Entries in Hearing (15)


With Hearing Implants, Experiencing Sound for the First Time

iStockphoto/Thinkstock(NEW YORK) -- After being born severely hearing-impaired, Sarah Churman heard her first clear sound at age 29 and promptly burst into tears.

“I hear Melinda say, “How does it sound?” Churman wrote in her memoir, Powered On, about the experience. “I start to answer her, and I realize I can hear the noises in my mouth. Then I realize how I sound. Then I get choked up. Then I laugh. Then that sends me into a fit of tears and choking up.”

Churman received cochlear implants at age 29. A video of her implants being turned on for the first time went viral in 2011.

Cochlear implants, which help people hear by electronically simulating the auditory nerve, have been used by 30,000 people worldwide, according to the American Speech-Language-Hearing Society.

With the spread of cochlear implants, videos of deaf people hearing for the first time have become a staple on such video sites as YouTube and Vimeo, garnering millions of views.

In the video shot by her husband, Churman lights up when she hears the noise for the first time and then quickly starts crying and laughing as she says, “This is weird.”

But what is it really happening when people gain a new sense?

The cochlear implant is not a replacement for an ear, but it can help many people who were effectively declared deaf. By stimulating the auditory nerve, signals are transmitted to the brain, which turns into “hearing.”

Although Churman wrote that she loved hearing and her cochlear implants, Dr. Daniel Lee, director of the Pediatric Ear, Hearing and Balance center at the Massachusetts Eye and Ear Infirmary, said many other adults sometimes describe the sound as metallic or robotic at first.

“[Usually you] are communicating through thousands of channels of information that are being sent to your brain,” said Lee. “These implants have no more than 22 electrodes.”

Lee said that the implants are key for many children or infants born with severe hearing loss who receive the devices so that they can grow up with auditory skills and have stronger language skills.

Doctors, however, have to be careful when exposing small children to sound for the first time. Since the children often do not have the means to communicate, doctors have to be very careful that the children have a positive experience and aren’t frightened by their new sense.

Lee said the key is to keep the electronic data to a minimum in the beginning of using the implant.
“[You] don’t want to overwhelm them,” said Lee. “Over a period of days to weeks to months [the implant] is slowly ramped up to provide more information to the ear or the brain.”

In videos usually a family member speaks to the child for the first time. In one memorable video an infant responds to hearing his mother’s voice by dropping his pacifier and looking at her in awe.

While cochlear implants have significantly helped people who were previously profoundly deaf, they do not work for people who either lacked an auditory nerve or had a damaged nerve.

But new technology is now being used that sends messages directly to the brain itself. A Food and Drug Administration clinical trial currently underway is looking at the effectiveness of electrical implants placed directly on the brain stem.

Placed directly on the brain stem, it bypasses all auditory nerves.

Grayson Clamp made headlines earlier this week for becoming the first child in the U.S. to receive the procedure.

Lee, who is involved in the trials but not in Grayson’s case, said that the auditory brain stem implants could be used to help deaf children communicate and develop lip reading skills. He said once children like Grayson are older, they can see how effective the devices are by evaluating their language skills.

But even before the implants can be measured and quantified, Grayson’s immediate response delighted his father, Lee Clamp.

“It was phenomenal to see him take that sound in and try to figure out what in the world is this? I’ve never had this sensation before,’” said Clamp.

Copyright 2013 ABC News Radio


Pharmacy Owner Takes Fifth in Meningitis Hearing

Chris Maddaloni/CQ Roll Call(WASHINGTON) -- The owner and co-founder of the New England Compounding Center, the pharmacy at the center of a deadly fungal meningitis outbreak, declined to testify before a congressional hearing Wednesday.

When pressed by members of Congress on his role in ensuring safe and sterile products, Barry Cadden invoked his Fifth Amendment right.

“On advice of counsel, I respectfully decline to answer on the basis of my constitutional rights and privileges including the Fifth Amendment of the Constitution of the United States,” he said at the hearing in Washington, D.C.

The House of Representatives had subpoenaed Cadden to the hearing to address the outbreak that has sickened 461 people in 19 states and killed 32. The outbreak has been traced to contaminated vials of methylprednisolone acetate, an injectable steroid used to treat back and joint pain made by Cadden’s pharmacy.

But the Subcommittee on Oversight and Investigations panel became clearly frustrated with the testimony of U.S. Food and Drug Administration Commissioner Dr. Margaret Hamburg.

Republican lawmakers noted inspections of NECC going back to 2002 that found sterility issues at the facility, and asked why the FDA had not taken action against the pharmacy.

“You are in charge of the FDA. You are the chief honcho. You’re the great Pooh-Bah of the FDA and I’m asking you, basically, could you have prevented this tragedy? And you are saying you can’t because you didn’t have jurisdiction?” said Rep. Cliff Stearns, R-Fla.

“I think it is very hard to know if any one action that we might have taken could have stopped this terrible tragedy,” Hamburg replied, adding that in her opinion, the FDA did nothing wrong.

The commissioner told the panel that her agency needed stronger authority over “compounding” pharmacies.

“The challenge we have today is that there is a patchwork of legal authorities that oversee the action we can take,” said Hamburg. She said there were “gaps” and “ambiguity” in the FDA’s authority, and described a “crazy quilt” of laws.

Compounding pharmacies traditionally fill special orders placed by doctors for individual patients, turning out a small number of customized formulas each week. NECC, however, acted more like a manufacturer by filling thousands of prescriptions and shipping across state lines causing confusion in the FDA’s jurisdiction.

Republicans were visibly irritated at Hamburg’s lengthy responses to their yes or no questions, at certain points even chastising the FDA commissioner for her responses.

“Can you ever give straight answer to a question?” Rep. Joe Barton, R-Texas, asked, while Stearns reminded Hamburg  that she was under oath.

Joyce Lovelace spoke about her husband Eddie, who died from fungal meningitis in September after receiving a tainted steroid injection, saying she wanted “people to know what kind of person has perished because of their lack of concern.”

“My family is bitter. We are angry. We are heartbroken. We’re devastated and I just come here begging you to do something about the matter,” Lovelace said. “It was a nightmare to see this man who was perfectly healthy one moment and then just so quickly going downhill and everything the doctors were doing for him was to no avail. The medicine, whatever they did, it was not helping him in the least.”

Lovelace begged the committee to “legislate this.”

“Whoever is responsible, I want them to know their lack of attention to their duties cost my husband his life, cost my family…It may not appear to be anything to you, but you are affecting valuable human lives,” she said. “I cannot beg you enough. Bi-partisan. I don’t care what party, work together and please legislate this so no family has to go through what we have.”

Copyright 2012 ABC News Radio


LISTEN: Scientists Find Harshest Sound in the World

Jupiterimages/Thinkstock(NEW YORK) -- Scientists studying the brain's reaction to sound have determined the piercing screech of a knife against a glass bottle to be the human ear’s worst nightmare.

A research team at the University of Newcastle examined brain activity in a group of volunteers, who listened to 74 recorded sounds under a MRI machine and ranked each experience.

The 13 subjects found the sound of a fork against glass to be the second most repulsive, while sounds like flowing water were much easier for the brain to bear.

In order to establish why, the researchers focused on the ear’s acoustic rather than associative reactions.  Sukhbinder Kumar, the study’s author, said he and co-author Tim Griffiths chose sounds that do not call to mind any negative emotional experiences, unlike a separate 2007 study that tested more evocative sounds and found that the sound of vomiting to be the most universally appalling.

The scans revealed that the unpleasant noises not only increase blood flow in the brain’s auditory cortex, where sound is processed, but also trigger the amygdala, a primitive region of the brain that processes emotions.

“The new finding in this study is to precisely define the loop between the auditory cortex and the amygdala,” Griffiths said.  “We were able to define a network within the brain that constitutes sound unpleasantness.”

The most disagreeable sounds had frequencies of 2000-5000 Hz, the part of the spectrum at which our ears are most sensitive, Kumar said.

While it is unknown why this range is most difficult for the human ear to tolerate, specific sensitivities like this one could have evolutionary advantages, Griffiths pointed out.

“Because this is such a fundamental level of unpleasantness, it could be a sort of alerting and arousing system in the brain,” he said.

The study, which was published in the Journal of Neuroscience, will help scientists understand what makes certain people more sensitive to disagreeable sounds than others, Kumar said, potentially leading to new insight into disorders like misophonia, a severe sound intolerance, and the more common tinnitus, which Kumar is currently studying.

Copyright 2012 ABC News Radio


US Prevention Task Force Eases Guidelines on Hearing Tests

Comstock/Thinkstock(WASHINGTON) -- Twenty to 40 percent of adults older than 50 -- and more than 80 percent of adults older than 80 -- show some evidence of hearing loss.  It can negatively affect a person's quality of life and the ability to live independently.

Even so, on Monday the U.S. Preventive Services Task Force posted a final guideline for hearing-loss screening in older adults.  In contrast to its 1996 guidelines, which recommended asking all older patients about hearing, the Task Force now says it can't recommend for or against it in patients not complaining of hearing loss.

"The Task Force recognizes this is important and common," Dr. Albert Siu, vice co-chairman of the Task Force, said.  "We also know for people who complain of problems with their hearing, hearing aids can be beneficial.  However, we don't believe there is sufficient evidence that older adults who don't have complaints should be screened."

The recommendations from the USPSTF are often used by doctors to determine what screening tests they should be conducting on certain patients.  Time, costs, the balance between risks and benefits, and study findings determine whether or not screening tests are recommended.

"Guidelines are important for the purpose of deciding what is the best way to practice," said Dr. Hamid Djalilian, director of neurotology at the University of California, Irvine.  "This guideline doesn't provide a recommendation one way or the other."

When it comes to screening for hearing loss, the tests are simple and can be performed in the office in just seconds.  So on the surface, it may seem like a slam dunk for a recommended test.

That being said, the costs of additional testing and hearing aids, which generally aren't covered by Medicare, can be very expensive.  Moreover, many hearing loss experts agree that there simply is not enough research in the area to make a solid recommendation on the tests.

Still, the impact of hearing loss on day-to-day living can be profound.

"Hearing loss may mimic dementia or depression and can lead to social isolation," said Dr. Jennifer Smullen at the Massachusetts Eye and Ear Institute.

And Dr. Frank Lin, a hearing-loss expert at Johns Hopkins Center on Aging and Health, said hearing loss could not only mimic dementia but even mild hearing loss could double the risk of dementia.

"We are increasingly recognizing that hearing loss in older adults is associated with real, detrimental consequences on poorer cognitive functioning and the risk of dementia," Lin said.  "We don't know yet if treating hearing loss could have an effect on these outcomes, but hearing loss screening and treatment are interventions that carry essentially no risk."

Copyright 2012 ABC News Radio


Cord Blood Stem Cells Restore Toddler's Hearing

Courtesy Stephanie Connor(LABELLE, Fla.) -- A virus infection Stephanie Connor acquired during pregnancy put her unborn daughter at significant risk for brain damage and lifelong hearing loss.

"It was traumatic," said Connor, of LaBelle, Fla., after learning about her daughter's condition.  "It was like mourning the loss of a child."

At age 1, baby Madeleine was completely deaf in her right ear and her hearing was severely lost in the left, said Connor.  While a hearing aid helped to amplify some sounds for Madeleine, it would never fully repair the damage in her ear.

But a simple experimental procedure that Connor enrolled in for Madeleine may have restored her hearing and reversed her condition.

In January 2012, Madeleine, now 2, became the first child to undergo an experimental hearing loss treatment through an Food and Drug Administration-approved trial at Memorial Hermann-Texas Medical Center that infused stem cells from her own banked cord blood into her damaged inner ear.  Within the last six months, Connor says she's seen a dramatic improvement in Madeleine's ability to hear.

"Before, when she would hear something she would look all around," Connor said.  "But now we notice that she turns in the right direction of the sound."

Madeleine was also able to speak for the first time, Connor said.

For more than two decades, umbilical cord blood transplantation -- either by a baby's own cord blood or another's, depending on the type of procedure -- has been used to treat otherwise fatal diseases including blood disorders, immune diseases and some types of cancers.

Infusing cord blood stem cells into the body may also have the potential to heal and regenerate damaged cells and tissues.  

Regenerative therapy using cord blood stem cells is currently being studied as therapies to treat conditions including cerebral palsy and brain injury.

And for the first time, doctors are experimenting with cord blood stem cells to regenerate hearing in children who have suffered hearing loss.

This year-long study will follow 10 children, including Madeleine, ages 6 weeks to 18 months, who have acquired hearing loss and who have donated their cord blood to a registry.

Madeleine has already had one follow-up appointment to test her speech and language development, which are indicators that her hearing has improved.  She will have another one mid-July.

Dr. Samer Fakhri, associate professor and program director in the Department of Otorhinolaryngology at Memorial Hermann-Texas Medical Center, and principal investigator of the study, said it's still too early to determine whether the procedure benefited Madeleine, or may be beneficial for other children.

Copyright 2012 ABC News Radio


Deaf 10-Year-Old Regains Hearing with Cochlear Implant

ABC News(DALLAS) -- The sound of Sammie Hicks's own breathing moved her to tears three weeks ago.

That's because it was the first time she ever heard it.

"I started to cry because it was overwhelming," 10-year-old Sammie told ABC affiliate WFAA. "I had no idea what the sounds were."

Born with a genetic mutation that caused her to lose her hearing as a toddler, Sammie was fitted with a cochlear implant – a kind of bionic ear that simulates hearing – and documented the process in an online video diary over the past several months.

In a video posted Wednesday from her home in Collin County, Texas, Sammie mimes robot arms and flashes a braces-clad smile when her mother asks what the implants sound like to her. The implants don't exactly mimic hearing, so Sammie thinks voices sound like robots.

Unlike a hearing aid, which amplifies existing sounds, the cochlear implant is designed to directly stimulate the auditory nerve, bypassing the damaged part of the ear. First, an implant is surgically placed beneath the skin. Three weeks later, it is turned on and works with an earpiece to process sounds and stimulate the nerve.

"When a cochlear implant is turned on, people will hear things they've never heard before," said Dr. Jennifer Smullen, who has performed hundreds of implant surgeries at Massachusetts Eye and Ear Infirmary. "It's like someone who really needed glasses, put them on and realized they were missing the sunset."

Hicks's new favorite activity is taking long walks outside so she can listen to the birds, her mother, Jenifer, told ABC News. Other sounds are more annoying, on the other hand, like the air conditioner and the sound of her classmates turning pages.

"She wasn't expecting school to be quite so loud," Jenifer said. "She can hear everyone eating their snacks and writing on paper. And she can hear them breathing."

Sammie's younger brother, 9, went deaf "rapidly" over the last two years and just had the same cochlear implant surgery, Jenifer said. His implants will be turned on June 7.

"When we brought him home from surgery, she broke down in tears because she knew what he was going through," Jenifer said. "She wouldn't leave his side."

In Wednesday's video diary entry, Jenifer asked Sammie if she had any advice for her brother.

"After it gets turned on, it's not going to be what you expected, of course," Sammie said. "If you jump around, the thing will fall off."

Sammie's brain learns how to process the information from the implants every day, Smullen explained. And as she learns, she'll update her video diary and share new sounds with the world.

Copyright 2012 ABC News Radio


Teen with Misophonia Can't Be Near Mom Without Risking Rages

ABC News(NEW YORK) -- They are a mother and daughter who consider each other best friends.  And yet, Bernadette and her 14-year-old daughter, Taylor, who asked that 20/20 not reveal their last names, don't eat meals together, don't share any activities and don't even speak to each other.  It's all because everyday sounds Bernadette makes -- clearing her throat or sighing -- can send Taylor to the brink.

"It's like an almost undescribable amount of anger and, like, rage that I just can't control," Taylor told 20/20.

Taylor suffers from misophonia, a mysterious condition whose name literally means "hatred of sound."  Misophonia makes it difficult to tolerate everyday noises such as chewing, coughing, even breathing.  And while many might say they get annoyed at such sounds, for those with misophonia, the consequences of hearing such "trigger" noises are far worse than mere irritation: violence, isolation, depression and even thoughts of suicide.

Taylor has attempted suicide three times -- attempts, she said, were triggered by anti-depressants that did nothing to help her misophonia.

"I don't want her to give up, 'cause she's tried to give up," said Taylor's sister, Alex.  "I just want her to keep moving."

Taylor's symptoms began when she was 8-years-old.

"I coughed, and she covered her ears, and she ran away," Bernadette said.

Eventually, it grew much worse.

"She's hit my head against the wall.  She's kicked me.  She's pushed me," Bernadette said, "just whatever she can [do] to stop the sound from coming from me. "

Once the rages pass, Bernadette said Taylor immediately feels remorse for her behavior.  But the teen told 20/20 that when in the grips of such a rage triggered by an offending sound, that "sound will replay in my mind until I get the anger out ... until I somehow get out all my frustration."

Audiologist Marsha Johnson of the Oregon Tinnitus and Hyperacusis Treatment Center is one of the only experts who treats misophonia patients.  Johnson said the cause of misophonia is suspected to be a "neurological glitch in a very low level of the brain."

Watch the full story on 20/20 Friday at 10 p.m. ET.

Copyright 2012 ABC News Radio


Rare Condition: College Student Suffers From ‘Hatred of Sound’

iStockphoto/Thinkstock(NEW YORK) -- For Emma Riehl, attending her college classes is a form of torture.  But she doesn’t blame the subject matter or the homework.

Riehl, 19, lives with a rare and still mostly unknown condition called misophonia.  Meaning “hatred of sound,” misophonia makes it difficult to tolerate everyday sounds such as chewing, coughing, even breathing.  Those who have it find the noises so intrusive that they can’t remain in the same area as the person making them.

Unlike people who find these noises merely irritating, people with misophonia have an extreme reaction that often leads to lives of isolation.

The specific sounds of sniffling and chewing make Riehl feel anxious, distressed and violent.  In her video diary, she describes her daily struggle to overcome the rage she feels whenever she hears these “trigger” sounds.

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Unable to participate in the typical college social scene, she lives alone and constantly wears headphones.

There is no cure for misophonia, but Riehl believes that eating a healthy diet and keeping to a strict schedule of exercise help her ease the stress caused by her condition.

Although there has been limited research, some experts believe misophonia has a genetic link and could result from a neurological defect.

Copyright 2012 ABC News Radio


Science Behind the 'Cocktail Party Effect'

Allan Danahar/Thinkstock(SAN FRANCISCO) -- It’s a familiar scene: you’re at a crowded party, but despite all the noise, you’re still able to make out the words of the one person with whom you’re talking.

Researchers from the University of California, San Francisco studied this phenomenon, known as the “cocktail party effect,” and found it has both psychological and neurological components.  Their data is published in the latest issue of the journal Nature.

What happens from a neurological standpoint is that the sounds all enter the ear as one cacophonous roar, but the brain processes all the information and tunes into one sound, such as a person’s voice, and filters out the rest.

“The psychological component is that it’s a sound we want or need to hear, which is why we can tune into it,” said co-author Dr. Edward Chang, an assistant professor of neuroscience.

Chang and postdoctoral scholar Rima Mesgarani looked at this effect in three subjects who were undergoing treatment for epilepsy.  All three had normal hearing and were able to process speech normally.  The authors hooked the subjects up to electrodes and asked them to listen to two speakers, but only focus on one.

By measuring brain activity, they were able to determine what the subjects heard, and found that their brains responded to the targeted speaker.

This line of research is important, Chang said, because it can contribute to the understanding of how language processing is impaired in people with attention deficit disorder, autism, disorders involving the learning of language and deficits that occur as people get older.

“People with these disorders have problems with the ability to focus on a certain aspect of the environment,” Chang said.  “They can’t always hear things correctly.”

Understanding how the brain processes the human voice can also contribute to the development of new technologies that rely on voice recognition, the authors said in a university press release.

Copyright 2012 ABC News Radio


Millions 50 and Older Suffering Hearing Loss But Not Using Aids

iStockphoto/Thinkstock(BALTIMORE, Md.) -- Of the 4.5 million 50- to 59-year-olds in the United States experiencing hearing loss, only about 4.3 percent are using hearing aids.

“These people are still working and going to meetings,” said Dr. Frank Lin, assistant professor in the department of otolaryngology and epidemiology at John Hopkins University. “They are the people who need it the most.”

In “The Prevalence of Hearing Aid Use Among Older Adults in the US,” which was published Monday in the Archives of Internal Medicine, Lin and Dr. Wade Chien, also of Johns Hopkins, found that of the 26.7 million Americans age 50 and older suffering from “clinically significant, audiometrically defined,” or real, hearing loss, just one in seven used hearing aids.

For the publication, the two examined data from the National Health and Nutritional Examination Surveys, which has collected health information from thousands of Americans since 1971.

Lin said there were several reasons for the gap between those suffering hearing loss and those using hearing aids. He said that hearing aids were rarely covered by medical insurance in the U.S., but noted that even in parts of the world where aids were covered, the rate of people using them was not much higher than that of the U.S.

“The biggest thing is the overall perception that hearing loss is an inconsequential part of aging,” Lin said.

He said that because of the perception, people felt there was nothing they could do to treat hearing loss and little research was done on the condition.

Pam Mason, the director of audiology professional practices at the American Speech-Language-Hearing Association, said that many Americans 50 and older didn’t know the dangers of untreated hearing loss.

“Those that have mild or even moderate hearing loss may tell themselves: ‘I can get by,’” she said. “Somebody who has been a typical hearing person...if the hearing loss has been creeping on them...may not be aware that they are experiencing hearing loss. They don’t recognize they are having a problem.”

Both Lin and Mason said that ignoring hearing loss had broader, negative consequences. It’s been associated with poor thinking and memory ability and can lead to social isolation, depression and even dementia.

Lin said that most people 50 and older who did get hearing aids stopped using them because of improper counseling and training.  As with prosthetic devices, he said, hearing aids required two or three months of auditory rehabilitation to use them properly.

“They’re complex devices,” he said. “It’s not like putting on eyeglasses.”

Mason said that treating hearing loss was a process.

“It may even include auditory training, retraining your brain. It may include lip-reading skill improvement, recognizing how sounds look on the face,” she said. “Everybody is unique. Hearing needs are unique.”

“The most important parts for this population of people [ages 50 and older] is to recognize the signs of hearing loss and understand the negative consequences of untreated hearing loss and where to go for help you may need,” Mason said.

Copyright 2012 ABC News Radio

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