Royal National Throat Nose and Ear Hospital, London (Summer 2016)
Otosclerosis fixing the stapes bone in the middle ear
In 1952 Dr. Samuel Rosen gently pushed the stapes of a man on whose ear he was operating.1 The stapes is the smallest bone in the body and the last of the three bones of hearing. Rosen was not sure whether or not the stapes had moved and so he pushed just a millimeter more and found that indeed it did move. Disappointed, he abandoned the operation, thinking he could not help his patient. But actually what he had just done was set to revolutionize ear surgery and restore hearing to millions around the globe.
Our ears pick up air-borne vibrations (sound) through the ear drum and send these vibrations through three small bones in the ear to a fluid-filled structure called the cochlea where the sound is heard (figure 1). Now in some people the last bone of hearing, the stapes, is afflicted by a condition called otosclerosis. For reasons we still do not understand, abnormal extra bone can develop around the base of this bone, and over time the laying down of this extra bone can become so extensive that it is enough to fix the stapes and stop it vibrating. The patient slowly develops hearing loss, sometimes on one side, but often both.
Joseph Toynbee, a London surgeon, was the first to describe otosclerosis in 1853.2 Over the next forty years a handful of surgeons decided to try an operation to move the fixed stapes, starting with Kessel in Germany, Boucheron and Miot in France, Blake and Jack in the US, and Faraci in Italy.3 This seemed very successful in some patients, particularly those where the disease was early and the stapes could be moved relatively easily. But the success was often short-lived: we now know that this was because the abnormal bone would grow back again and once again fix the stapes. But also, in more advanced cases of otosclerosis, the amount of force used to move the stapes was enough to leak fluid from the nearby balance organs, and patients would then suffer weeks of severe dizziness after the operation.4 At the time, several prominent ear surgeons denounced the idea of moving the stapes. Siebemann stated the procedure was “not only useless, but often harmful.”5 The idea of stapes mobilization was abandoned.
Over the next half century, a remarkable plethora of alternative treatments for otosclerosis were described.4 They included massage of the bones of hearing using any of a number of specially adapted pumps, or the inhalation of vapors of chloroform, ether, bromethyl, or iodine. When these were found to be ineffective, physicians tried electrically stimulating the bone or the muscles of the ear, or applying radiotherapy using pencils made of radium placed into the ear canal. Unsurprisingly, none of these treatments had any beneficial effect at all.
In 1938, Julius Lempert, an immigrant surgeon to New York from Poland, returned some sense to the treatment of otosclerosis.6 He reasoned that if the stapes was fixed, then perhaps he could re-route sound around it. By removing a small segment of bone, he surgically created a new hole (fenestra) into the cochlea, so that sound could travel via this hole instead of the normal route. Lempert’s “fenestration procedure” was a success. Although only around two-thirds of patients seemed to benefit, and actually a handful of patients suffered serious complications, the procedure was nevertheless a huge advance on the quackery of treatments that it replaced. Lempert’s fenestration procedure rapidly became popular. Lempert himself became renowned, settling in New York and providing instructional courses to surgeons from around the world.
In 1947 one of the students on Lempert’s course was a fellow surgeon from New York by the name of Samuel Rosen. Rosen went on to become a well-known fenestration surgeon in his own right at the Mount Sinai hospital. But Rosen had started to question why the fenestration procedure did not always work. He had come to realize that in some cases the stapes was not fully fixed, and he found that in such cases the fenestration procedure did not help. It seemed that although sound would enter through the new hole created by the fenestration surgery, perhaps it simply leaked out through the gaps around the partially mobile stapes.
Rosen decided that he must check that the stapes was fixed solid to be confident of helping his patients. So he devised a new protocol for surgery: before proceeding to fenestration, he would gently push the stapes to be certain it was fixed. In 1952 he reported on six patients he had operated on.7 Three had a fixed stapes, and so he performed the procedure with good effect; two had a mobile stapes and so he did not operate. In the final patient he was not sure whether or not the stapes had moved and so he pushed just a millimeter more. The stapes did then move, and Rosen was disappointed.1 But the patient told him that as soon as he had moved that last bit, his hearing was instantly restored. In fact the patient rang him every few weeks after the event, and said that the hearing was still fantastic, and enquired as to when he could have his other ear fixed. Rosen had accidentally rediscovered that stapes mobilization could be an effective treatment for otosclerosis.
A few weeks later the police turned up at Rosen’s house over concerns that he was a serial murderer. Two people had been killed in the area and the police had received reports of Rosen handling dead bodies. Indeed Rosen did have cadavers in his basement, but he had obtained these from the medical school to allow him to dissect the stapes more carefully. He used his cadavers to devise surgical access to the stapes and to incessantly practice the fine hand movements needed to gently move this bone. Rosen may have accidentally rediscovered stapes mobilization, but it was this scientific rigor and determination that meant he would be the one to forever change the world of stapes surgery.
The following year Rosen presented to the Medical Society of New York his results of successful restoration of hearing after mobilization of the stapes in five patients.1 Perhaps because of historic warnings against mobilizing the stapes, or perhaps because of Rosen’s liberal political views, these ideas were again denounced by Rosen’s colleagues as dangerous.
Undeterred, Rosen presented his data and demonstrated his technique to eminent ear surgeons of the time: Cawthorne in London, Frankel of Los Angeles, and Shambaugh of Chicago. They were impressed and started to emulate Rosen’s method, and the success stories began to speak for themselves. More and more ear surgeons started to mobilize the stapes.
But Rosen’s method could only be used in about a third of patients with otosclerosis: the other two-thirds of cases were too advanced to allow the stapes to be moved. The next big advance came from Rosen’s student, John Shea. In 1958 Shea furthered Rosen’s ideas and suggested that instead of mobilizing the stapes, it could be removed entirely and replaced with an artificial mobile stapes made of plastic.8 This turned out to be a procedure with a very high chance of success in restoring hearing. At this time there were many patients with otoclerosis in Europe and the US who were suffering with no known cure. This new surgery was a game changer: within a few years, thousands of these patients were successfully treated by Shea’s adaptation of Rosen’s technique.4 Except for a few small changes, the technique pioneered by Rosen and Shea is still that used today to surgically treat otosclerosis. Millions around the globe have been cured.
Samuel Rosen’s little push to the stapes ignited in his mind the idea of a new way to treat otosclerosis. But it was Rosen’s drive and determination that saw that this little push would go on to revolutionize the world of stapes surgery.
- Rosen S. The Autobiography of Samuel Rosen. New York: Alfred Knopf, 1973.
- Toynbee J. Case of complete bony ankylosis of the stapes to the fenestra ovalis. trans Pathol Soc London 1853;4:253.
- Tos M. Surgical solutions for conductive hearing loss. Stuttgart: George Thieme Verlag, 2000.
- Hillel AD. History of stapedectomy. American journal of otolaryngology 1983;4(2):131-40.
- Shambaugh G. Surgery of the ear. Philadelphia: WB Saunders, 1987.
- Lempert J. Improvement of hearing in cases of otosclerosis. Archives of otolaryngology 1938;28:42.
- Rosen S. Palpation of stapes for fixation; preliminary procedure to determine fenestration suitability in otosclerosis. A.M.A. archives of otolaryngology 1952;56(6):610-5.
- Shea JJ, Jr. Fenestration of the oval window. The Annals of otology, rhinology, and laryngology 1958;67(4):932-51.
, FRCS (ORL-HNS) DPhil, is currently undertaking subspecialty training in ear surgery in London at senior resident level. His research looks at molecular pathobiology of ear disease, where he leads recruitment and analysis to the largest cohort of otitis media genetics in the world, comprising 7,000 individuals. He is the only trainee elected to the board of the International Society for Otitis Media. His awards include the Phizackerley Senior Scholarship at Balliol College (University of Oxford) 2009-11, the 2013 European Academy of Otology & Neuro-otology Award, and the 2014 Margaret Witt Scholarship for Clinical Excellence from the Royal College of Surgeons of England.
Highlighted in Frontispiece Summer 2016 – Volume 8, Issue 3