Would you cure your hearing loss?

At the moment, that’s a hypothetical question. A cure for hearing loss does not exist. But as Dr. Tom Friedman of the National Institutes of Health told an audience at the Hearing Loss Association of America’s annual convention in June, a cure for at least one type of hearing loss may be around the corner.

Genetic, or hereditary, hearing loss is responsible for about 60 percent of hearing loss present at birth or developing in early childhood. It is also responsible for some adult-onset hearing loss, either on its own or in combination with exposure to noise or other toxins, including some medications like streptomycin or aminoglycoside antibiotics.

Hereditary hearing loss is caused by one or more mutations on a specific gene or genes. As of May 2019, scientists have identified 129 different genes causally associated with hearing loss. (“Causally” is the important word here.) Others are still being investigated. Gene sequencing can show which specific genes carry a mutation.

Hela Azaiez, PhD at the University of Iowa, who also spoke, noted that there are over 7000 possible mutations in those genes. She also said that an expert can sometimes predict which gene is responsible for the loss by the pattern on an audiogram. The more a person knows about the pattern of loss in the family, the easier it is to find the defective genes that are causing the loss, so a large family tree is helpful.

Autosomal recessive genes are the cause of hearing loss in 59 percent of cases. When the gene is recessive, the effect — in this case hearing loss — may skip generations and a pattern may not be apparent. Thirty-six percent of the genes that cause hearing loss are autosomal dominant, which means they always cause hearing loss. That leaves five percent, of which four percent are linked on the x chromosome and 1 percent in mitrochondrial DNA.

Two-thirds of those with hereditary hearing loss have no other related symptoms. But the remaining third have what is called syndromic deafness, with symptoms in addition to hearing loss. These include Usher syndrome (the disorder that Rebecca Alexander, subject of my previous post, has), Alport syndrome, and Perrault syndrome.

The next step for researchers is developing a way to correct those mutations. Several laboratories are moving ahead quickly with research.

Dr. Friedman is chief of the Laboratory of Molecular Genetics at the National Institute on Deafness and Other Communication. Dr. Hela Azaiez focuses on autosomal dominant genes at the Molecular Otolaryngology and Renal Laboratory at the University of Iowa. In the afternoon Dr. Azaiez gave a workshop with former HLAA Executive Director Brenda Battat and her family, who have hereditary hearing loss. Brenda Battat wrote about her family’s genetic search in an article for the January-February issue of Hearing Life Magazine called “Caught Up in a Whirlwind of Genetic Hearing Loss . They have been working with Dr. Azaiez.

To return to the question in my title: Would You Cure Your Hearing Loss? Many in the culturally Deaf community would answer with an emphatic No. Hearing loss/deafness/sign language are what makes them a community. As for me, with many years of hearing loss behind me, I would also probably say no, because the speech pathways in my brain are attuned to the way I hear now with a hearing aid and a cochlear implant.

But there are good reasons for determining the cause of hereditary hearing loss even if you don’t plan to treat it. Families may decide to correct the mutation in future generations. If it is too late to correct in younger family members, or if the family does not want to correct it, finding out what the mutation is and which family members have it allows them to plan ahead for future health care and other decisions.

The research symposium at Convention is always fascinating. This year’s panel included other researchers and much more information than I have room for here. Don’t miss next year’s symposium at the HLAA Convention in New Orleans.

For more about living with hearing loss, read my books, available at Amazon.com and Barnes and Noble, and maybe at your favorite bookstore or library. If it’s not there, ask for it!

Sunday is Mother’s Day. I miss my mom. But I especially miss all the things I didn’t hear her say.

In her 80s, my mother’s mind and body succumbed to aging. She developed dementia, she had frequent falls and she often needed a wheelchair. But her hearing remained acute. Mine did not.

After my father died, when Mom was 85, she wanted to stay in the house they’d lived in together. It was far away from any of her children, and she had 24-hour-a-day nurses’ aides. Despite this attention, she often fell or had other physical issues that would send her to the hospital, then into rehab to recover, then home again with an aide, only to fall or suffer heart problems or infections once again, and start the cycle over.

Eventually, against her wishes, my siblings and I decided she would be better in the long-term nursing care facility at the community where they lived. She ended up thriving there.

But while she was still living at home, my hearing was a significant obstacle. It was difficult for me to hear her on the phone — and frustrating for us both — so I visited as often as I could. Even when I visited her, though, her soft voice and increasing dementia made it hard for me to understand her. My hearing loss also created some potentially dangerous situations. If I had to call her doctor or even 911, I couldn’t hear their responses. I’d hand the phone to my mother to listen for me and hope she was correctly repeating whatever the doctor said. I didn’t know about captioned phones then, or I’d surely have ordered one. If Text 911 had existed, that would have removed another barrier to communication.

Once she was in the nursing facility, there were fewer crises. But as she became less clear mentally, and as her voice weakened, I not only couldn’t understand what she was saying, but I was never sure she was saying what I thought she was saying. It’s hard enough for a fully hearing person to converse with someone with dementia. Imagine what it’s like when you aren’t sure you’ve understood correctly.

My mother died in 2014. Every mother’s Day I think about all that I missed in those last years of her life. Despite hearing aids and a cochlear implant, and hearing-assistive devices galore, my hearing and her dementia still created an enormous gap. The only way across it was with smiles and hugs and just being there — for her and for me.

Most people’s hearing problems are not as severe as mine. But if you’re having trouble hearing an elderly parent with a whispery voice — or if the parent is having trouble hearing you — don’t let that happen. If you are not ready for hearing aids, buy yourself a handheld device such as a pocket talker.

But whatever solution you come up with, don’t let those words be lost forever.

(A version of this post was first published in 2015 in AARP online.)

Photo courtesy of Katherine Bouton

For more about hearing health, my book “Smart Hearing.” will tell you everything I know about hearing loss, hearing aids, and hearing health.

You can get it online at Amazon or Barnes & Noble, in paperback or ebook for Kindle or Nook. You can also ask your library or favorite independent bookstore to order it.

Last week I went to a panel discussion organized by the New York Museum Access Consortium. There were four panelists representing different disabilities and a moderator from MAC. The audience was made up mostly of people from disability rights organizations and museum personnel.

The evening’s topic was “Mindful Communication.” Avoiding hurtful or offensive language when talking about disability isn’t as easy as it sounds. “People first” is a generally accepted standard. To use hearing loss as an example, a person is not “hearing impaired,” because that suggests that the way he or she hears defines who he or she is. We prefer “people with hearing loss.” (For more on parsing terms for hearing loss see my post “Deaf, Hard of Hearing, Hearing Impaired? Be Careful What You Call Us.”) It took me a long time to realize that “hearing impaired” is a distasteful term to many with hearing loss – and I have hearing loss! So I’m relatively easy on others who slip up on the politically correct terms.

After the panelists introduced themselves, with visual descriptions for the blind in the audience, the moderator led the discussion with a variety of questions. I can’t do justice to the full hour’s discussion but a couple of comments stuck with me.

The panelists were asked what kind of language they found offensive. I expected to hear them say that casual ignorant slurs were hurtful. Instead, the answers were much more complicated.

Ansel, who has visible disabilities and uses a wheelchair, said he dislikes it when someone trying to be kind says: “God bless you.” He doesn’t feel the need to be blessed, and finds it condescending. But he doesn’t get angry. “If it makes them feel good, it’s okay.”

Nefertiti, who is blind, dislikes being called “amazing.” When she was growing up, people were always saying how amazing she was. “I was such an amazing kid just for getting up in the morning! It gave me a little bit of an ego.” She prefers to think of herself as “just a human being doing what I do. It doesn’t mean I’m amazing just because I’m blind.” Of the term “disabled” she said cheerfully, “I feel pretty abled.”

In the question and answer period, one museum employee spoke about the difficulty of using correct and acceptable language in written literature like museum guides. “We’re working with a staff with a fear around language,” she said, to nods of agreement from others in the audience. Another of the panelists, Madison, suggested that staff take their lead from the community in question. Nefertiti similarly suggested listening to the person or group and then mirroring the language they use.

This brought the conversation back around to the panel’s topic: Mindful Listening. Mindful Listening is always important, as Nefertiti pointed out. If, for instance, a sighted person offers to help a blind person cross the street, and doesn’t listen to a polite “No thanks,” nobody wins: “The blind person is offended and the sighted person is baffled.”

Some of the more digressive responses were also insightful. One of the challenges Nefertiti faces as a blind woman was something I’d never considered. Being blind, she said, means that she misses the culture of body language. Someone will say “She’s a nice girl but….” Think of all the ways you could end that sentence without saying another word:  With a shrug (suggesting “… she’s not very effective at her job”), with a roll of the eyes (“…. the clothes she wears make her look like a slut”), with a shake of the head (“… not for me!”), with raised eyebrows accompanied by a shoulder hitch (“… who knows what she’ll get into next”). Etc.

A full transcript of the discussion can be found here. 

Coincidentally, this week Major League Baseball announced that it would discontinue use of the term “Disabled List” for players who were injured. The change came about after advocates for people with disabilities complained, according to the New York Times. The Times quoted Jay Ruderman, president of the Ruderman Family Foundation, which encourages greater inclusion of people with disabilities in society: “The disability community identifies with the term ‘disabled,’” he said “When it’s used incorrectly, when someone is injured, not disabled, that’s offensive.” He went on: “People with disabilities do not consider themselves injured….Someone who tears an A.C.L. is not permanently disabled.”

It’s true (usually) that they are not permanently disabled, but should they happen to use a wheelchair for a period of time, they are entitled to the same rights under the Americans with Disabilities Act as people with more permanent disabilities. Nobody ever said the ADA was restricted to those with permanent injuries. In any case, goodbye to the DL and welcome the “Injured.”

Most of my readers have hearing loss. I’d love to hear your comments about terms and comments that offend you.

For more about hearing health, my book “SMART HEARING.” will tell you everything I know about hearing loss, hearing aids, and hearing health. Available online at Amazon or Barnes & Noble, in paperback or ebook for Kindle or NOOK.

Whether or not you are open about your hearing loss, there often comes a point at which you just can’t try to listen anymore.

I’m very open about my hearing loss. At this point, in fact, my hearing loss practically defines me, since I write and speak about it. The other night at a dinner party in a restaurant, the woman sitting next to me said she’d heard I was some kind of disability guru. A slight distortion but I like it.

There were seven of us at dinner, the others were in couples, I was on my own and knew only the hosts. They had told me in advance who would be there, so I didn’t have to struggle with names. The specials were on a blackboard, so I knew what I was ordering. The restaurant was reasonably quiet. So far so good.

The woman on my right was a well-known feminist scholar and I was slightly intimidated. But she was funny and friendly and very interesting. She was on my good side so I heard her her well.

After a while it seemed only polite to turn to the people on our other sides. On my left, my bad ear, was a man who was also hard of hearing. His bad ear was facing my bad ear. By twisting and leaning in and trying not to fall into my dinner plate, I caught enough for a reasonably coherent discussion.

Towards the end of dinner, over dessert and coffee, the conversation got more general, and apparently more hilarious, as everyone was laughing. It’s fun to watch people laugh – at least for a while. As it got funnier and funnier. I got more and more tired and stressed.

Under other circumstances- – with closer friends, if I were not a guest – I might have excused myself. But I didn’t know these people well and everyone had been very generous in acknowledging my hearing loss and speaking clearly. Now they were just enjoying themselves, and it seemed wrong to interrupt and ask that I be included. In fact, at that point there was probably no way I could have been included as the restaurant had gotten busy and loud.

The alternative was to excuse myself and leave. But I knew that would break up the conversation and probably end the evening. So I didn’t. Instead I relied on that old standby – faking it. I think it was the right decision.

What would you have done?

For more about hearing health, my book “Smart Hearing.” will tell you everything I know about hearing loss, hearing aids, and hearing health.

You can get it online at Amazon or Barnes & Noble, in paperback or ebook for Kindle or Nook. You can also ask your library or favorite independent bookstore to order it.