Breaking the Sound Barrier: 50 Years of Advancing the Frontiers of Hearing Research
“I can hear the grass grow!” Collette Ramsey Baker exulted after her ear surgery in 1956. Having suffered 25 years of hearing loss caused by otosclerosis, Ramsey Baker was so thrilled with the improvement in her hearing that she decided she must help others who were also struggling with hearing ailments.
Her daughter, Collette Wynn, remembers, “My mother made a promise that, if the operation worked, she would do something to support research to find the causes of deafness and develop better treatments.”
The operation did work, much to Ramsey Baker's delight. So pleased was she with her improved hearing that she went on to found Deafness Research Foundation (DRF), and to become an ally of tremendous vision, commitment and tenacity for everyone with hearing loss, as well as the investigators working in hearing and balance sciences.
Raised in Florida, Ramsey Baker lost her mother at the age of 12. The following year, while swimming with friends, she dove into a shallow lake and hit her head on the bottom with considerable force. Although she managed to avoid paralysis, in later years Ramsey Baker began to suffer serious spinal problems, resulting in seven or eight back surgeries throughout her life. Though not likely related to the diving accident, she also began to experience rapid hearing loss during her teenage years. As Wynn recalls, “Between her back and her ears, I was constantly visiting her in the hospital.”
Although Ramsey Baker’s hearing was much improved after her operation, a procedure called fenestration, she was only able to hear sounds of 25 decibels and louder, the lower end of the range of normal hearing. Her level of hearing loss is not known precisely but Wynn remembers, "She was quite severely deaf my whole childhood. In those days hearing aids were awful. They didn’t help her at all and so she didn’t wear them. It was very hard for her.”
In spite of these trials, Ramsey Baker still managed to become a Florida junior swimming champion and later, after moving to New Jersey at the age of 16, a state-level amateur golf champion. “Mom is probably the most driven person I’ve ever met,” Wynn says. “When she makes up her mind she’s going to do something, she does it.”
Launching the Deafness Research Foundation
The main thing Ramsey Baker did to keep her promise to help others who struggle with hearing issues was launch DRF. The organization’s aim was, and still is, to raise money to support researchers working in hearing and balance sciences.
A major boost to Ramsey Baker’s vision came from her husband, Hobart Ramsey. At 28 years her senior, he was chairman and CEO of the Worthington Corporation, maker of pumps and steam turbines for the Navy. President Eisenhower appointed Ramsey to the Committee on the Reorganization of the Navy Department, where his work resulted in a Distinguished Service Medal award. Through her husband’s many business and government connections, Ramsey Baker was able to secure the seed money required to get DRF operational.
With the initial funding secured, Ramsey Baker’s own physician, Walter Petryshyn, M.D., director of the Department of Otolaryngology at Mountainside Hospital in New Jersey, helped to connect her to doctors and researchers in the field. Two of those were Gordon Hoople, M.D., an otolaryngologist who taught the subject for 25 years at Syracuse University, and George Shambaugh, M.D., chairman of the Department of Otolaryngology at Northwestern University Medical School in Chicago. In 1966, Wesley Bradley, M.D., professor of surgery at Albany Medical College in New York, along with Jack Hough, M.D., became the first physician members of the DRF board. Ramsey Baker also began to make contacts among researchers in the field of hearing loss and, as Wynn recalls, she frequently went to Washington to meet with people at the National Institutes of Health (NIH).
A Solution for Otosclerosis
In the same year Ramsey Baker underwent the fenestration surgery, a new and much better solution for otosclerosis came to the fore: the stapedectomy, successfully performed for the first time in 1956 by John Shea, M.D. Along with other surgeons, Shea had been employing a procedure called stapes mobilization, developed by Samuel Rosen, M.D., at Mt. Sinai Medical Center in New York. As George Gates, M.D., medical director of DRF, relates, “The stapes was immobilized by otosclerosis but if you went in with tiny instruments and gently pried it loose, it sometimes became mobile again and the person’s hearing was then restored. The problem was that in a year or two, the bone re-grew and all advantage was lost.”
What Shea discovered with the stapedectomy was that if the stapes was completely removed and replaced by a prosthetic device, the patient was able to hear almost perfectly. It was thought at the time that removal of the stapes caused certain death to the ear. In fact, the stapedectomy offered a long-term solution to otosclerotic hearing loss with none of the drawbacks Ramsey Baker has had to endure as a result of her fenestration. As DRF strived to fund promising research on a wide variety of hearing-related ailments, many of what Gates calls the “first wave” of DRF grants in the 1950s and 1960s went to enable further research on otosclerosis and stapedectomies.
The Centurions Lend Support
While Shea pioneered the stapedectomy, Howard House, M.D., “probably performed more stapes surgeries than any other human being,” according to Gates. Not only was House prolific, but he made a huge contribution in the early days of DRF by enthusiastically joining and supporting The Centurions, an association founded by Ramsey Baker and Hoople in 1963. Each member of this innovative DRF-sponsored fellowship of otolaryngologists and researchers contributed $100 per year to DRF, helping defray the administrative costs of the foundation.
Also giving The Centurions and DRF a significant boost was Harry Rosenwasser, M.D., an otolaryngologist at Mt. Sinai Medical Center, who taught other young surgeons at the Lempert Institute, founded by Julius Lempert, M.D., the doctor who operated on Ramsey Baker in 1956. So committed was Rosenwasser to DRF and to the cause of The Centurions, at the annual meetings of the American Academy of Otolaryngology, he set up a DRF publicity table and sought to persuade his colleagues to join. Robert Ruben, M.D., an otolaryngologist at Albert Einstein College of Medicine in New York, recalls that Rosenwasser “was a very affable, friendly gentleman who would stand at that booth and graciously but firmly collar people into joining The Centurions, as well as urge young researchers to apply for DRF grants. Harry strongly believed in the necessity of having young people do research to advance the capability of hearing science to solve the hearing problems people were facing.”
The existence of The Centurions enabled a young DRF to assure donors that 100 percent of their contributions were going directly to fund hearing research.
Investing in the Future
Ruben recalls that through Rosenwasser and others in the early days of DRF, the foundation provided a significant stipend to young researchers. “DRF was a catalyst in enabling a group of young people who were interested in doing research to do it,” he says. “Whether they would prove they could do anything or not, nobody knew. But it was worth providing the seed money to as many of these as possible with the idea that a certain number would come through. And DRF helped encourage these young researchers.”
Edwin Rubel, Ph.D., professor of otolaryngology at the University of Washington, agrees: “DRF has played a major role in the evolution of hearing research. It provided a way for young people to develop ideas that might be different from those of their mentors and to get funding to try these new ideas. The secret of success of American science, in contrast to European and Asian science, is the philosophy of allowing young, energetic people to figure out their own ideas and their own approaches and to obtain independent funding for new approaches directly without having to go through a superior who might discount their new approach.”
When the flow of funding and grant requests became significant, Rosenwasser, working with Laurence Boies, M.D., of the University of Minnesota, and John Lindsay, M.D., of the University of Chicago, set up a grant program whereby applicants could submit research proposals. A scientific peer review committee then evaluated the aims and potential benefits of the envisioned research.
Rosenwasser, interviewed in 1985 by the John Q. Adams Center for the History of Otolaryngology, recalled, “In the first grant period, two grants were made totaling $14,000.” The program has come a long way. Over its 50-year history, DRF has awarded nearly $24 million through more than 2,100 grants, while The Centurions, headed now by Richard T. Miyamoto, M.D., chairman of the Department of Otolaryngology at Indiana University, continue to give generously to directly support research. This year begins a new Centurion initiative: the DRF Centurion Clinical Research Award (see more in “Meet Our Partners,” p.46).
In addition to securing funding, researchers in the early years of DRF desperately needed to be able to study human temporal bones. The temporal bones contain the inner ear structures, enabling researchers to study the disease process and gain a greater understanding of how to treat various hearing maladies. Rosenwasser, working with Boies and Lindsay, saw this need and enabled DRF to establish a program in 1960 which encouraged people to donate their temporal bones to medical research upon death. It grew over the years into the National Temporal Bone Hearing and Balance Pathology Resource Registry, sponsored by NIH’s National Institute on Deafness and Other Communication Disorders, an initiative that has led to countless breakthroughs in the understanding and treatment of hearing disorders. Those seeking answers to the problems caused by otosclerosis benefited tremendously from this registry.
Cochlear Implants
With stapedectomies becoming increasingly prevalent as the solution to otosclerosis, DRF began to focus attention on what Gates calls the “second wave” of research emphasis in the 1970s and 1980s: the cochlear implant, a device that artificially stimulates the auditory nerve and thus provides a new means of hearing. For those whose hearing was so profoundly impaired that hearing aids were of no help, this promise was tantalizing. In the 1960s real progress in the preliminary development of cochlear implants began to occur with William House, M.D., brother of Howard House, and Blair Simmons, M.D., of Stanford University, achieving the first human implantations of a cochlear device. The early devices were crude and cumbersome, with Simmons’ implant requiring the recipient to cart around a car battery to power it. But after House developed the first wearable signal processor in 1972, DRF began to fund his continuing research to refine the cochlear implant for widespread use.
A cochlear implant does not enable normal hearing and those who receive one must learn, or re-learn, to hear. Nevertheless, implants do permit those who would otherwise be deaf, or nearly so, to live relatively normal lives. The impact has been particularly favorable for children, who are able to learn to communicate orally.
Indeed, one of the greatest attributes of the cochlear implant is its benefit to children; the advantage cochlear implants provide to deaf youngsters is incalculable. In order to help children learn to speak and develop language ability, however, the devices must be implanted early in a child’s development; the earlier a child receives an implant, the better the results in language acquisition.
This promising treatment for infants as young as 12 months naturally spurred an interest in early identification of hearing loss – another of DRF’s initiatives. When hearing loss is detected early, children may receive hearing aids, become candidates for cochlear implants and receive speech/language therapies, while parents are able to access supports to minimize the impact of hearing loss on their children. To promote this sort of early intervention, DRF spearheaded a program called Hearing Healthy Kids as part of the National Campaign for Hearing Health. The five-year campaign, begun in 1995, sought to put hearing health onto the national agenda, educate the public about the potential dangers of hearing loss and the need to protect hearing, and advocate for universal newborn hearing screening and early intervention. Due in part to this successful initiative, 95 percent of newborns now receive hearing screenings before leaving the hospital or birthing center. (See “More Road Work Ahead” on p. 36.)
The Hope of Hearing Restoration
By the late 1980s, stapedectomies had solved the problem of otosclerosis, cochlear implants enabled many severely hearing-impaired people to hear again and remarkable progress was achieved on many other fronts. Then a whole new arena of promise for those with hearing problems emerged in the field of biological regeneration of inner ear hair cells.
When sound enters the outer ear via the auditory canal and is transmitted to the ear drum, the sound waves cause the ear drum to vibrate. These vibrations are then transmitted via three tiny bones called the malleus, incus and stapes – informally known as the hammer, anvil and stirrup – through the oval window into the inner ear. The oval window conveys vibrations into the fluid that fills this inner portion of the ear, which also contains the snail-shaped cochlea and the organ of Corti. In this organ are contained a beautifully ordered array of tiny cells that have little hair-like structures on their surfaces. When the vibrations cause these “hairs” to bend, they in turn create nerve impulses which are transmitted to the brain, which receives the impulses and interprets them as sound.
Hearing loss occurs when hair cells become damaged or destroyed. According to Yehoash Raphael, Ph.D., director of the Otopathology Laboratory at the Kresge Hearing Research Institute at the University of Michigan, “The majority of cases of profound deafness, whether hereditary or environmental, involve a loss of hair cells, as well as a disruption of the fragile microenvironment in and around the region where hair cells reside: the organ of Corti.” The problem for human beings is that, if our hair cells are destroyed – whether through loud noise, drugs which have hearing-damaging side effects or other trauma – they don’t regenerate.
However, a surprising discovery came to light in 1986 when Rubel, at the University of Washington and Doug Cotanche, Ph.D., now at Boston University, independently discovered that some animals can replace damaged hair cells. Cotanche says, “Birds are the only other vertebrates besides mammals with a cochlea, but we discovered that when bird hair cells are damaged, they are replaced spontaneously.” Hearing Health Spring 2006 reported, “Cotanche and his team learned that when bird hair cells die, they send out signals to supporting cells to ‘rescue’ them and more cells are made. Mammals do not have this ability but Cotanche is hopeful that within 20 years we will have a biological repair mechanism, modeled on chickens’ innate abilities, which will successfully regenerate hair cells in human ears.”
Rubel says, “We’re looking at ways of preserving hair cells, as well as regenerating them. It’s really an exciting time in hearing research and I’m probably more enthusiastic about the field than I’ve ever been in my career. The field is really wide open in terms of potential therapies.” Rubel himself has not received DRF funding, but many young researchers working in his laboratory in Seattle have received their first research funding from DRF. Cotanche, who received several DRF grants in the 1980s, says, “DRF was incredibly critical in providing the seed money to allow that project to go forward. Like many people in this field, I’m indebted to them.”
Recognizing the astonishing potential of regeneration biology to restore hearing, DRF is spearheading the ambitious Hearing Restoration Project (HRP). This new endeavor aims to raise $50 million over the next decade to fund the groundbreaking work of the DRF-initiated Hearing Research Consortium, a team of internationally recognized senior scientists who will lead and design a coordinated, integrated approach to solving the puzzle of regenerating human hair cells. Because current funding systems are based on competitive review, collaboration between researchers has been problematic. Thus, laboratories vying for limited financial resources rarely combine forces. Under the consortium model DRF has developed, the best and brightest minds in this field will work in an integrated and collaborative way to achieve the mission of the HRP, namely, to develop methods for replacing missing or dysfunctional sensory cells of the inner ear with the ultimate goal of restoring lost hearing and balance function in humans.
As Rebecca Ginzburg, DRF’s current board chair says, “The Hearing Restoration Project could lead to groundbreaking research that could ultimately cure hearing loss. I am excited by the prospect of having talented doctors, researchers and scientists collaborating to move research forward at an unprecedented pace.”
Helen Keller observed, “Blindness separates you from things. Deafness separates you from people.” Just over a half-century ago, Collette Ramsey Baker was reunited with family and friends through the restoration of her hearing, made possible by doctors, scientists and concerned citizens who gave of their labor, time and resources to press forward the boundaries of medical knowledge and expertise. From this promising frontier have come answers and hope for those eager to reconnect with sounds long gone or never heard, and most importantly, with other human beings.
Collette Ramsey Baker, now 89, lives in Florida with her husband Maurice Baker, whom she married in 1988, her first husband Hobart having died in 1981. The restoration of her hearing has enabled her to enjoy 52 years of connection to her two daughters, eight grandchildren and two great-grandchildren, not to mention countless friends and colleagues. As DRF embarks on its next 50 years, looking forward to the bright horizon of potential hearing restoration, Ramsey Baker could echo the heartfelt words she uttered in 1961: “To each who has joined our crusade to bring new hope to those now deafened and to spare future generations from this isolating affliction, we offer our prayerful thanks on their behalf.”
