病谱系障碍患者的发病年龄，确诊年龄以及纯音 diagnosis age, pure tone audiometry, tympanometry, auditory 听阈、声导抗测试、听性脑干反应、畸变产物耳声发射的 brainstem response, distortion product otoacoustic emission 结果。 　患者中男34例（56.7%），女26例（43.3% ）；发 结果 of sixty patients with auditory neuropathy spectrum disorder 病年龄6 ～32岁，平均年龄（16.6 ±4.7 ）岁，以青少年居多； were retrospectively analyzed. RESULTS　Thirty-four ANSD平均确诊年龄（24.9 ±6.8）岁，2例患者伴有视神经萎 males and twenty-six females (56.7% versus 43.3%) were 缩。60例ANSD患者均为双侧发病，纯音听力图以峰型或碟 included. The age of onset of hearing loss ranged from 6 to 形102耳（85.0%）为主，波峰多出现在2 kHz或4 kHz处；依 32 years old (mean=16.6, SD=4.7). Most of the patients were 据听力损失程度分级，好于轻度听力损失28耳（23.3% ）， adolescence. The age of diagnosis with ANSD was 24.9 ±6.8 轻度听力损失59耳（49.2% ），中度听力损失28耳（23.3% ）， years old. Two patients were noted to have optic atrophy. All 重度听力损失4耳（3.3% ），极重度听力损失1耳（0.8% ）。 ANSD subjects were diagnosed with bilateral ANSD. The 60例患者均引出畸变产物耳声发射，115耳鼓室图A型，3耳 most common configuration of audiogram among 60 patients 为As ，2耳为Ad 。92耳（76.7% ）均未引出镫骨肌声反射，28 was peaked or saucer type (85.0%), with the peak usually 耳（23.3% ）可引出1 kHz镫骨肌声反射，但阈值均升高。