In Rural Areas Where Audiologists are Rare, Telemedicine Can Help

In November, the FDA issued a ruling that will make life easier for cochlear implant recipients living in rural areas.

Implant surgery and immediate follow-up for mapping and programming must be done at a medical center or specialized clinic, but the new FDA ruling would allow remote programming for later adjustments. The audiologist and patient communicate via two-way video, with the audiologist programming the implant via computer just as he or she would in person.1495316546168 copy

The new ruling applies to Cochlear America’s Nucleus Cochlear Implant System, but other implant companies will probably follow with similar features. The ruling may eventually apply to programming hearing aids as well.

The FDA approved remote programming for patients age 12 and over who have had their implant for at least six months and who are comfortable with the programming process.

The FDA based its ruling on a clinical study of 39 patients, each of whom had had an implant for at least one year. The study included one in-person programming and two remote programming sessions for each patient. “Speech perception tests one month after each session showed no significant difference between in-person and remote programing,” the FDA said.

Twenty percent of Americans live in rural areas, while only nine percent of physicians practice there, according to an article by Greg Slabodkin in the online newsletter Health Data Management.

This comes to about 62 million Americans, for whom access to affordable healthcare is a major concern, according to an earlier article in Health Data Management. David Schmitz, M.D., president of the National Rural Health Association, testified to a congressional committee last July. He added that broadband providers must invest in the necessary technological infrastructure to make telemedicine possible.

Audiologists in rural areas are even rarer than physicians. Telemedical programming allows qualified audiologists to reach far more patients than they would ordinarily be able to do.

Speaking of the November FDA ruling, Malvina Eydelman, MD, director of the Division of Ophthalmic, and Ear, Nose and Throat Devices in the FDA’s Center for Devices and Radiological Health, said that telemedicine can “reduce the burden to patients and their families, especially those who must travel great distances or need frequent adjustments.”  Cochlear implants need adjustments not only as the user’s hearing adjusts to the implant, but as new technology becomes available. Since programming is done via the external processor, telemedicine is an easy and appropriate tool.

 

 

 

One Step Closer to Prevention of Hereditary Hearing Loss

Almost half of all hearing loss has an underlying genetic cause. Late-onset hearing loss, which occurs after the acquisition of speech, may appear in generation after generation, often progressing to a severe or even profound loss. Or it may skip generations, passing the faulty gene along to unsuspecting offspring.

Those affected, even if they were aware that they might eventually lose their hearing,  are usually part of the hearing world and do not know sign language. The loss may be severe enough to be disabling, even with sophisticated hearing technology. Preventing this loss, even when anticipated, has not been possible.

On December 20th, researchers at the Broad Institute of MIT and Harvard and the Howard Hughes Medical Institute published a study in the prestigious journal “Nature” that holds promise for prevention of hereditary loss.

Why do we care about a study on mice? Mice, like all mammals including humans, cannot regrow damaged hair cells. If prevention works on mice, it may work on other mammals. Mice are also the test subject for studies on regeneration of hair cells, which would allow a reversal of hearing loss.

The mutant gene, whimsically called Beethoven by researchers, is found in the hair cells of the inner ear. Because the gene is dominant, it takes only one to cause damage. That also means it may exist alongside a healthy copy of the gene. One of the challenges for researchers was to find a way to target just the mutated gene without disrupting the normal copy.

The technique the researchers used, called CRISPR, is a gene editing technique that the journal “Science” cited as the 2015 Breakthrough of the Year. I won’t try to explain the technique (or even the acronym) but here’s a link to a reader-friendly  article in the L.A. Times that laid out the technique – and its potential dangers.

What distinguished the new study, according to the scientists, is that this is the first time a genome-editing protein has been ferried directly into the relevant cells to halt progression of genetic hearing loss.

Direct delivery of the protein allows “exquisite DNA specificity,” according to the press release. The specificity is needed to selectively disrupt the pathogenic copy of the gene without disrupting the normal copy.

Since this type of genetic hearing loss generally manifests as late onset, it would allow researchers to test suspected carriers of the defective gene (the Tmc1 gene) and to treat carriers. As co-senior author Zheng-Yi Chen, associate professor at Massachusetts Eye and Ear said, the later onset allows “a precious time window for intervention.”

The subjects of the study were 100 Beethoven-model mice carrying one copy of the defective gene and one normal copy. Untreated, the mice began to show hearing loss at four weeks, and by eight weeks were profoundly deaf (as measured by auditory brain-stem response). The treated mice, in comparison, responded to sound at about 65 decibels, the level of normal human speech.

Peter Barr-Gillespie, a sensory biologist at Oregon Health and Science University who was not involved in the study, praised it (in the “New Scientist”) as a “pretty significant piece of work.”  He noted, however, that the decibel level at which the treated mice could hear was relatively loud compared to the hearing threshold in wild mice, which is 30 to 40 decibels. “It’s nowhere near the threshold of the wild-type mice, [but] the 10-15 decibels could make a huge difference in humans,” he said. “That sort of loss of hearing is very noticeable in people and could make for substantial improvements in quality of life.”

As always with gene editing, one concern is possible undesirable changes in the DNA. Senior co-author David Liu said the researchers had not observed “any off-target editing in the animal.” In the specific cells treated, they found only one modification, in an area not known to play a role in hearing. One major concern, however, would be the potential to develop cancer. Stephen Tsang, a clinical geneticist at Columbia, praised the study as “good for basic research“ (in “Axios”) but, like others, noted that there are many more steps to prove it safe and effective for humans.

This study showed that gene editing prevented, rather than reversed, hearing loss in mice. But for those affected by genetic hearing loss, it’s a promising step. “A lot of additional work is needed before this strategy might inform the development of a therapy for humans,” co-senior author David Liu said, “but at this stage, we’re delighted and excited that the treatment preserved some hearing in the animal model.”

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For more information on living with hearing loss, see my books on Amazon.com.

 

 

 

 

 

Let’s Make Hearing Loss a Visible Disability

Hearing loss is often referred to as an invisible disability, because there are no telltale markers — no wheelchair, no white cane. It’s invisible even compared to Deafness, with its vibrant silent language.

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NYPD Officer Daniel Carione and his attorney, Colleen Meenan, who successfully sued for the right to wear hearing aids.

For a long time, people with hearing loss wanted to keep it invisible. They wanted hearing aids no one could see, they pretended they could hear when they couldn’t. Even today hearing aid companies advertise: “So small no one will ever know you’re wearing them.” Hearing loss is for old people, or damaged people, and our culture values youth and health.

But as more and more of us use hearing aids – both because we are getting older and because we live in a noisy society – we want accommodations. We want captions on our TV’s and in movie theaters, we want hearing assistive devices that work in lectures and live theater. (The hearing assistive device that works best in large venues is the induction loop, hands down.) We want to be able to hear – even if it means “hearing” through captions – in an emergency room or hospital, in a courthouse, at a town hall meeting, at a house of worship, at a lecture, on an airplane, at a political rally, on public transportation.

We want to be able to work, and we want the accommodations that make that possible.

Hearing loss is a disability that prevents us from participating in corporate, municipal, religious, cultural, and educational life — unless accommodations are provided. Accommodations insure equal access.

We are guaranteed accommodations under the Americans with Disabilities Act. But if we want to claim those accommodations, we need to acknowledge not only that we have hearing loss but that it is a disability. (That’s the name of the act, after all.)

This notion was reinforced by the speaker at our HLAA-NYC chapter meeting last week. Dan Carione, a New York City police offer with an illustrious 28-year career, was forced to retire in 2011 when the NYPD decided officers could not wear hearing aids. He fought that ruling and won. (You can read about his four-year fight in an article published by Hearing Loss Magazine, or in a New York Times Op-Ed.)  But before he had any legal ground to stand on, he had to make an important admission to himself.

“The Americans with Disabilities Act is not this heroic shield that falls from the sky and protects each and every person who may or may not be disabled,” he said. “You have to be disabled.  That was very difficult for me to accept.”

Dan Carione does not look disabled. He was – and is – a powerful physical and intellectual presence. To use the word disabled about himself defied the visible reality.  “God bless Colleen [his attorney Colleen Meenan],” he said. “One of the first things she taught me was to use the word disabled.  It’s counter-intuitive.  Counter-intuitive, it hit me in the head like a dart because I didn’t want to use the word disabled.  But if you’re not disabled, the ADA can’t protect you.”

So if we want access equal to the access that hearing people have, we have to be open about our hearing loss. We have to acknowledge that it is a disability. That does not mean it’s disabling – it’s only disabling if we are denied the accommodations that make us equal.

For more information on living with hearing loss, see my books on Amazon.com.

Thanksgiving TLC

It’s Thanksgiving, the beginning of the holiday season. In rapid succession come Christmas and Hannukah, New Year’s Eve and New Year’s Day, not to mention office parties. Like many with hearing loss, I approach the holidays with some trepidation. The most difficult situation for me is a large holiday dinner.images.png 

Here’s my advice. Practice a little TLC.

T is for Technology

Assistive Listening Devices. Use them! These can be something as simple as a Williams Sound Pocket Talker or as sophisticated as a Phonak Roger system with multiple microphones.

The Pocket Talker is an amplifier with a microphone. The listener uses earbuds or a headset. It won’t allow you to hear the conversation at the other end of the table but if your neighbors speak into the microphone, you will hear them better These are available at many outlets. Amazon.com lists them at $138.95. The Pocket Talker works with two AAA batteries.

Your IPhone can act as an assistive listening device, using a detachable mic and wired headphones or earbuds with a downloaded app. My favorite tech guru Richard Einhorn recommends quality earphones or headphones. (You’ll probably have to pay $75 or so.) Richard also recommends the Jacoti ListenAPP or EarMachine. The external mic situation is in flux. Try a few and see what works best for you. Best Buy and other electronics stores should have options. Android phones have been slow to keep up with IOS systems. Try the Google Pixel 2, though I don’t know anyone who has used it, so can’t recommend.

If you’d rather have instant captions, try HearFree, also an IPhone app, and as the name says it’s free. It’s surprisingly accurate although if it’s a noisy room, the speaker will have to hold the mic close to his mouth. You can also use systems like Dragon Dictation.

Don’t forget the most basic tech fix of all: a pen and paper.

L is for Location.

The best location for a person with hearing loss depends on the nature of their hearing.

I hear better from my right ear, for instance, so I try to position myself with at least one strong voice to my right. That way I can ask that person what was said.

For others, sitting at the center of the table may allow them to hear the whole table better.

If there’s background music, sit as far from the speakers as possible, or ask the host to turn the music down — or, preferably, off.

If you end up in a particularly noisy spot, ask if you can change seats with someone. Often the easiest time to do this is between courses, or before dessert. Some hosts move guests around at a dessert as a matter of course, a social tradition that can be useful for the hard of hearing.

C is for Care.

There are lots of ways the word Care should be part of your Thanksgiving TLC.

First, take care in how you listen. I think of this as mindful listening. As I wrote in an earlier post, “Watch the impulse to say What? or ‘Huh? Think before you respond. What’s the context of the conversation? What parts of the sentence did you get? Is there a logical missing word? We always tell people with hearing loss not to pretend they’ve heard, not to guess. But guessing can be an effective strategy for getting someone to repeat in a way that makes the whole sentence comprehensible.”

In other words, when asking what was said, you’ll get a much more helpful response if instead of “What?” You ask “Did he say this butter is made in Mongolia?”

Take care of others. There a few things more emotionally rewarding than doing for someone else. Volunteer to serve dinner at a homeless shelter. Invite friends who may not have anywhere else go. I always like to include people newly arrived in America, who don’t know much about Thanksgiving. Often these are friends of my children’s, whose social network is much farther flung than mine.

Take care of yourself. When it gets too noisy, take a break in another room. Help serve and clear the table. Wash some dishes. Play with the baby. Go home early if you have to.

The T could also stand for Thankful. I hate to sound sentimental, but remember to be thankful for what you do have, rather than making yourself miserable about what you don’t, like good hearing.

 

For other observations about coping with the holidays, a hardy perennial for us bloggers, here are a few other posts from, in order, me, Shari Eberts, Ruth Bernstein, Arlene Romoff, and Gael Hannan: Holiday Hearing: You Can Do It; How to Enjoy Thanksgiving with a Hearing Loss; Holiday Madness; Hearing Tips for Happier Holidays; Happy Holidays for HOH’s.

For more on living with hearing loss see my books “Shouting Won’t Help” and Living Better With Hearing Loss, available Amazon.com.

 

 

 

 

 

 

 

Emergency Preparedness for the Deaf and Hard of Hearing

This past summer, people all over the world experienced floods, tornadoes, wildfires, earthquakes and terrorist attacks, not to mention a host of other catastrophes. This is an old column but I thought it was worth reposting. .

It goes without saying that everyone should be prepared with an emergency plan. But for people with hearing loss, being prepared goes beyond the usual.

The Centers for Disease Control and Prevention offers information on its website for emergency preparedness plans, and the American Red Cross suggests what you should have in your emergency kit.

For more specific advice for those with hearing loss, the New York City chapter of the Hearing Loss Association of America recently hosted a presentation on emergency planning for the deaf and hard of hearing. Representatives of New York City Emergency Management offered information about basic emergency planning, as well as these useful tips for adults with hearing loss:

Use “legacy technologies.” These include old-fashioned pen and paper for communicating with friends, family and emergency workers if you do not have access to your hearing aid or cochlear implant. Another legacy technology is a landline telephone. Even when cellphones, Internet, power and everything else goes, a landline may still work. Check with your provider.

Keep extra batteries in your emergency kit. Be sure to include batteries and chargers to keep your hearing aids, cochlear implants and assistive listening systems safe and working. You’ll want to have several packets of backup hearing-aid batteries and a sealed waterproof container in your emergency kit, for your hearing aid or cochlear implant. The bag or container should be big enough for extra batteries, chargers and assistive listening devices.
Don’t forget to include your medications, written copies of your medical information, your prescriptions, and your driver’s license and passport.
Consider a portable battery charger. Cochlear implants are more of a challenge during an emergency because their rechargeable batteries generally last at most about eight hours. This is where a portable battery charger — or two, if you want to be extra careful — may be useful. The chargers themselves need to be charged, however, so be sparing in how you use them.
Don’t forget your car as a power source. Even when all other power is out, your car (depending on the model, and as long as you have gas) will have some power for charging things like a cochlear implant battery pack, your cellphone and so on. These will be charged through the USB port.
Flashlights are a must. They are especially important for the hard of hearing. If it’s dark, you may need a flashlight to help in reading lips. Make sure you have them in your emergency kit and in a handy place in your home. Learn how to use the flashlight on your smartphone
Use Facebook’s Safety Check. If cellular phone service is still working, this feature allows Facebook to notify you in the event of a disaster in your area and ask if you’re safe. You click the “I’m safe” button and the message will go to your Facebook network. Facebook can also give you a list of friends who might be affected by the disaster. Facebook is selective in the disasters it covers with Safety Check, and has been criticized for implementing it in some disasters and not others, but it’s still a good way for the deaf and hard of hearing to check on friends and relatives.
Get a weather-alert radio. If you live in an area where tornadoes, hurricanes, floods, landslides or other natural disasters are common, you may want a NOAA (National Oceanic and Atmospheric Administration) weather-alert radio. They come in all price ranges, and a variety can be found by Googling “weather alert radio” or checking online at Amazon. If you are deaf or hard of hearing, you can activate a warning light. Some weather alert radios have an LCD screen for alerts.
Sign up for emergency alerts issued by your city or community. In New York that is Notify NYC.
Finally, for anyone who lives alone, hearing or not, form a support network. Ask two or three friends, neighbors or coworkers to be in your network so you don’t go through an emergency alone. Your network partners should make a plan to stay in contact during an emergency. They also should know your medical conditions and needs, and where to find emergency and medical supplies.

Vertigo, Redux

I haven’t posted for a while not because I was on a restful summer holiday but because I’ve been experiencing bouts of vertigo and dizziness over the past few weeks. Not only do these sideline me, they also affect my brain.Vertigomovie_restoration

They affect my memory, for starters. As I began to write this post I did a quick Google search and discovered that I wrote the exact column I was thinking of, under exactly the same title (“Deaf and Dizzy”), a year ago. If you want to know more about vertigo, Meniere’s and migraine-associated vertigo (MAV), you should read it. (My diagnosis is MAV.)

Memory loss is just one of the ways vertigo and dizziness mess with your head. During acute attacks, which in my case last for a couple of hours, I have to lie down with my eyes closed. If I open them a crack and see the room still spinning, I shut them again. If I don’t lie down soon enough, I vomit. Even sitting is no remedy. I have to lie prone.

In between acute attacks I’m dizzy. I walk unsteadily and sometime lurch, depending on the intensity of the dizziness. I look like a drunk. It’s not just memory that suffers. The brain seems generally confused. I pour orange juice into my coffee. I try to unlock the front door with my car key. I worry it’s a precursor to dementia but my doctor assures me it’s not.

I can’t detect a trigger for my vertigo, but luckily I recognize the symptoms of onset and can try to get to a safe place before it hits me flat out. For a few hours or even days preceding an attack, the back of my neck aches and the ache gradually spreads upwards. I feel pressure in my left eye. I experience hyperacusis. I may be a little dizzy. I’m tired. But it’s when my eyes swing to the right and the world starts spinning counterclockwise that I know I’m on the edge of vertigo.

Sometimes, the timing is not only inconvenient but dangerous — usually when I’m driving. Before and after a full attack, I can focus on the road ahead and make it to a safe place. Interestingly, focusing on one point is recommended as a way to control vertigo. I couldn’t possibly drive during a full-blown vertigo attack, but experience has shown me that I can get to safety — through what feels like force of will.

Alan Desmond, who writes the blog “Dizziness Depot” on Hearing Health and Technology Matters and is the director of the Vestibular Audiology Program at Wake Forest University, was hit with an episode of vertigo himself recently. He described how he used that focusing technique, training his eyes on a doorknob. I doubt he would ever endorse focusing on the road from behind the wheel of a car as a cure.

I watch my diet. Migraine-associated vertigo, also called vestibular vertigo, is triggered by many delicious foods, among other causes. The Meniere’s Diet is similar. If you look at the list at the banned foods, you may wonder if there is anything you can eat. The Vestibular Disorders Association recommends avoiding red wine, chicken liver, smoked meats, yogurt, chocolate, bananas, citrus fruits, figs, ripened cheeses (for example,  cheddar and Brie) and nuts. All these foods contain the amino acid tyramine. The association also recommends avoiding caffeine and alcohol. Sometimes salt intake is restricted. Aged meats are easy to avoid. Caffeine is not.

Like many with vertigo, I take preventive medication. For me it’s been a small dose of the tricyclic antidepressant amitriptyline, the generic for Elavil. (The dosage is much lower than would be used to treat depression.) That dose clearly had stopped being effective, so now I am taking a larger dose, still well below the antidepressant level. The fact that I’m writing this is an indication that the brain fog is starting to lift. I’m also signed up for vestibular therapy, which is a big commitment of time. But if it works it will be worth it, and I’ll be able to go off the medication.

The biggest mystery to me is that every doctor I have consulted – two neurologists and three balance specialists – says that my vertigo is not related to my hearing loss. I do not have Meniere’s Disease. I have migraine-associated vertigo (MAV) or vestibular vertigo.

MAV is only recently defined and was frequently misdiagnosed as a variant of Meniere’s in the past. The overlap between the two is substantial, and a review study published last year in the journal Behavioral Neurology, comparing the “intimate relationship” between migraine-associated vertigo and Meniere’s, suggests that the underlying causal mechanism may be the same for both conditions. I’ll discuss that study in my next post.

I’d like to know how others handle vertigo. Please comment. We can all learn from each other’s experience.

 

 

About Those OTC Hearing Aids

Thanks to Gael Hannan for inviting me to write about Over the Counter hearing aids for her column this week, on Hearing Health and Technology Matters.

http://hearinghealthmatters.org/betterhearingconsumer/2017/4790/

This puts together much of what I have written previously on the subject, in one tidy package.