Summary

对于突发性耳聋神经康复治疗方法

Published: January 25, 2016
doi:

Summary

This article reports the development of a neuro-rehabilitation approach, “constraint-induced sound therapy (CIST)” for sudden sensorineural hearing loss. The aim of CIST is to prevent maladaptive cortical reorganization by using an enriched acoustic environment. CIST represents a safe, easy, inexpensive, and effective approach to treat sudden sensorineural hearing loss.

Abstract

突发性耳聋(SSHL)的特点是急性特发性听力损失。估计发病率为每年每10万人5-30例。 SSHL的原因,目前基本SSHL机制尚不清楚。基于几个假设,如一个循环障碍的耳蜗,病毒感染,和自身免疫性疾病,药理治疗方法已被应用到治疗SSHL患者;然而,规范的治疗,皮质类固醇治疗的疗效,目前尚在争论。暴露于激烈的声音已被证明导致对听觉系统永久损坏;然而,置身于一个中等水平的富集噪声外伤后的声环境可减少听力障碍。一些神经影像学研究最近提出,SSHL发作引起的适应不良的皮质重组人类听觉皮层和皮层重组的急性SSHL期负科尔度兴高采烈的与听力损失的回收率。这篇文章报道了一种新的神经康复治疗方法的发展SSHL,“强制性使用声音疗法(CIST)”。在CIST协议的目的是为了防止或通过使用丰富的声环境适应不良的减少皮质重组。 SSHL患者的完整耳朵的耳道插入,以激励他们积极使用受影响的耳朵,从而防止适应不良皮层重组的进展情况。受影响的耳朵通过耳机也接触到音乐,每天6小时,住院期间。 CIST的协议似乎是一种安全,方便,廉价且有效的治疗SSHL。

Introduction

突发性耳聋(SSHL),或突发性耳聋,是特点是听力1的快速流失的特发性条件。一些流行病学研究2,3报道的每年每10万人5-30例工业化国家SSHL发病率。尽管SSHL的原因和潜在的SSHL的机制已经被广泛地研究,我们对SSHL知识仍然有限。在特发性SSHL的许多潜在原因,常见的假设包括循环障碍4,病毒感染5-7,和自身免疫性疾病8,9。虽然糖皮质激素治疗10提出了一种基于这些假设,是目前最常用的治疗,这一标准疗法的有效性还存在争议3,11-14。因此,创新SSHL治疗策略,通过不同的视角动机,强烈期望。

SSHL影响神经活动不仅在耳蜗,而且在听觉皮层。几个神经影像学研究15-17表明SSHL诱导皮层塑性于人听觉皮层中其发病几天。此外,皮质重组的听觉对侧半球dominancy的损失为代表的程度诱发领域出现了负回收率从听力损失18相关。皮质可塑性诱导SSHL的发作可以被认为是适应不良为受影响的耳朵的听觉能力。因此,预防与SSHL有关此适应不良的皮质重组可能代表一种新的治疗策略。

本文提出了一种神经康复方法,防止“学习不使用”皮质的变化,以防止或减少适应不良的皮质重组。例如,在运动功能障碍的情况下,得知不使用一个limbis一种现象,其中运动最初抑制因不良反应和任何活动的失败尝试与患肢,最终导致行为和对应的神经活动19,20的抑制。了解到非使用不会出现在限于运动功能障碍,但也可能涉及感官残疾21。 SSHL患者主要使用并注意完整的耳朵听。出现这种监听行为,以增加神经活动相对应的完整的耳朵,在同一时间,减少受影响的耳蜗及听觉皮层22之间的神经连接。为了防止这种适应不良皮质变化引起的'不使用“时,它似乎是有益的SSHL患者以执行”约束诱导声音疗法(CIST)'23,其激励参与者经由患耳集中听音乐并要听觉注意患耳( 图1)。相比传统的药物治疗,该CIST据称是SSHL一个安全,方便,价廉,有效的治疗方法。

Protocol

伦理声明:程序已经批准医学院的明斯特大学的道德委员会,大阪大学附属医院的伦理委员会,以及由大阪Rosai医院的伦理委员会。 1.准备设备获得耳模,便携式音乐播放器,密闭式耳机,均衡器,和耳机放大器,如图2。 准备不同类型的音乐(流行,摇滚,古典等)的便携式音乐播放器的图书馆。 获得不同类型的音乐光盘。 从CD用声音编辑应用程序(例如,Adobe Audition的3.0或类似)将音乐传输到计算机上。 放置在计算机的CD-ROM驱动器的音频CD。选择文件>从CD中提取音频。您可以选择所有所选驱动器支持的提取速度,最大速度选项。 确认日在音乐讲解了如何使用声音编辑应用程序宽的频率范围( 即 125-8,000赫兹或更宽)( 例如,Adobe Audition的3.0或类似)。 选择所有的波形,选择Edit>选择整个波。选择Window>频率分析。视图频率沿着水平轴,​​以及振幅沿着在频率分析面板垂直轴。 通过连接电缆从计算机音乐文件传输到便携式音乐播放器。 连接使用USB连接线便携式音乐播放器和电脑。保存音乐文件的便携式音乐播放器为.mp3文件的[MUSIC]文件夹中的192 kbps的码率。 2.参加测量听力阈值水平(空气和骨传导)双耳使用5 dB的步长中 ​​根据修正的Hughson-韦斯特莱克程序24,由纯音audiome的装置之三。 设置一个听力计到1000赫兹的频率控制,并设置完整耳被测试到耳机。 设置强度级别到50分贝。 按声演示按钮1秒给患者1000 Hz的音调。等待他们的回应( 例如 ,通过按下按钮或举手)。 如果患者的语气回应,在步骤2.1.3中描述的方式呈现10dB的柔和色调。如果患者不回应,则转到步骤2.1.6。 重复步骤2.1.4,直到患者不能响应任何更长的时间。 呈现出5分贝更大声,等待病人作出回应。 重复步骤2.1.6如果患者不回应。 注意柔软的强度水平的患者响应。 重复步骤2.1.4-2.1.8直到相同强度水平应注意两次或三次。这是在1000赫兹既定听力阈值。 测试频率重复步骤2.1.2-2.1.92000,4000和8000赫兹。 重新测试听觉阈1000赫兹,并确认在1000赫兹在第一和第二阈值内5 dB的同意。 重复步骤2.1.2-2.1.9测试频率500,250和125赫兹。 将受影响的耳朵进行测试,耳机和重复步骤2.1.2-2.1.12。使用平稳方法25,如果左和右听力阈值之间的差等于或超过25分贝的较低频率(125,250,和500赫兹),或等于或超过经由耳机呈现一个适当的窄带掩蔽噪声的未经测试的耳朵40个分贝或以上千赫兹。 测量骨导听觉阈用与步骤2.1.1-2.1.13以类似的方式掩蔽噪声。为了测量骨导听力,使用骨传导振动器,而不是耳机。为了避免串扰施加适当的窄带噪声掩蔽25向未测试耳。 绘制听取阈值水平ñ听力图的形式。一个SSHL患者的示例性听力图示于图3。 检查的标准,参与CIST。 检查自SSHL发病的天数少于基于自我报告5。 确认耳的听力水平差均跨越500,1000,和2000个赫兹小于50基于DB在步骤2.1中获得的气导纯音测听。 注意:如果在图3所示的听力图,听力水平差是(40 + 40 + 45)/ 3 – (5 + 0 + 5)/ 3 =38.3分贝。 确认患者可以舒适地收听音乐与他们患耳。 确认患者急性单侧听力损失(以自我报告)和中至少为30 dB急性单侧耳聋特发性疾病的困扰在三个或多个相邻的频率在步骤2中得到的纯音听力图10.1。例如,如果在图3所示的听力图,听力水平和高于250赫兹的右耳指示至少为30 dB感觉神经性听力损失。 确认患者有SSHL的基础上自我报告和病历既往史。同时确认其具有基于自我报告和医学历史上没有神经或精神并发症。 考虑鉴别诊断并排除谁收到其他诊断,如美尼尔氏病26,头部外伤,自身免疫性内耳病8,9患者,柯根综合征27,28,遗传性疾病29,耳毒性药物30,蜗后障碍与前庭神经鞘瘤31 ,听神经病32,或中风33。 3.启动CIST 注:建议住院用于patients的安全。由于患者从因堵塞和音乐环境声音监听密封固有的CIST过程中,预计事故在他们的日常生活中的风险增加。 塞的使用耳模患者的未受影响的耳朵的外部管。紧紧包耳模入外管,以确保存在于外耳道没有空间。 如果病人感觉疼痛或如果密封是不完整的,及时清除耳模,然后重新插入。 询问患者从库中选择一个愉快的音乐类型。 注:他们还允许用户听自己的音乐,如果后者涵盖了足够宽的频率范围(125-8,000赫兹或更宽)。 询问病人穿密闭型耳机。呈现所述选定的音乐只对受影响的耳朵如图1。 使用均衡器来增加或减少每个FRE的音量昆西根据“半增益规则”,其中规定,增益级别等同于听的耳朵之间的高度差在每个频率的一半。 例如,如果耳的听力水平差分别为X 125,X 250,X 500,X 1000,X 2000,X 4000和X 8000在125,250 500,1000,2000,4000,和8000赫兹,设置均衡器在1000赫兹到“×1000/2 – (X + 125×250 + X + 500×1000 + X 2000 + X 4000 + X 8000)/ 14”。 注意:如果在图3所示的听力图的,均衡器设置为125,250,500,1000,2000,4000,和8000赫兹为-5,-3,0,2,2,5,并0dB时,分别。 向患者本身,使得音乐声音如正执行的声级和均衡器设置微调atural和舒适。如果音乐播放器的内部均衡器是不够有效,使用硬件均衡器, 如图2。 询问患者是否他们认为音乐与受影响的耳朵,以便确认交叉听力34不 ​​会发生。排除谁听音乐的插入未受影响的耳朵由于交听证患者。 4.程序启动后CIST 指导他们被允许接受标准治疗SSHL如除了CIST协议糖皮质激素的患者。 有患者听音乐,每天6小时,使用密闭式耳机(图1)在受影响的耳朵。指示他们花费听音乐的时候可分段患者。另外,让患者来执行其他任务(包括看书和上网)所花的时间清单当然期间宁音乐。 指导患者整天使用的耳模,直到他们离开医院。 测量患耳的气导的阈值水平以相同的方式每两天如在步骤2.1。调整音量和均衡器设置中提到的步骤3.5和3.6。 当病人出院和当他们返回作为门诊患者,测量以相同的方式的听力阈值的水平,如步骤2.1。 5.停止CIST的取出耳模,如果病人报告关于插完整的耳朵(如耳鸣或疼痛)不适。 测量听力在原封耳以同样的方式的阈值水平,如步骤2.1。 停止CIST程序,如果听力水平跨越500平均,1000,2000赫兹获得步骤4.4和/或5.2恶化超过5分贝相比,那些在步骤2.1获得的。例如,在情况下,听力图的图 3中所示</stro纳克>中,听力水平平均跨越500,1000,2000赫兹是(40 + 40 + 45)/ 3 =41.7分贝在受影响的耳朵和(5 + 0 + 5)/ 3 = 3.3分贝的完整耳。

Representative Results

第二十二条谁匹配在协议中所述的标准,谁SSHL住院病人愿意接受CIST被分配到目标群体,这行CIST除了标准的糖皮质激素治疗(CIST + SCT组)23。对照组包括谁只接受标准的糖皮质激素治疗(SCT组)31 SSHL住院病人。所有与会者都充分了解有关的研究,并签署了知情按照批准的医学院的明斯特大学的道德委员会,大阪大学附属医院的伦理委员会,并通过大阪Rosai医院的伦理委员会的程序同意。按照赫尔辛基宣言进行这项研究。在每两组(SCT + CIST与SCT)的参加者有相似年龄和时间延迟SSHL的发生和初始听力图之间。 <p class="jove_content" fo:keep-together.within-page ="“1”">所有参与者的听阈测定(一)治疗前(1日考试),(ii)离医院(2 次检查排液:1 和第2次测量之间的时间间隔(平均值±标准差(SCT + CIST:9.41±3.14天,SCT:10.42±3.18天),并再次(三)几个月后(3 次检查:平均值±标准差(SCT + CIST:63.45±28.56天,SCT:84.64±38.68天))使用纯音听力计。 如图4所示,处理前(1次测量),无显著差异,观察在听力组之间的阈值,在所有测量的频率(125,250,500,1000,2000,4000,和8000赫兹)在受影响的耳朵。治疗后(2 次和第3 次考试),听力受影响的耳朵都frequ有所改善encies在两个对SCT + CIST和SCT基( 图4)。在所有频率的听力阈值水平分别平均为每个耳朵和耳朵之间再听到水平差异计算( 图5)。受影响的和完整的耳的听力水平的差异归因于SSHL。耳朵之间的计算出的听力水平差异的治疗在第 1次检查(图5)之前,组之间类似。然而,这两个群体之间,观察在出院显著差异( 第二次检查:P <0.05(邦费罗尼校正)),并在几个月后(3 次检查:P <0.001(邦费罗尼校正))。关于在原封耳听力水平,平均的阈值并没有显著组之间的任何中任三查的频率的不同。该结果表明堵塞第Ë耳道并未以对完好耳有负面影响。在22名学员谁接受CIST,并没有观察到明显的副作用。 图约束引起的声音疗法(CIST)1示意图。完整耳朵的耳道插入为了激励患耳的使用。音乐monaurally呈现给经由封闭型耳机患耳;相应于完整的耳朵另一个通道保持沉默(这图是改编自冈本等人 。23:绘制礼貌洛萨Lagemann的)。 图2.连接和设备在CIST使用。所述的便携式音乐播放器,均衡器,耳机放大器,和密闭式耳机依次连接。均衡器和耳机放大器仅用于当便携式音乐播放器的数字均衡器是不够有效。 请点击此处查看该图的放大版本。 图3.一个SSHL患者的示范性听力这个数字代表的听力水平(范围:125〜8000赫兹;一个倍频程步)在受影响的耳朵(气导:空心圆;骨传导:左括号)和完整耳(气导:十字架;骨传导:右括号)治疗前的SSHL病人。 请点击此处查看该图的放大版本。 e_content“FO:保持together.within页=”1“> 图4.时间的患耳平均听力水平当然本图显示的平均听力图(范围:125〜8000赫兹;一个倍频程步)谁接受约束引起的声音疗法除了接受标准的参与者受影响的耳朵糖皮质激素治疗(CIST + SCT:空心方块),或在那些谁只接受标准的糖皮质激素治疗(SCT:实心方块)。 第一届, 第二届和第三届听力考试进行(一)治疗前,(二)在出院;及(iii)几个月后,分别。误差线表示95%的置信区间(此图已经被修改Okamoto等23)。 请点击此处查看该图的放大版本。URE。 图5.平均听力的耳朵之间的等级差听力受影响的和完整的两耳之间的阈值差异在所有测量频率的平均值在第1(左:治疗前),2号(中心:出院时)和第三(右:几个月后),纯音测听检查。开放和实心方块表示约束引起的声音疗法+标准的糖皮质激素治疗组(CIST + SCT)和标准糖皮质激素治疗治疗组(SCT),分别。误差条表示95%置信区间(此图已经被修改Okamoto等23)。 请点击此处查看该图的放大版本。

Discussion

This article describes a behavioral treatment approach for SSHL. The CIST protocol merely requires the use of easily available devices. A comparison of the treatment effects of CIST + SCT with those of SCT alone revealed significantly better hearing improvements with the CIST + SCT treatment. No apparent side effects were associated with the CIST protocol. The cost of CIST is markedly lower than other newly suggested treatments (including a stellate ganglion block and hyperbaric oxygen therapy). The CIST protocol represents an effective, inexpensive, easy, and safe treatment for SSHL; however, several important points must be considered. One point is the prevention of acoustic trauma. Although the maximal volume settings of recent commercial portable music players are limited to remain below the harmful levels, the sound level needs to be observed when a large adjustment is made to the equalizer settings or when a headphone amplifier is used. The second point is ‘cross hearing’. The purpose of the CIST procedure is to enable SSHL patients to actively use their affected ear for listening. When patients develop severe hearing loss, they may still hear sounds with the intact ear despite plugging. The third point is ‘accidents’. In the case of traditional pharmacotherapy, the intact ear is not plugged, and thus SSHL patients can pick up environmental sounds via this intact ear. On the other hand, plugging and music listening inherent to the CIST protocol seal patients from environmental sounds, and thus the accident hazard is likely increased. Therefore, the CIST protocol should be conducted in a safe environment.

Unlike pharmacotherapy, the CIST protocol has no limitations concerning medications, allergies, or other diseases such as diabetes, hypertension, and hyperlipidemia. However, the limitation of this protocol is that it is intended for patients who are able to listen to sounds with their affected ear. As such, the CIST protocol is not suited for SSHL patients with severe hearing loss. This protocol is applied to acute stage SSHL (days since SSHL onset ≤ 5) because previous studies reported that a shorter time delay between the onset of SSHL and the start of treatment led to better hearing recovery2. It remains elusive whether there is a time restriction for starting the CIST protocol. Moreover, the optimum duration of the CIST protocol and the total music listening time require further investigation.

This protocol utilizes the corticosteroid therapy in addition to CIST. At present, corticosteroids are the most commonly used treatment for SSHL, and therefore it is ethically not feasible to stop this treatment. However, recent triple-blinded SSHL treatment studies14 revealed that the recovery of non-treated patients was similar to that of patients who had received corticosteroids. Even though one cannot exclude the possibility that the combination of CIST + SCT led to the results obtained, it appears reasonable to assume that CIST alone will be beneficial, especially for patients with diseases that are worsened by corticosteroids such as infections, diabetes, and glaucoma.

In this protocol, patients are hospitalized in order to avoid accidents. However, SSHL patients often cannot be hospitalized because of work, family, and financial reasons. The utilization of a hearing aid may allow these outpatients to perform the CIST protocol. SSHL patients who wear a hearing aid in their affected ear are ‘functionally’ exposed to an enriched acoustic environment, are not in danger to be exposed to harmful sound levels, and are able to detect warning sound signals. However, the daily monitoring of hearing threshold levels and appropriate adjustments to the hearing aid settings would be necessary since hearing capability of SSHL patients can improve rapidly. Generally, the CIST protocol will not disturb other treatment approaches and may actually manifest a synergic effect when used in combination with other SSHL treatment strategies.

In the present study, a limited number of SSHL patients underwent the CIST protocol, and the participants were not randomized to the different treatment conditions. Thus, a randomized controlled study including a larger number of patients should be executed in the future. Moreover, the effectiveness of the CIST protocol should be investigated in patients with different types of hearing loss. Further, in the present study, all participants who performed the CIST protocol also received the corticosteroid therapy. Therefore, it remains unresolved whether the CIST protocol alone can improve the hearing ability of SSHL patients. It would be valuable to perform a randomized controlled study in which SSHL patients, for whom corticosteroids may cause severe side effects (such asinfectious diseases or diabetes mellitus), would either merely receive the CIST protocol or merely a standard corticosteroid therapy. Notably, in our previous report23, the effects of the CIST protocol within the human auditory cortex were examined by means of magnetoencephalography35. The results showed that the CIST protocol could have prevented maladaptive cortical reorganization in the human primary and non-primary auditory cortices. Of course, it is difficult to conduct neuroimaging studies in practices and hospitals; however, speech test, hearing in noise test36, and tinnitus related examinations37 may contribute to reveal the functional plasticity in the central auditory system induced by the CIST protocol. Eventually, even though the CIST protocol is in a very early stage in development, and although further investigations are needed, the CIST protocol as an effective, inexpensive, and safe treatment option can complement the corticosteroid therapy, which may induce severe and potentially lethal side effects.

Disclosures

The authors have nothing to disclose.

Acknowledgements

We thank Yoshimasa Sekiya for demonstrating the protocol technique on film. This work was supported by the ”Japan Society for the Promotion of Science for Young Scientists (26861426)” and by the “COI STREAM (Center of Innovation Science and Technology based Radical Innovation and Entrepreneurship Program)”.

Materials

Ear mold RION Co.Ltd, Tokyo, Japan EM-59
portable music player Sony Corporation, Tokyo, Japan NW-S775
headphone SENNHEISER Electronic GmbH & Co. KG, Hannover, Germany HD280pro
equalizer Roland Corporation, Hamamatsu, Japan GE-7
headphone amplifier FiiO Electronics Technology Co. Ltd, Guangzhou, China E11
sound editing application Adobe Systems Inc., CA, USA Audition 3.0

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Cite This Article
Sekiya, K., Fukushima, M., Teismann, H., Lagemann, L., Kakigi, R., Pantev, C., Okamoto, H. Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss. J. Vis. Exp. (107), e53264, doi:10.3791/53264 (2016).

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