韩国组合hotshot:为什么有些人会听到自己眼球滚动的声音？

这听起来像艾伦·坡的恐怖故事。一个人因听到奇怪的声音而激动，却发现那是从他自身发出的——他的心脏、他的脉搏、他眼眶中眼球的每一次运动。上半规管裂综合征（SCDS）是由覆盖内耳的某部分骨头上出现漏洞而引起。这项病症非常折磨人——听力效果扭曲以及平衡感受损。                  

人类的耳朵由3部分构成。外耳包括耳垂和外耳道——用于把声波引入鼓膜，使之振动鼓膜。中耳将振动鼓膜的声波转化成对耳蜗——内耳的接听结构——的机械振动。在耳朵里，也存在一个由三部分充液半规管的系统——在上下、前后、水平方向上向大脑反馈关于头部角度运动的信息。当保护上半规管的部分骨头缺失时，SCDS就会发生。   

鉴于难以知晓SCDS的流行情况具体如何，几项研究案例显示病症对病患的日常生活影响严重。Stephen Mabbutt，一位罹患SCDS长达6年的57岁英国人，描述“每当眼球在眼眶中运动的时候，就听到他们像砂纸一样磨来磨去。”本月早前，在将覆盖着上半规管的引脚大小的骨头缺口填补的手术成功之后，他重新回到了工作岗位。Toby Spencer，一位来自Maine, Skowhegan的41岁IT专家，描述了跟Mabbutt一样的症状，同时还伴有让他感觉失衡的吵杂噪音。Spencer在四月份进行手术解决了这个问题。[阅读更多关于内耳疾病及治疗方法: "装仿生耳，重获平衡感"]  

Musician Adrian McLeish suffered from the disorder for more than two decades, even having to give up his livelihood playing the French horn, before in 2008 seeing Lloyd Minor, provost and senior vice president for academic affairs at Johns Hopkins University in Baltimore. McLeish sought out Minor, a professor of otolaryngology—head and neck surgery—because he is the lead author on the study in 1998 providing the first description of SCDS in Archives of Otolaryngology—Head & Neck Surgery.

音乐家Adrian McLeish罹患这项疾病超过20年，在2008见到Lloyd Minor（位于Baltimore的Johns Hopkins University学术事务处高级副主任和教务长）之前，不得不暂停他吹奏法国号的职业生涯。McLeish找到了Minor，这位精于头部颈部手术的耳鼻喉科学专家，同时也是1998年首次在《耳鼻喉科学档案——头颈部手术》中描述SCDS的论文第一作者。  

McLeish described (video) not only hearing a buzzing, distorted version of his own voice but also being able to feel the vibrations of the sound whenever he spoke. Essentially, the missing piece of bone in McLeish's left ear was allowing the superior semicircular canal to respond to sound and pressure in abnormal ways. It was enough for the canal to act as an amplifier for every sound in his body. McLeish's horror story came to a happy ending soon after meeting with Minor, who corrected the problem and allowed the musician to resume his career.

McLeish描述不仅听到嗡嗡声以及自己声音的扭曲，还能在自己说话时感受到振动。基本上， McLeish左耳骨头的缺失部分让上半规管对声音和压力做出了异常反应。这已经足够让半规管作为体内每处声音的放大器了。McLeish的恐怖故事在和Minor会面不久后终有一个快乐结局，Minor解决了他的烦恼，并让他重返乐坛。  

Scientific American? spoke with Minor about the nature of SCDS, the toll it takes on those who suffer from it and why it's best to plug (rather than cover up) such an opening.

  科学美国人和Minor针对SCDS的本质、它为患者带来的苦恼以及为何堵塞而非填补漏洞是更好地疗法展开了访谈。  

[An edited transcript of the interview follows.]

[以下是经过整理的访谈内容。]  

What is superior canal dehiscence syndrome (SCDS)?

  什么是上半规管裂综合征（SCDS）?  

Superior canal dehiscence syndrome is a disorder caused by an opening in the bone that should cover the inner ear's top balance canal (called the superior semicircular canal). The inner ear consists of a bony labyrinth that has two parts: the cochlea, which enables us to hear, and the vestibular labyrinth, which enables us to keep our balance. Located within the labyrinth are three semicircular canals that act as angular accelerometers for the head. When these are working properly, they allow us to maintain a steady gaze and keep images stable on our retinas even when our heads are moving.

  SCDS是一项由于本该覆盖内耳中负责上下平衡的半规管（即上半规管）的骨头出现漏洞引起的一种紊乱。内耳由骨迷路——耳蜗（让我们拥有听觉）和前庭迷路（让我们保持平衡）——构成。在骨迷路上，有3个作为头部角加速度计的半规管。当他们工作正常时，我们能够凝视物体。即便我们头部晃动，我们也能在视网膜上保持图像的稳定。  

The inner ear is a closed system surrounded and protected by the petrous portion of the  temporal bone. In superior canal dehiscence, a portion of the bone covering the superior balance canal is missing. Without this bone, the membranous canal is exposed to the overlying dura mater membrane of the brain's temporal lobe. Mechanical stimuli such as sound and pressure can then cause motion of the fluid in the membranous canal resulting in abnormal neural activity from the sensory receptor cells associated with the canal. This can lead to hearing loss, abnormal eye movements and a sudden sensation of movement (vertigo) as a result of loud noises or pressure, such as coughing or sneezing.

内耳是一个受到颞骨坚硬部分包围和保护的封闭系统。对于SCDS患者，覆盖上半规管的部分骨头缺失了。这样膜管便会暴露在大脑的颞叶的覆硬脑膜中。像声音和压力这样的机械刺激会引起膜管内液体的运动，导致和半规管连接的感官受体细胞的异常神经反应。这会引起由于压力或嘈杂噪音（咳嗽、打呼噜等）引发的听觉丧失、眼球异常运动和突发感觉（如眩晕）。

How were you able to first identify SCDS?

  你如何首先发现SCDS？  

It was a study of the eye movements. The eye movements of this disorder, which is evoked by sound and pressure, are quite distinctive. Because the superior semicircular canal is affected, the eye movement is typically vertical-torsional, meaning the eye closest to the affected ear will twitch in a direction up and away from that ear (toward the middle of the face) in response to loud noises or by stimuli that change middle ear or intracranial pressure (such as coughing, sneezing or straining).

首先是眼球运动的研究。由于声音和压力，引发的眼球异常运动是非常独特的。因为上半规管受到影响，受到噪音、改变中耳的刺激或者颅内压（像咳嗽、打呼噜或者用力）变化后，眼球运动在垂直方向发生扭曲，也就是说靠近患耳的眼球运动会在靠近和远离该耳的方向上（向着脸部中线的方向）发生扭曲。   

Can someone be born with SCDS or is this something that afflicts people only later in life?

  SCDS是先天的还是后天的？  

Our hypothesis is that about 1 or 2 percent of the population fails to develop a normal thickness of bone overlying the superior canal. If you have a normal thickness of bone, which is about 0.6 or 0.7 millimeter covering the superior canal, that's very unlikely to ever erode. If you started out with 0.1 or 0.2 millimeter of bone, yes, that can be eroded over time for a number of reasons: pressure from the temporal lobe sitting on top of it, changes in intracranial pressure or maybe by trauma. The median age of onset is in the early 40s, and it does tend to affect men and women equally. But you rarely see it in kids. That suggests that the opening probably doesn't develop until later in life.

我们的假设是大概1%~2%的人头部无法形成覆盖上半规管的正常厚度骨头。如果你有正常厚度——大概0.6~0.7毫米——的骨头覆盖上半规管，那就很难发生病变了。如果你只长有0.1或0.2厚度的骨头，那有几个原因诱发病变：颞叶上部的压力或者创伤改变了颅内压。发病的年龄中位数是40岁出头，对男女影响大致一致。不过你很少在儿童中发现病例。这表示，骨肉漏洞直到成年之后才逐步扩大。 

Does SCDS worsen over time if it is not treated?

  如果不治疗的话，SCDS会恶化吗？  

In many cases, it will stay at a certain level, although it's hard to predict. That very first patient I identified with this disorder back in 1995—whose eye movements were so profound in response to sound that I could see just on the exam that there was something wrong with the superior canal—has elected never to have treatment. His most disturbing symptom was loud noises caused his eyes to move, so he's avoided loud noises.

  上半规管裂综合征，在许多病例中，它会保持在一定的程度。早在，1995笔者确诊了第一例病例——病患的眼球运动对声音的反应非常大，以至于我通过常规检查就能发现其上半规管有病症——已经到了无法治疗的地步。最让他困扰的症状是吵闹的噪音会让他的眼球运动，所以他避免听到吵闹的噪音。   

Other people are more disturbed by the disorder. Some people develop eye movements that follow the rhythm of their pulse, and that's extraordinarily disturbing because you're constantly sensing motion. Another thing that's disturbing to people is hearing their own voice, this sense of autophony. Even a conversational voice reverberates and is uncomfortably loud, causing them to feel motion. The range of symptoms is quite diverse, so therapy has to be tailored to the individual.

其他人则大多受到病症本身的影响。有些人的眼球运动的节奏和他们的脉搏一致，这相当令人困扰——因为你时刻都感受到运动。另一件令人困扰的事情是听到他们自己的声音——自声过强。即便是稍微有点大声的对话声震动，也会让他们感受眼球运动。症状的范围十分分散，因此治疗只能因人而异。  

Do people get this in both ears?

双耳都会有这种病症吗？  

In about one third of cases it's bilateral when diagnosed. Usually there's a more symptomatic ear, and we treat that ear first (although some have opted to have both ears treated, but not at the same time). In the minority, both ears appear to be equally affected.

大概1/3的病例被确诊为双耳得病。通常有一只耳朵症状更加严重，我们也首先治疗这只耳朵（也有的人选择治疗两只耳朵，但不是同时治疗）。少数情况下，两只耳朵的病症一样严重。   

How is this treated?

如何治疗？  

The way we have treated this disorder is to mechanically inactivate the balance canal, to plug it with fascia (the covering of muscle) and tiny chips of bone taken from the patient. It's a tiny structure, so you need only the smallest amount of fascia and chips. We've used canal plugging with success. Bone cement is often used to cover the canal after it is plugged. You'd think that if the problem was the covering being missing, why not just replace the covering? In principle that sounds great. In practice, however, we found that function in the canal is often diminished before surgery, and replacing the covering itself may lead to inactivation of the canal anyway.

  我们治疗这项并征得方法是使用机械破坏声道平衡，用筋膜（肌肉的表层）和病患身上取出的小碎骨堵塞声道。这是非常微小的结构，所以你仅需要最小的筋膜和碎骨。我们成功地把这些堵塞声道。堵塞之后，骨结合剂常用于覆盖在声道的表面。你可能会想，如果问题是因为失去表层而引起的，为什么不更换一个呢？理论上这是个好主意。然而在实践中，我们发现声道的功能早在手术前就没有了，更换表层本身可能会引发声道永久失衡。  

A person can function perfectly fine with five balance canals, so we believe the best treatment is to plug the canal. We can selectively inactivate one balance canal without affecting the other balance canals. Superior canal plugging is a procedure that usually takes about four hours and requires patients to be in the hospital for a couple of days. I had worked with that canal-plugging technique in a lot of my basic research, so I was comfortable with it as a surgical technique.

一个人可以完美的协调五个声道平衡，因此我们相信最好的疗法是堵塞其中一个。我们可以选择堵塞其中一个（实质失衡）而不影响其他的声道平衡。精确的声道堵塞是一项手术花费近四小时同时要求病人在医院疗养多天的漫长过程。在笔者的基础研究中，已经处理过许多声道堵塞技术，因此笔者相信这是一项可行的手术技术。   

Are there preventative measures that can be taken or tests that determine whether a person is at risk for SCDS?

有没有预防性措施或者评估患SCDS风险的检验？  

There are screening tests that can be done. We've seen people in their teens and 20s that have this disorder. If someone is having symptoms that are suggestive of superior canal dehiscence, then the evaluation begins with a physical exam to determine if any of the distinctive signs like eye movements evoked by sound or pressure are present. A vestibular evoked myogenic potential test (a neurophysiological assessment technique to inspect the function of organs in the inner ear) can also show abnormalities characteristic of SCDS. A high-resolution CT scan can also be performed to look at the temporal bones for dehiscence.

筛查检验可以胜任这项工作。我们已经在一些青少年还有20来岁的人们身上发现这项病症。如果有人有疑似SCDS的病症，那么就会检查他是否有一些像由声音或压力导致的眼球运动这样的症状出现。前庭诱发筋电位检查（一项检查内耳技能的神经生理学检测技术）可以显示出SCDS的异常特性。高分辨率的CT检查也能用于检查颞骨是否开裂。  

You mentioned that 1 or 2 percent of the population could be at risk for SCDS. Do you know how many people are afflicted with this disorder?

你提到人群中1%~2%的人有患SCDS的风险。你知不知道有多少人受到该病症的折磨？  

Experience with this is relatively new. When we started out, we thought, well, this is probably quite rare. We don't have enough data to give an actual number of people afflicted with the disorder, you can just see from the papers and the cases being reported that it may not be all that rare.

  这项研究相对还较新。当我们开展的时候，我们因为患病率应该很低。我们还没掌握足够的数据，去给出患病人数的准确数据。不过你可以从论文和报道的病例中发现，患病率并不是那么低。  

Why are we hearing more about SCDS recently?

为什么我们最近越来越多地听说SCDS？  

It's being better recognized because it's been in the medical literature now. Most major ear centers in the world now have experience with it and are comfortable making the diagnosis. It's also a gratifying diagnosis to make, because you can do something about it. The symptoms seem bizarre—I mean, hearing your eyes move. The first patient referred to me was referred by a psychiatrist because his symptoms were so bizarre that it was clear that something was going on. It's a neat story—there's good science in it and an effective treatment once you've made a definitive diagnosis, and the outcomes tend to be very good. It's the kind of thing you'd like to see more of in medical science: Start out with a mystery, come up with an explanation, develop the right screening tests to make the diagnosis, and then come up with an effective treatment.

目前这项病症已经载入医学文献，所以病症也更容易辨认出来。世界上大多数耳科中心都遇到过这种病例，也处理得非常得体。这也算一项可喜的诊断，因为你能采取有效措施。它的症状听起来很惊人——听到你自己的眼睛在动。第一位推荐到我这来的病患是由一位精神科医生引见来的，因为病患的病症让人如此吃惊以至于很明显可以了解到发生了什么。这也算有始有终——能了解到个中的科学原理，一旦确诊能进行有效治疗，治疗效果也非常好。从这你能看出医疗科学的发展：从一种怪病出发，提出病理解释，发展一种可以确诊的检查手段，最后研究出有效的治疗方法。