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.”

*****

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.

IMG_2787 2
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.

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.

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.

Great News for People With Hearing Loss

Today the Senate passed the Over-the-Counter Hearing Aid Act, which HLAA has actively supported, in the belief that this will bring relief to millions of Americans who do not now have hearing aids and improve hearing services for people with all levels of hearing loss.

You can read HLAA’s press release here:

Senate Passes the Over-the-Counter Hearing Aid Act of 2017 | Hearing Loss Association of America

Thu, 08/03/2017 FOR IMMEDIATE RELEASE: August 3, 2017 CONTACT: Barbara Kelley Executive Directorbkelley@hearingloss.org 301.657.2248 Senate Passes the Over-the-Counter Hearing Aid Act of 2017 Bringing Affordable and Accessible Hearing Health Care Closer to Reality Bethesda, MD: The U.S. Senate today…

HEARINGLOSS.ORG

The Toll of Hearing Loss is Global

A new study published in the prestigious medical journal The Lancet offers an unblinking look at the tremendous burden of hearing loss worldwide. “Global hearing health care: new findings and perspectives” was published on July 10th. The authors are Blake S. Wilson, Debara L. Tucci, Michael H. Merson and Gerard M. O’Donoghue. The first and fourth authors organized a three-day discussion at Duke University on the subject, which was followed by a massive review of the existing literature.images

Half a billion people have disabling hearing loss, a number that is far higher than earlier estimates. This is not just a little trouble hearing the TV, your wife mumbles, this restaurant is too noisy hearing loss. This is disabling hearing loss.

“Disabling” means that 500 million people worldwide cannot hear well enough to learn to speak (if they are children), with resulting lower literacy and lower quality of life. If they are adults, “disabling” means they may have a sense of profound isolation, typically withdrawing from community and family, prone to psychological illness and likely to develop earlier and more severe dementia than their peers. “Association is not causation,” as the authors remind us, and in fact causation is the subject of a number of ongoing research studies into the hearing loss/dementia link. But the numbers are alarming: “Indeed, the hazard ratio for developing dementia increases two, three, and five times with mild, moderate, and severe losses in hearing, respectively.”

Eighty percent of those with disabling hearing loss live in low and middle-income countries, and their hearing loss has severe economic and personal consequences. But those in wealthy countries are not immune to these consequences. “In high-income countries… adults with disabling hearing loss have twice the prevalence of unemployment and half the median income of their normally hearing peers.”

The answer, the authors say, is twofold: prevention and treatment. Prevention and treatment of childhood hearing loss would be most effective in poorer countries. Special attention to adults would be more effective in wealthier areas.

Prevention could reduce prevalence by 50 percent or more in some regions of the world, according to the World Health Organization. These preventive efforts include vaccinations against rubella, measles and mumps; education and treatment of HIV, syphilis, hypertension and other conditions. It also includes maternal nutrition and neonatal care, attention to ototoxic drugs, and and universal hearing screening of infants. Chronic or acute otitis media should be treated promptly with antibiotics.

Treatment costs could be reduced by strategies like more competition and lower prices for hearing devices, a change in service provision – and “with disruptive and parsimonious designs” of hearing aids and cochlear implants.

These parsimonious and disruptive designs include many of the solutions now being discussed in the United States: the use of personal amplification devices (PSAP’s), smartphone apps, elimination of the need for a physician’s clearance, revision of regulatory requirements to allow “more competition and comparison shopping for hearing aids.”

Sound familiar? That’s because these are the very same recommendations made by the National Academies of Sciences, Engineering, and Medicine in June 2016, and the earlier PCAST report to the President.

The report calls for a global initiative to reduce “the currently unbridled burden of hearing loss.” It cites the interagency partnership VISION 2020, which began in 1999 with a goal of reducing avoidable vision loss by 2019. Indeed, as the study points out, disabling hearing loss is almost twice as common as disabling vision loss. In the category of mild to complete loss, hearing loss outnumbers vision loss by 46.2 v. 24.5 million in years lived with disability. Hearing loss is the fourth leading cause of disability worldwide.

The report is complex and fascinating and if you are interested in reading the full report please contact me via the comments section on this blog.

And if you suspect you have hearing loss, be grateful that you live in a country where you may be able to find treatment at a reasonable cost. Over the counter hearing aids will not become a reality for several years, but in the meantime get a hearing aid if you can afford it, try Costco or good online retailers for lower prices, get a PSAP if you can’t afford a hearing aid, try out some smart phone apps. But don’t ignore it.

shoutingwonthelp.jpg

For more information about living with hearing loss, read my book “Shouting Won’t Help: Why I and 50 Million Other Americans Can’t Hear You.