It sounds like something out of an Edgar Allen Poe tale of horror. A man becomes agitated by strange sounds only to find that they are emanating from inside his own body—his heart, his pulse, the very movement of his eyes in their sockets. Yet superior canal dehiscence syndrome (SCDS) is a very real affliction caused by a small hole in the bone covering part of the inner ear. Such a breach results in distortion of hearing and, often, impaired balance.

這聽起來像艾倫·坡的恐怖故事,。一個(gè)人因聽到奇怪的聲音而激動(dòng)，卻發(fā)現(xiàn)那是從他自身發(fā)出的——他的心臟,、他的脈搏,、他眼眶中眼球的每一次運(yùn)動(dòng)。上半規(guī)管裂綜合征（SCDS）是由覆蓋內(nèi)耳的某部分骨頭上出現(xiàn)漏洞而引起,。這項(xiàng)病癥非常折磨人——聽力效果扭曲以及平衡感受損,。                  

The human ear consists of three parts. The outer ear includes the ear lobe and external auditory canal, which funnels sound waves toward the eardrum (or  tympanic membrane) allowing it to vibrate. The middle ear converts sound waves that vibrate the eardrum into mechanical vibrations for the cochlea, the hearing part of the inner ear. This area, however, also includes of a system of three fluid-filled semicircular canals in each ear—superior, posterior and horizontal—responsible for giving the brain information about angular motion of the head. SCDS can occur when some part of the bone protecting the superior semicircular canal is missing.

人類的耳朵由3部分構(gòu)成。外耳包括耳垂和外耳道——用于把聲波引入鼓膜,，使之振動(dòng)鼓膜,。中耳將振動(dòng)鼓膜的聲波轉(zhuǎn)化成對耳蝸——內(nèi)耳的接聽結(jié)構(gòu)——的機(jī)械振動(dòng)。在耳朵里,，也存在一個(gè)由三部分充液半規(guī)管的系統(tǒng)——在上下,、前后、水平方向上向大腦反饋關(guān)于頭部角度運(yùn)動(dòng)的信息,。當(dāng)保護(hù)上半規(guī)管的部分骨頭缺失時(shí),，SCDS就會發(fā)生。   

Whereas it is difficult to know exactly how prevalent SCDS is, several reported cases define how it impacts the lives of those suffering from the disorder. Stephen Mabbutt, a 57-year-old Englishman who suffered from SCDS for six years, described "hearing his eyes scratching like sandpaper every time they moved in their sockets." He returned to work earlier this month after successful surgery to plug a pin-size hole in the bone covering the semicircular superior canal in one of his ears. Toby Spencer, a 41-year-old IT professional from Skowhegan, Maine, described similar symptoms as Mabbutt as well as the feeling that loud noises made him feel as though he was losing his balance. Spencer had surgery in April to correct the problem. [Read more about inner ear ailments and remedies: "Regaining Balance with Bionic Ears"]

鑒于難以知曉SCDS的流行情況具體如何,，幾項(xiàng)研究案例顯示病癥對病患的日常生活影響嚴(yán)重,。Stephen Mabbutt，一位罹患SCDS長達(dá)6年的57歲英國人,，描述“每當(dāng)眼球在眼眶中運(yùn)動(dòng)的時(shí)候,，就聽到他們像砂紙一樣磨來磨去。”本月早前，在將覆蓋著上半規(guī)管的引腳大小的骨頭缺口填補(bǔ)的手術(shù)成功之后,，他重新回到了工作崗位,。Toby Spencer，一位來自Maine, Skowhegan的41歲IT專家,，描述了跟Mabbutt一樣的癥狀,，同時(shí)還伴有讓他感覺失衡的吵雜噪音。Spencer在四月份進(jìn)行手術(shù)解決了這個(gè)問題,。[閱讀更多關(guān)于內(nèi)耳疾病及治療方法: "裝仿生耳,，重獲平衡感"]  

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.

音樂家Adrian McLeish罹患這項(xiàng)疾病超過20年，在2008見到Lloyd Minor（位于Baltimore的Johns Hopkins University學(xué)術(shù)事務(wù)處高級副主任和教務(wù)長）之前,，不得不暫停他吹奏法國號的職業(yè)生涯,。McLeish找到了Minor，這位精于頭部頸部手術(shù)的耳鼻喉科學(xué)專家,，同時(shí)也是1998年首次在《耳鼻喉科學(xué)檔案——頭頸部手術(shù)》中描述SCDS的論文第一作者,。  

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描述不僅聽到嗡嗡聲以及自己聲音的扭曲，還能在自己說話時(shí)感受到振動(dòng),?；旧希?McLeish左耳骨頭的缺失部分讓上半規(guī)管對聲音和壓力做出了異常反應(yīng),。這已經(jīng)足夠讓半規(guī)管作為體內(nèi)每處聲音的放大器了,。McLeish的恐怖故事在和Minor會面不久后終有一個(gè)快樂結(jié)局，Minor解決了他的煩惱,，并讓他重返樂壇,。  

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.

  科學(xué)美國人和Minor針對SCDS的本質(zhì)、它為患者帶來的苦惱以及為何堵塞而非填補(bǔ)漏洞是更好地療法展開了訪談,。  

[An edited transcript of the interview follows.]

[以下是經(jīng)過整理的訪談內(nèi)容,。]  

What is superior canal dehiscence syndrome (SCDS)?

  什么是上半規(guī)管裂綜合征（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是一項(xiàng)由于本該覆蓋內(nèi)耳中負(fù)責(zé)上下平衡的半規(guī)管（即上半規(guī)管）的骨頭出現(xiàn)漏洞引起的一種紊亂。內(nèi)耳由骨迷路——耳蝸（讓我們擁有聽覺）和前庭迷路（讓我們保持平衡）——構(gòu)成,。在骨迷路上,，有3個(gè)作為頭部角加速度計(jì)的半規(guī)管。當(dāng)他們工作正常時(shí),，我們能夠凝視物體,。即便我們頭部晃動(dòng)，我們也能在視網(wǎng)膜上保持圖像的穩(wěn)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.

內(nèi)耳是一個(gè)受到顳骨堅(jiān)硬部分包圍和保護(hù)的封閉系統(tǒng),。對于SCDS患者，覆蓋上半規(guī)管的部分骨頭缺失了,。這樣膜管便會暴露在大腦的顳葉的覆硬腦膜中,。像聲音和壓力這樣的機(jī)械刺激會引起膜管內(nèi)液體的運(yùn)動(dòng),，導(dǎo)致和半規(guī)管連接的感官受體細(xì)胞的異常神經(jīng)反應(yīng)。這會引起由于壓力或嘈雜噪音（咳嗽,、打呼嚕等）引發(fā)的聽覺喪失,、眼球異常運(yùn)動(dòng)和突發(fā)感覺（如眩暈）。

How were you able to first identify SCDS?

  你如何首先發(fā)現(xiàn)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).

首先是眼球運(yùn)動(dòng)的研究,。由于聲音和壓力,，引發(fā)的眼球異常運(yùn)動(dòng)是非常獨(dú)特的,。因?yàn)樯习胍?guī)管受到影響，受到噪音,、改變中耳的刺激或者顱內(nèi)壓（像咳嗽,、打呼嚕或者用力）變化后,，眼球運(yùn)動(dòng)在垂直方向發(fā)生扭曲,，也就是說靠近患耳的眼球運(yùn)動(dòng)會在靠近和遠(yuǎn)離該耳的方向上（向著臉部中線的方向）發(fā)生扭曲。   

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

  SCDS是先天的還是后天的,？  

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.

我們的假設(shè)是大概1%~2%的人頭部無法形成覆蓋上半規(guī)管的正常厚度骨頭,。如果你有正常厚度——大概0.6~0.7毫米——的骨頭覆蓋上半規(guī)管，那就很難發(fā)生病變了,。如果你只長有0.1或0.2厚度的骨頭,，那有幾個(gè)原因誘發(fā)病變：顳葉上部的壓力或者創(chuàng)傷改變了顱內(nèi)壓。發(fā)病的年齡中位數(shù)是40歲出頭,，對男女影響大致一致,。不過你很少在兒童中發(fā)現(xiàn)病例。這表示,，骨肉漏洞直到成年之后才逐步擴(kuò)大,。 

Does SCDS worsen over time if it is not treated?

  如果不治療的話，SCDS會惡化嗎,？  

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.

  上半規(guī)管裂綜合征,，在許多病例中，它會保持在一定的程度,。早在,，1995筆者確診了第一例病例——病患的眼球運(yùn)動(dòng)對聲音的反應(yīng)非常大，以至于我通過常規(guī)檢查就能發(fā)現(xiàn)其上半規(guī)管有病癥——已經(jīng)到了無法治療的地步,。最讓他困擾的癥狀是吵鬧的噪音會讓他的眼球運(yùn)動(dòng),，所以他避免聽到吵鬧的噪音。   

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.

其他人則大多受到病癥本身的影響,。有些人的眼球運(yùn)動(dòng)的節(jié)奏和他們的脈搏一致,，這相當(dāng)令人困擾——因?yàn)槟銜r(shí)刻都感受到運(yùn)動(dòng),。另一件令人困擾的事情是聽到他們自己的聲音——自聲過強(qiáng)。即便是稍微有點(diǎn)大聲的對話聲震動(dòng),，也會讓他們感受眼球運(yùn)動(dòng),。癥狀的范圍十分分散，因此治療只能因人而異,。  

Do people get this in both ears?

雙耳都會有這種病癥嗎,？  

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的病例被確診為雙耳得病。通常有一只耳朵癥狀更加嚴(yán)重,，我們也首先治療這只耳朵（也有的人選擇治療兩只耳朵,，但不是同時(shí)治療）。少數(shù)情況下,，兩只耳朵的病癥一樣嚴(yán)重,。   

How is this treated?

如何治療？  

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.

  我們治療這項(xiàng)并征得方法是使用機(jī)械破壞聲道平衡,，用筋膜（肌肉的表層）和病患身上取出的小碎骨堵塞聲道,。這是非常微小的結(jié)構(gòu)，所以你僅需要最小的筋膜和碎骨,。我們成功地把這些堵塞聲道,。堵塞之后，骨結(jié)合劑常用于覆蓋在聲道的表面,。你可能會想,，如果問題是因?yàn)槭ケ韺佣鸬模瑸槭裁床桓鼡Q一個(gè)呢,？理論上這是個(gè)好主意,。然而在實(shí)踐中，我們發(fā)現(xiàn)聲道的功能早在手術(shù)前就沒有了,，更換表層本身可能會引發(fā)聲道永久失衡,。  

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.

一個(gè)人可以完美的協(xié)調(diào)五個(gè)聲道平衡，因此我們相信最好的療法是堵塞其中一個(gè),。我們可以選擇堵塞其中一個(gè)（實(shí)質(zhì)失衡）而不影響其他的聲道平衡,。精確的聲道堵塞是一項(xiàng)手術(shù)花費(fèi)近四小時(shí)同時(shí)要求病人在醫(yī)院療養(yǎng)多天的漫長過程。在筆者的基礎(chǔ)研究中,，已經(jīng)處理過許多聲道堵塞技術(shù),，因此筆者相信這是一項(xiàng)可行的手術(shù)技術(shù)。   

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

有沒有預(yù)防性措施或者評估患SCDS風(fēng)險(xiǎn)的檢驗(yàn),？  

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.

篩查檢驗(yàn)可以勝任這項(xiàng)工作,。我們已經(jīng)在一些青少年還有20來歲的人們身上發(fā)現(xiàn)這項(xiàng)病癥。如果有人有疑似SCDS的病癥,，那么就會檢查他是否有一些像由聲音或壓力導(dǎo)致的眼球運(yùn)動(dòng)這樣的癥狀出現(xiàn),。前庭誘發(fā)筋電位檢查（一項(xiàng)檢查內(nèi)耳技能的神經(jīng)生理學(xué)檢測技術(shù)）可以顯示出SCDS的異常特性,。高分辨率的CT檢查也能用于檢查顳骨是否開裂。  

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的風(fēng)險(xiǎn),。你知不知道有多少人受到該病癥的折磨,？  

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.

  這項(xiàng)研究相對還較新。當(dāng)我們開展的時(shí)候,，我們因?yàn)榛疾÷蕬?yīng)該很低,。我們還沒掌握足夠的數(shù)據(jù)，去給出患病人數(shù)的準(zhǔn)確數(shù)據(jù),。不過你可以從論文和報(bào)道的病例中發(fā)現(xiàn),，患病率并不是那么低。  

Why are we hearing more about SCDS recently?

為什么我們最近越來越多地聽說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.

目前這項(xiàng)病癥已經(jīng)載入醫(yī)學(xué)文獻(xiàn),，所以病癥也更容易辨認(rèn)出來,。世界上大多數(shù)耳科中心都遇到過這種病例,，也處理得非常得體。這也算一項(xiàng)可喜的診斷,，因?yàn)槟隳懿扇∮行Т胧?。它的癥狀聽起來很驚人——聽到你自己的眼睛在動(dòng)。第一位推薦到我這來的病患是由一位精神科醫(yī)生引見來的,，因?yàn)椴』嫉牟“Y讓人如此吃驚以至于很明顯可以了解到發(fā)生了什么,。這也算有始有終——能了解到個(gè)中的科學(xué)原理，一旦確診能進(jìn)行有效治療,，治療效果也非常好,。從這你能看出醫(yī)療科學(xué)的發(fā)展：從一種怪病出發(fā)，提出病理解釋,，發(fā)展一種可以確診的檢查手段,，最后研究出有效的治療方法。