Gene editing has been very much in the news this spring. In particular, there have been reports from physicians and medical researchers in the People’s Republic of China, in collaboration with Massachusetts Eye and Ear Infirmary, at Children’s Hospital of Philadelphia, and Regeneron that young deaf children with the otoferlin gene (DFNB9) have had some of their hearing restored through gene therapy. While these were small-scale studies – six children in China, an 11-year boy in Philadelphia, and one child in the Regeneron study – other clinical trials are underway elsewhere, with support from Akouos, Inc., a subsidiary of the pharmaceutical firm Eli Lilly and Company pharmaceutical firm.

These studies may have far-ranging implications for the Deaf community – or not. Otoferlin, the gene that is being studied – is just one of many genes that are associated with deafness. Many people are much more familiar with Connexin-26 (GJB2), which has been widely studied but for which there are no known current clinical trials. Also, many deaf people have no genetic causation. Nonetheless, there is concern that advances in genetic engineering – a catch-all phrase that includes gene editing, gene therapy, gene modification, and other terms – could result in a decline in the numbers of deaf people worldwide. 

In short: “Are we an endangered species?”

Gallaudet University hosted a panel discussion on April 24 as part of its ongoing #WeAreGallaudet Conversation Series. Panelists were Dr. Derek Braun, ’95, a professor and geneticist in the Biology program in the School of Science, Technology, Accessibility, Mathematics, and Public Health; Dr. Brian Greenwald, ’96, a history professor in the School of Arts and Humanities who has studied and written about eugenics; and Dr. Teresa Blankmeyer Burke, a philosophy and bioethics professor and director of the School of Arts and Humanities. Brandi Rarus, Chief Communications and Undergraduate Admissions Officer, moderated the panel.

Rarus began by saying that there is a long history of people wanting to “fix, eradicate, or cure deaf individuals,” and the recent studies seemed to be yet another manifestation. She invited the panelists to provide insight from their respective areas of expertise. She also noted that this kind of conversation was closely related to one of the five Grand Challenges in The Gallaudet Promise: Realizing the value of Deaf people across the spectrum of identities.

A person with short brown hair, light goatee, and glasses, is wearing a white button down shirt, navy blue blazer is signing.
Dr. Derek Braun, ’95

Braun explained that gene therapy has been successful in mitigating some diseases and conditions, such as cystic fibrosis. CRISPR (“clustered regularly interspaced short palindromic repeats”) is a technology that research scientists use to selectively modify the DNA of living organisms, and it has potential. However, he noted that none of the children who had gene therapy in the clinical trials regained full hearing, and it was important that the child, their families, and the professionals working with them temper their expectations accordingly. He also distinguished between somatic gene editing, where material is injected into patients, and germline, a technique used in in vitro fertilization for genetic editing of embryos.

Greenwald gave a far-ranging historical overview of eugenics. “It has modern roots in the latter part of the 19th century,” he said, and possibly originated with Sir Francis Galton, an Englishman who was a cousin to Charles Darwin, and who coined the word “eugenics.” Galton was concerned about social problems of his day, crime, poverty. He wanted to improve English society, and became a proponent of improving the human race through selective breeding. While Galton’s intentions may have been good, the actual use of eugenics in many cases amounted to mass sterilization, of immigrants here, and of Jews and other “undesirable” or “imperfect” people in Nazi Germany. Additionally, deaf people were not allowed to marry in Finland, and there was a move in the United States, backed by Alexander Graham Bell and others, to actively discourage deaf people from marrying other deaf people.

A person with hair on the sides, wearing glasses, a white button down shirt and yellow tie with a black blazer and dark pants, is sitting in a teal stool signing.
Dr. Brian Greenwald, ’96

Bell, in fact, was married for over 50 years to Mabel Hubbard, a deaf woman, who chose not to associate with other deaf people. Bell did associate with deaf people, but he disapproved of their use of sign language. In his memoir, Bell wrote about the proliferation of the deaf race as a “great calamity.” We know that a deaf variety of the human race has not developed, and in fact, most deaf children are born to hearing parents.

Blankmeyer Burke reiterated that there was a long history of trying to “fix” deaf people. Cochlear implants are one manifestation; genetics is essentially the newest battleground. “We have to think about what that means for us to continue to exist. What we see now, if children have some change in their hearing status, they become effectively more hard of hearing. They still need language and support.

This is not a cure,” she said.

Greenwald stated that the idea of a cure for deafness has been around for thousands of years, as evinced by faith healers, unorthodox medicinal treatments such as mixtures of chicken blood and deer blood, onion juice and ants, etc. Other techniques in the mid-20th century included air travel to see if the popping of ears on descent would result in restored hearing, magnets, and electroshock. 

As to whether deafness is viewed as a disease, Braun noted that the Online Mendelian Inheritance in Man (OMIM) compendium of genes and genetic phenotypes lists several “horrible” human diseases for which viability is rarely past the age of three, and that several “DeafPlus genes” were listed there. However, he noted that many DeafPlus people lead “great lives.” He also said that there is evidence that Connexin-26, the most common “deaf gene” may provide resistance to certain diseases. 

A person with brown hair pulled back in a bun, wearing glasses, a black shirt, black pants, and a black, red, and gold plaid blazer is signing.
Dr. Teresa Blankmeyer Burke

Blankmeyer Burke said that framing was important, and posited that Deaf Gain, the concept that deaf people can flourish, live good lives, and remove societal barriers was one way to challenge the assumptions held by hearing people and the medical profession. 

Blankmeyer Burke, a bioethicist, said that fertility clinics have contacted her, concerned about the ethics of deaf couples wanting to have deaf children. “It’s complicated,” she said; all parents want their children to be like them, but clinics question whether it is ethically justifiable for deaf parents to wish being deaf on their children. 

In response to a question, Greenwald explained that deaf people were sterilized in Finland, Germany, and Japan. The eugenics advocate Harry Laughlin listed nine categories of people as “defectives.” He labeled deaf people as “socially inadequate” and said that deafness was a trigger for sterilization. In the United States, sterilization of “defestives” was mandated in many states, beginning with Indiana in 1907 and ending in the 1930s. Deaf people were never named in these laws, though. Possibly, said Greenwald, this was because deaf people by and large were responsible, tax-paying citizens, able to advocate for themselves. Medical advances also played a role; people with diabetes were considered “defective” until the advent of injectable insulin in the 1920s. From that point, Greenwald said, “Eugenicists left them alone.”

Still, Greenwald said, in the 1920s, even the National Association of the Deaf encouraged deaf people not to marry.

Another medical advance is that we can determine almost at birth whether a child is deaf. This makes it easier to determine genetic causation, as opposed to later-onset deafness. 

Two people wearing matching navy blue Gallaudet University polos are standing on stage debating in American Sign Language. There are teal stools surrounding them.
Joe Cherichello and Trent Mora

The panelists concluded by posing several existential questions to the in-person and online audience. 

Following the panel, Gallaudet debate team members Joe Cherichello and Trent Mora reprised their debate two weeks earlier during the Philodemic Society debate at Georgetown University on the question “Should deafness be cured?”

Watch this #WeAreGallaudet Conversation Series panel discussion.

Read about the Gallaudet University debate team.

Read the STAT News article by Timmy Broderick on gene editing.

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