摘要
目的分析1名儿童（4岁）听力下降与体温变化相关的病因机制。方法对患儿连续3年的听力学检查结果进行分析并对患儿进行了连续15天、每天分七个时间点（6、8、10、12、14、16、18点）测量体温，选择每天的8点30分及16点30分进行游戏测听、声导抗、非声刺激镫骨肌反射、言语测听检查、瞬态及畸变产物耳声发射、白噪声抑制试验、听性脑干反应以及疏密波交替声测试、耳蜗电图、多频稳态诱发电位及前庭功能。观察患儿波动性听力变化与自然状态下的体温变化的相关性。并进行颞骨CT扫描、耳蜗水呈像核磁共振、呼吸睡眠检测及血液生化检查。结果患儿自1岁开始发病，病史提示在发热时听力全部丧失。在正常体温时的听力学检查符合听神经病表现：游戏测听显示平坦型听力曲线（40-60dB HL）；DPOAE正常、白噪声抑制失败；ABR未引出；鼓室导抗图A型、声刺激镫骨肌反射均未引出、非声刺激镫骨肌反射正常；多频稳态反应阈高于游戏测听听阈。每天的游戏测听显示听力在早上8点30分时明显好于下午16点30分时，但早上运动后的听力明显差于下午16点30分时。颞骨CT及耳蜗水呈像核磁共振检查未见异常。呼吸睡眠检测提示中度低氧血症，呼吸紊乱指数（RDI）为8．35，最低氧饱和度（SO2）为77％。生化检查提示高密度脂蛋白高于正常，内耳膜迷路抗体阴性。结论患儿连续3年的听力学检测结果一致证明患儿的听功能异常但耳蜗外毛细胞功能正常。在自然体温下随体温的轻微波动即可出现听力的变化，提示体温与听功能状态具有相关性。体温升高可能导致听神经脱髓鞘病变引起神经传导阻滞而出现听神经的失同步化，患儿的长期低氧血症也是诱发疾病的原因之一。
Objective To analyse and define mechanisms accounting for fluctuating hearing impairment in one child （4 years old） which had variable temperatures. Methods A series of observations were performed in the child including the temperature measure in fixed point time for every day and the audiology tests within consecutive fifteen days for the purpose of finding the relationship between the temperatures and the fluctuating heating. The audiology tests included play audiometry, tympanometry, middle ear muscle reflex thresholds and stapedius muscle reflex （ acoustic and nonacoustic）, speech and sentence comprehension, otoacoustic emission （OAE）, auditory brainstem response （ABR）, alternative click, cochlear microphonics and auditory steadystate response （ASSR） as well as vestibular function evaluation. High- resolution computed tomography（CT） scan of the temporal bone and magnetic resonance imaging was used. The respiration and sleep monitor was performed for knowing the occurrence of apnea and the information of SO2 during sleep. A review on the past three years＇ audiologic data was performed to investigate the audiologlc characteristics in a long term. Results The child suffered from the hearing disorder at the beginning of one year old and had remarkable histories of transient deafness when he became febrile. His hearing showed the special characteristics as following： the flat andiogram in play audiometry （40-60 dB HL）, normal distortion product otoacoustic emission （DPOAE） with the absent of ABR and failure of inhibition with white noise, normal A type of tympanogram and no response to the acoustic stapedius reflex while normal reflex at non - acoustic, ASSR showed higher thresholds than that of play audiometry. The play andiometry showed the better hearing and understanding at 8：30 am than at 16：30 pm, however, became worse than at afternoon when the child had an experiment of exercise in the morning. CT scan and MRI showed normal in the inner ear structure. The monitor for respiration and sleep disclosed respiration index （RDI） was 8.35 （mild anoxia） and the lowest SO2 was 77 % associated with 46. 7 sec obstructive partial apnea. Blood chemistry showed high density lipoprotein （HDL） was abnormal. The antibody of inner ear membranous labyrinth was negative. Conclusion All of the above datum suggeste a consentaneous audiologic characteristics of a disorder of auditory nerve function in the presence of normal cochlear outer hair cell functions. However, a typical different point in the child is that presented the fluctuated hearing loss due to the core body temperature mild fluctuated. The development of deafness with slight elevation of body temperature is most consistent with a demyelinating neuropathy of the auditory nerve. The long term anoxia in the patient may be another main cause of the temperature sensitive auditory neuropathy.
出处
《听力学及言语疾病杂志》
CAS
CSCD
2006年第1期21-26,共6页
Journal of Audiology and Speech Pathology
基金
国家自然科学基金面上项目(编号30470956&30370782)
关键词
听神经病
体温敏感
耳声发射
听性脑干反应
耳聋
Auditory neuropathy
Temperature - sensitive
Otoacoustic emission
Auditory brainstem response
Deafness
