Transient deafness due to temperature-sensitive auditory neuropathy
Transient deafness due to temperature-sensitive auditory neuropathy
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DOI：
10.1097/00003446-199806000-00001
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发表时间：
1998-06-01
期刊：
影响因子：
3.7
通讯作者：
Michalewski, HJ
中科院分区：
文献类型：
--
作者：
Starr, A;Sininger, Y;Michalewski, HJ
目的：确定三名儿童（两名兄弟姐妹，年龄分别为 3 岁和 6 岁，以及一名无关儿童，年龄 15 岁）发热时短暂性耳聋的机制。设计：听力测试（纯音听力测定、言语和句子理解）、鼓室导抗测试、中耳肌肉反射阈值、耳声发射 (OAE) 和电生理学方法（听觉脑干反应 [ABR]、感觉诱发）结果：当儿童不发热时，ABR 异常，IV-V 波严重延迟，I-III 波缺失。其中一名儿童在发烧时进行的 ABR 检测显示没有 ABR 成分。使用 OAE 测量耳蜗受体功能在发热和非发热状态下均正常。三个孩子都存在耳蜗微音电位，两个孩子可能存在求和电位。当不发烧时，15 岁的孩子所有频率的阈值都有轻微升高，而两个兄弟姐妹的低频阈值也有轻微升高。安静时的言语理解能力正常，但噪音时的言语理解能力受损。其中一位兄弟姐妹在发烧时接受测试，其纯音阈值大幅升高（>80 dB），并且言语理解能力缺失。发热和不发热时，中耳肌肉和橄榄耳蜗束的声反射消失。所有儿童均未发现其他周围神经或脑神经异常。其中一名儿童的正中神经感觉神经动作电位在将手加热至 39 摄氏度时未显示异常。结论：这些儿童患有听神经病，表现为在耳蜗外毛细胞功能正常的情况下听神经功能紊乱。当他们的核心体温升高时，他们的听觉神经传导阻滞很可能是由于听觉神经脱髓鞘疾病所致。两个受影响的兄弟姐妹的听觉神经病很可能作为隐性疾病遗传。
Objective: To define mechanisms accounting for transient deafness in three children (two siblings, ages 3 and 6, and an unrelated child, age 15) when they become febrile.Design: Audiometric tests (pure-tone audiometry, speech and sentence comprehension), tympanometry, middle ear muscle reflex thresholds, otoacoustic emissions (OAEs), and electrophysiological methods (auditory brain stem responses [ABRs], sensory evoked potentials, peripheral nerve conduction velocities) were used to test the children when they mere afebrile and febrile.Results: ABRs, when afebrile, were abnormal with a profound delay of the IV-V and absence of waves I-III. The ABR in one of the children, tested when febrile, showed no ABR components. Measures of cochlear receptor function using OAEs were normal in both febrile and afebrile states. Cochlear microphonic potentials were present in the three children, and a summating potential was likely present in two. When afebrile, there was a mild threshold elevation for all frequencies in the 15-yr-old and a mild elevation of thresholds for just low frequencies in the two siblings. Speech comprehension in quiet was normal but impaired in noise. One of the siblings tested when febrile had a profound elevation (>80 dB) of pure-tone thresholds and speech comprehension was absent. Acoustic reflexes subserving middle ear muscles and olivocochlear bundle were absent when febrile and when afebrile. No other peripheral or cranial nerve abnormalities were found in any of the children. Sensory nerve action potentials from median nerve in one of the children showed no abnormalities on warming of the hand to 39 degrees C.Conclusion: These children have an auditory neuropathy manifested by a disorder of auditory nerve function in the presence of normal cochlear outer hair cell functions. They develop a conduction block of the auditory nerves when their core body temperature rises due, most likely, to a demyelinating disorder of the auditory nerve. The auditory neuropathy in the two affected siblings is likely to be inherited as a recessive disorder.