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Genetics, Disability, and Deafness John Vickrey Van Cleve (editor).

The Formation of 'A Deaf Variety'

By Cal Montgomery

Genetics, Disability, and Deafness, John Vickrey Van Cleve (ed.). Washington: Gallaudet University Press. 2004. Hardcover, xi + 227 pages, $39.95.

In 1883, Alexander Graham Bell -- best known in hearing culture as the inventor of the telephone and famous in Deaf culture for his oralist stance -- announced that he wanted "to draw attention to the fact that in this country deaf-mutes marry deaf-mutes." Drawing on the accepted eugenic theories of the time, he expressed concern that the practice of deaf-deaf marriages might lead to "a deaf variety of the human race."

Bell is quoted in Joseph J. Murray's essay, "'True Love and Sympathy': The Deaf-Deaf Marriages Debate in Transatlantic Perspective," one of 13 essays in .Genetics, Disability, and Deafness. Information from the book's cover informs us that these papers, "from science, history, and the humanities, ...show the many ways that disability, deafness, and the new genetics interact and what this interaction means for society." They were originally presented at a conference held at Gallaudet University in 2003

One advantage of the cross-disciplinary nature of the conference seems to have been that presenters did not assume that their audiences had extensive backgrounds in the fields from which the presenters came. This means that while it's an academic book, it's relatively accessible to an audience without advanced degrees. It also means that the endnotes and bibliographies (provided by every essayist except Nora Groce) are helpful to those of us interested in learning more about fields in which we are relatively ignorant.

The corresponding drawback, of course, is that a presenter with limited time who cannot assume that the audience has a solid background in the field under discussion will not be able to provide a detailed, nuanced, thorough discussion of whatever it is he or she is there to discuss. The better prepared I was for the subject of an essay, as a rule, the more frustrated I was at what I thought was missing. (In light of this, I regret to say that I wasn't nearly as frustrated as I would have liked.) This is another reason to value the bibliographies and notes: there are longer pieces, and pieces aimed at people who are much better prepared in specific fields, cited there.

The possibility of "a deaf variety of the human race" has long been rejected by people who note, among other things, that the overwhelming majority of families with two deaf parents have hearing children. More recently, the eugenic theories on which Bell and others based their concerns have been discredited. The idea that a deaf variety (should one arise) would be a bad thing is still going strong.

Many Deaf people do not consider deafness a disability, because the important limitations imposed on the lives of deaf people are imposed by society from outside -- and not by physical characteristics inside -- their bodies.

Many disabled people, however, argue that the limitations on all disabled lives are imposed by society from the outside; and the principal beneficiaries of a society designed for hearing/nondisabled people seem intent on believing, no matter what anyone says to them, that the limitations imposed on deaf/disabled lives are wholly the result of our tragically not being more like them than we are.

These are the same folks, of course, who are shocked that those of us who are tragically not more like them might not be hoping against hope that we can be made to resemble them more closely or, at the very least, that those of us alive today will be the very last people who are so afflicted.

They were my constant, if incorporeal, companions as I went through Genetics, Disability, and Deafness. They worried about how they might go about helping to ensure that we are the very last of our kinds. Their mouths gaped open when they were faced with our noncompliance with their plans. They scuttled around devising industrious schemes to overcome our noncompliance.

And somehow they found themselves having circled 'round to where Bell stood all those years ago; and started up again.

"For decades," writes Walter E. Nance in the essay "The Epidemiology of Hereditary Deafness," geneticists have taught students that they should not be concerned that finding cures for serious genetic diseases, such as phenylketonuria [PKU] or cystic fibrosis [CF], will increase the frequency of those diseases in the general population. . . ."

Why might they worry? Because as long as having those genes makes people less likely to have children than people without those genes, relatively few of them will be passed from one generation to the next. But if you find a way to give people with PKU or CF the same chances in life as everyone else, more of those people's children will be born, and even more of their grandchildren; plus, people who judge the genetic risk of their children having PKU or CF to be too great will be reassured and may decide to go ahead and have children anyway.

Why shouldn't genetics students worry, according to decades' worth of geneticists? Because the number of people with PKU or CF will increase slowly. There'll be more children with the relevant genes, but not many more.

But that's assuming that people pick their sexual partners pretty much at random -- which, as far as a lot of genes are concerned, is a reasonable assumption. Genes for deafness, on the other hand, are probably different. People want partners with whom they can communicate and with whom they have things in common, and culturally Deaf people living in a dominant hearing culture may quite reasonably prefer other Deaf people as partners.

"There is a great battle going on in our intellectual culture today," writes Louis Menard in the first essay in the book, "The Science of Human Nature and the Human Nature of Science." "It is a battle between people who believe that science opens new possibilities for human life and people who worry that it closes them."

It's probably more complicated than that: in his essay "Not This Pig: Dignity, Imagination, and Informed Consent," Mark Willis talks about the differences in the ways he approaches his genetically-related blindness, heart problems and risk for Alzheimer's. Many disabled people believe that some applications of science open new possibilities, and others close possibilities off.

And by the time the reader gets to the last essay in the book, Michael Bérubé's "Disability, Democracy, and the New Genetics," and encounters Bérubé's challenge to the idea of "modest, circumspect, prudent scientists" versus "fuzzy-headed, antediluvian humanists who come into these discussions armed only with citations from Nathaniel and Aldous Huxley," the reader will have encountered a variety of ways to look at Menard's battlefield: in these pages is found not only an attempt to briefly explain some of the science, but a look at how some people struggle with the implications of that science in different times and places.

Before the rise of Deaf culture, deaf people may have reproduced pretty much at random, but with Deaf culture has come assortative mating, people choosing people who are like them as partners. Assortative mating is not limited to Deaf people, either: Nance points out that Tay Sachs disease and sickle-cell anemia are more common than they would be if Jews and African-Americans picked their partners from the population at random.

And that's where the gene called GJB2, which codes for a protein called connexin 26, comes in. There are variations in GJB2 that can cause deafness, and a great many deaf people have those variations in their genes. In fact, as Genetics, Disability, and Deafness contributors Kathleen S. Arnos and Arti Pandya report in "Genes for Deafness and the Genetics Program at Gallaudet University," in a study involving 737 unrelated deaf individuals, about 22 percent were deaf as a result of GJB2 -- including 42 percent of those with two deaf parents and 32 percent of those with two hearing parents and one or more deaf siblings.

Worrying about "deaf-mutes marrying deaf-mutes" has been replaced by worrying about aborting on the basis of hearing status, and about the use of genetic technology to select which embryos to try to bring to term.

Nance, drawing on work done by Gallaudet College professor Edward Allen Fay about a century ago and on mathematical models that assume assortative rather than random mating, argues that the number of people in America who have connexin 26 deafness is increasing.

One of my favorite insights in Martin Pernick's The Black Stork is that you don't need a technology that works to make you wrestle with tricky moral questions -- when must you use it? when may you use it? -- you just have to believe that you have a technology that works.

Connexin 26 makes Bell relevant again today -- because, once again, scientists believe that the reproductive practices of some deaf people will result in an increase in births of deaf children; debates about practices that would produce fewer, or more, deaf children are current again. Worrying about "deaf-mutes marrying deaf-mutes" has been replaced by worrying about people with certain variants of GJB2 commingling their genetic material, about choosing sperm donors, about aborting on the basis of hearing status, and about the use of genetic technology to select which embryos to try to bring to term.

The section on genetics, which was my introduction to connexin 26, comes in the middle of the book, after Louis Menard's "The Science of Human Nature and the Human Nature of Science" and Nora Groce's "The Cultural Context of Disability" and the history section, with essays by Brian H. Greenwald, Joseph J. Murray, and John S. Schuchman.

I talk to dead people -- not in the Haley Joel Osment sense, but in the sense that, being much better at interacting with text than at interacting with people, I'm a lot more comfortable working through a "conversation" with someone who has lived, written, and died (and whose responses to what I have to say I can therefore at least attempt to fill in) than I am trying to deal with someone face to face (and figuring out how he or she is actually responding to me). I therefore enjoyed Greenwald's, Murray's, and Schuchman's chapters as I was reading them, assuming that they were there to give some idea of how we had arrived at the situation we now find ourselves in. I never doubted their importance, but by the time I had gotten through the science section I was struck by their urgency.

If I were recommending this book to a friend, I would suggest reading Menard and Groce, then jumping ahead to the science section and then back to the history section before carrying on with the discussions about contemporary people and arguments.

Directly after the science comes a set of pieces by Kathleen S. Arnos and Arti Pandya, Anna Middleton, Shifra Kisch, and Mark Willis; the topics being discussed arise from many of the same concerns that become clear in the history section despite the overwhelming differences in the sciences of heredity that inform them. The science is very different, the human attempts to grapple with the science less so.

And then come Krentz and Bérubé, finishing off what Menard and Groce began by putting the intervening essays in perspective as they locate the discussions about deafness within the discussions about disability, and then the discussions about disability within the discussions about genetics.

For a book that arose from a conference and was presumably not carefully structured in advance, Genetics, Disability, and Deafness does seem -- even with the history before the science -- remarkably well-put-together, in large part due to the well-matched opening and closing pairs. The pieces in between, though not as even as a single writer would have made them nor as systematic as an attempt to cover the issue comprehensively might have made them, are not as jarring a collection as sometimes results from a conference, and especially a multidisciplinary conference.

It's actually a pretty good read, which is not something you can always say about academic books.

Posted Nov. 1, 2004

Cal Montgomery once had a conversation with her sister in which she was informed that any future nieces or nephews would have joined the Montgomery family through adoption, because Cal's genes weren't welcome in her sister's babies. This news went over about as well as you would expect, given that Cal reads Mouth, writes for Ragged Edge, and likes her genes exactly the way they are. Read her essay, Critic of the Dawn.


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