The Least of My Mother’s Gifts: A Gene that Sparked A Life Plague, and Purpose

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As I draft this on Mother’s Day I think of my mother, who blessed me with nurturing and many other gifts, including, alas, the gift of her hearing loss . . . which she, in turn, had received from her mother. I began my memoir, A Quiet World: Living with Hearing Loss, with this recollection:

On one of those treasured visits to my parents' home on Bainbridge Island, Washington, I use a magic pad to communicate with my eighty-year-old mother, who four years previously took the final step from hearing-impaired to deaf as she gave up wearing her by then useless hearing aids.

“Do you hear anything?” I write.

“No,” she answers, her voice still strong although she cannot hear it. “Last night your Dad came in and found the T.V. blasting. Someone had left the volume way up; I didn't hear a thing.” (Indeed, my father later explained that he recently tested her hearing by sneaking up while she was reading and giving a loud clap just behind her ear. Her eye never wavered from the page.)

What is it like, I wonder. “A silent world?”

“Yes,” she replies, “it's a silent world.”

As with Mother, so, I expect, with me. I have known for many years that I am on a trajectory toward the same deafness. When tested as a teenager, my hearing pattern mimicked Mother's—an unusual “reverse slope” pattern of good hearing for high-pitched sounds and poorer hearing for low-pitched sounds (making soft male voices harder to discern than higher female voices). From upstairs, I can hear the high-pitched microwave oven timer, though my wife, Carol, snuggled beside me in bed, cannot. But I cannot recall ever hearing an owl hoot. Carol touches my leg at each hoot: “There, can you hear it?” I hear nothing.


A quarter century and more later, I continue on that trajectory, unable now (with my hearing aids out) even to hear my wife’s voice from the adjacent pillow, unless she speaks directly into my ear. In daily life I mostly cope well enough, thanks to powerful digital hearing technologies that my mother never knew. Even so, I struggle to hear amid noise—at a party, in a restaurant—or when a questioner is across a room. Like all who suffer this invisible disability, I strain to hear. I move closer. Or, with a smile and a nod, I fake hearing.


On the brighter side, the hearing loss plague has also given me an added life purpose—supporting people with hearing loss by advocating for a “hearing loop” transformation in how America provides listening assistance in public places (through this website, through three dozen articles such as this one, and via nearly 20,000 e-mails). And this advocacy led me to four years representing people with hearing loss on the advisory council of NIH’s National Institute on Deafness and Other Communication Disorders.


There I was privileged to meet and hear from some world class hearing researchers, including the University of Iowa physician-geneticist Richard Smith, who is amassing data on the genes of many thousands of people with hearing loss. When I showed him my audiogram—my profile of hearing loss at various frequencies—he guessed that I carry a mutation on the WSF1 gene, and offered to confirm that.


So I sent in my spit tubes, and last week Smith confirmed: “You have DFNA6/14 hearing loss caused by a mutation in WFS1.”


In psychological science, we teach our students that complex traits, such as intelligence or personality, are the product of “many genes having small effects.” So this is my reminder that some important traits and medical conditions are predisposed by single genes (which my siblings and I each had a 50% chance of inheriting—with my older brother and I, among the four of us, drawing the unlucky cards).


If so, I asked: Is there not some hope that gene editing, such as with the new CRISPR technique, could prevent future hearing loss in children or young adults who carry the gene?  Yes, Smith tells me—this is, indeed, his lab’s exciting aim. Moreover, they plan to conduct the experiment by attempting the gene therapy on but one ear of each volunteer, thus enabling the other ear to serve as what we psychologists call a “within subjects control condition.”


In the meantime, I’m content to be the person Dr. Seuss described in You're Only Old Once!

           You'll be told that your hearing's so murky and muddy,

           your case calls for special intensified study.

They'll test you with noises from far and from near

and you'll get a black mark for the ones you can't hear.

Then they'll say, "My dear fellow, you're deafer than most.

But there's hope, since you're not quite as deaf as a post."

About the Author
David Myers has spent his entire teaching career at Hope College, Michigan, where he has been voted “outstanding professor” and has been selected by students to deliver the commencement address. His award-winning research and writings have appeared in over three dozen scientific periodicals and numerous publications for the general public. He also has authored five general audience books, including The Pursuit of Happiness and Intuition: Its Powers and Perils. David Myers has chaired his city's Human Relations Commission, helped found a thriving assistance center for families in poverty, and spoken to hundreds of college and community groups. Drawing on his experience, he also has written articles and a book (A Quiet World) about hearing loss, and he is advocating a transformation in American assistive listening technology (see