Ototoxic

Hearing Better Through the Ages

By Rebecca Huzzy, Au.D.

Chances are, you visit your doctor for an annual physical, wear a seatbelt, and use sunscreen. These are just a few small efforts we regularly make to stay healthy and injury-free.

Tending to the health of our hearing is another important, simple way we can maintain our overall physical and emotional well-being. Supporting hearing health begins at birth, when we test newborns for hearing loss, and continues into our elder years, when assistive technology can vastly improve overall health and quality of life.

Diagnosing Newborns & Infants

According to the Centers for Disease Control and Prevention, hearing loss is one of the most common congenital conditions, impacting approximately 12,000 infants per year. About half of these cases are linked to certain genetic syndromes, such as Down syndrome, Treacher Collins, and Usher syndrome.

But with the advent of universal newborn hearing screening programs in the early 1990s, hearing loss can now be identified and treated very early. According to what we call the “1-3-6” EHDI (Early Hearing Detection and Intervention) national goals, infants should be screened by age 1 month; diagnosed by age 3 months; and in an early intervention program by age 6 months.

“The effects of providing acoustic stimulation to the immature neurological system, including the brain, and combining the input with a rich and meaningful environmental experience, allows children to develop sufficient auditory skills to learn spoken language at a very young age,” says Janice C. Gatty, Ed.D., the director of Child & Family Services at Clarke Schools for Hearing and Speech.

This means families should expose their infants to sound frequently and consistently—talking to them, naming objects, narrating actions, singing, and reading books. With access to sound and an early intervention program at this young age, a child with hearing loss can begin learning to listen, babble, and eventually talk.

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Common Risks for Adolescents & Teens

Since the prevailing cause of hearing loss in young people with typical hearing is noise exposure, we need to educate kids early, as many begin listening to music on personal devices, playing in bands, and attending concerts at a young age.

According to the American Speech-Language-Hearing Association, exposure to sound that is higher than 85 decibels (the volume of a blender, hair dryer, or siren) for an extended period of time can cause permanent hearing damage. And the maximum output of most MP3 players is a powerful 110 decibels!

Fortunately, there are options for volume-limiting software that can mitigate unhealthy sound levels. Many devices offer parental controls and volume-controlling apps that limit noise levels, and there are various kid-friendly, hearing-healthy headphones available.

Follow the 80/90 rule: Set the maximum headphone volume to be 80 percent (not 100 percent), and listen for up to 90 minutes daily. If you listen for longer, lower the volume even more.

How Sound Exposure Catches Up With Us in Middle Age

“Adult onset hearing loss typically progresses slowly over the course of a number of years,” says audiologist John Mazzeo, Au.D., the audiology supervisor at Nemours/Alfred I. duPont Hospital for Children in Wilmington, Delaware.

Noise-induced hearing loss (NIHL) can have a sneaky, cumulative effect, similar to the impact of years of exposure to the sun. The people at the highest risk for NIHL work in noisy professions and include musicians, farmers, dentists, airport workers, and military service members. For those who spend time in loud environments, wearing hearing protection is the best way to guard against NIHL.

Ototoxic drugs (drugs harmful to hearing) and certain conditions, such as Ménière’s disease, can also contribute to progressive hearing loss over time. Regular screenings, prior to the recommended age of 50, are especially important if hearing loss runs in the family, or if you have symptoms associated with hearing loss, such as tinnitus, dizziness, or a perceived decrease in hearing.

Caring for Seniors as Hearing Abilities Change

Hearing loss becomes much more prevalent with age, affecting more than 30 percent of people over age 65, and 80 percent of adults over 80.

Hearing loss in seniors is linked to serious health conditions, including dementia. When communication is difficult, many people will avoid social situations, and research shows that social isolation is linked to cognitive decline, a key symptom of dementia. Additionally, difficulty hearing can impact the effectiveness of our other neural processes.

The risk of falls also becomes more likely with age, due to both decreased spatial awareness and increased cognitive load. A 2012 Johns Hopkins study found that older adults with mild hearing loss were nearly three times more likely to have a history of falling.

Staying Fit

If you’re diagnosed with a hearing loss, remember: Hearing loss is not only very common, it’s also very treatable! A licensed audiologist or hearing healthcare professional can discuss options with you, including hearing aids and assistive listening devices.

When it’s a loved one struggling to hear, or being stubborn about getting help, be patient. Gain their attention before talking, rephrase sentences instead of repeating them, and encourage trying out some kind of amplification.

Think of your hearing health as essential to your body’s complete performance. Our bodily systems are all interconnected; neglecting to protect our ears or refusing helpful interventions can have cascading health effects. When you take even small steps to protect your hearing health and that of loved ones, such as through regular hearing screenings and using earplugs in noisy environments, take heart in knowing you have bolstered your overall well-being.

Rebecca Huzzy, Au.D., CCC-A, is an educational audiologist at Clarke Schools for Hearing and Speech at its Philadelphia location and a clinical audiologist at Nemours/Alfred I. duPont Hospital for Children in Wilmington, Delaware. For more, see clarkeschools.org. This article also appeared in the Spring 2018 issue of Hearing Health magazine. For references, see hhf.org/spring2018-references.

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Untreated Hearing Loss Puts Overall Health at Risk

Hearing Health Foundation (HHF) CEO Nadine Dehgan’s “Treating Hearing Health for Better Overall Health” was published online to My Prime Time News following its original print appearance in The American Legion’s December 2017 issue.

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The article details how the state of the inner ear impacts other critical functions, like the heart and the brain. Cited are the various conditions that can arise as a result of untreated hearing loss, including cardiovascular disease, dementia, diabetes,  depression, and falls. When the auditory system is functioning well, however, the risk for these ailments declines.

Additionally, hearing loss is also linked to other medical conditions and drugs. People with anemia are twice as likely to have hearing loss. According to Peter Steyger, Ph.D., a scientific adviser to HHF. Further, certain cancer-fighting chemotherapy drugs, such as cisplatin, may permanently harm hearing.

While the relationship between hearing health and overall health is always significant, the publicity of “Treating Hearing Health for Better Overall Health” is an especially timely and helpful follow-up to ERG recipient Harrison Lin, M.D.’s new findings concerning the gaps between self-reported hearing loss and patients evaluation and treatments for hearing loss, which appeared in this month’s issue of JAMA Otolaryngology—Head & Neck Surgery.

Individuals who believe they may have a hearing loss are encouraged to consult an audiologist or ENT, and can learn more about the relationship between hearing health and overall health in the full article on My Prime Time News.

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A Fight Against Cancer Is a Fight Against Hearing Loss

By Frankie Huang

In honor of World Cancer Day on February 4, Hearing Health Foundation (HHF) wants to raise awareness of the connection between cancer and hearing loss. Every year, 8.2 million people worldwide die from cancer, a disease that is responsible for 13% of all deaths globally.

Depending on the type of cancer, patients that undergo chemotherapy are sometimes required to take certain drugs that could cause many side effects, including hearing loss. Cisplatin is a chemotherapy drug that is often used to treat testicular, bladder, ovarian and lung cancers. However, an excessive dose of cisplatin can be ototoxic (toxic to the ear), which could lead to temporary or permanent hearing loss.

One study suggested that cisplatin-induced hearing loss is generally bilateral (both ears) and irreversible. The study also found that cisplatin accumulates in cochlear tissue, preventing the cochlea from flushing out toxins. The same researchers found that patients receiving doses of cisplatin between 150-225 mg/m2 showed some degree of hearing loss. For testicular cancer patients, more than 50% of the patients that took cisplatin in doses greater than 400 mg/m2 had permanent hearing loss. Hearing loss may occur within hours or days after the treatment, or hearing may gradually decline after completion of therapy. After following up more than two years later, the study authors found that 44% of patients who took cisplatin had significant hearing loss.

In another recent study, researchers found that the WFS1 gene is associated with cisplatin-related ototoxicity; the heavier the dose, the more severe the hearing loss. Also, a mutation of the WFS1 gene results in Wolfram syndrome, a disorder with deafness as a major symptom.

As of now, there are no safe and protective agents against cisplatin, but scientists are hard at work to find a protective agent that would eliminate the negative side effects of cisplatin. Currently there’s a solution for children that are receiving cisplatin-based chemotherapy: The use of sodium thiosulfate may minimize or protect children and adolescents against cisplatin-induced hearing loss. HHF hopes more preventative therapies and cures for hearing loss can be discovered for all cisplatin-treated patients.

Interested in funding research in this area? Email us at development@hhf.org.

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Life-Saving Antibiotics Increase Risk of Hearing Loss

By Oregon Health & Science University

Approximately 80% of infants in neonatal intensive care units receive antibiotics known to be toxic to the ear. 

Seeking to stem the tide of permanent hearing loss from the use of life saving antibiotics, researchers at Oregon Health & Science University have found that patients stricken with dangerous bacterial infections are at greater risk of hearing loss than previously recognized. Inflammation from the bacterial infections substantially increased susceptibility to hearing impairment by increasing the uptake of aminoglycoside antibiotics into the inner ear, the researchers report. Their findings are published in online in the journal Science-Translational Medicine.

“Currently, it’s accepted that the price that some patients have to pay for surviving a life-threatening bacterial infection is the loss of their ability to hear. We must swiftly bring to clinics everywhere effective alternatives for treating life-threatening infections that do not sacrifice patients’ ability to hear,” said Peter S. Steyger, Ph.D.*, professor of otolaryngology, head and neck surgery, Oregon Hearing Research Center, Oregon Health & Science University School of Medicine. “Most instances in which patients are treated with aminoglycosides involve infants with life-threatening infections. The costs of this incalculable loss are borne by patients and society. When infants lose their hearing, they begin a long and arduous process to learn to listen and speak. This can interfere with their educational trajectory and psychosocial development, all of which can have a dramatic impact on their future employability, income and quality of life.”

Aminoglycosides, antimicrobials that are indispensable to treating life-threatening bacterial infections, are toxic to the ear. Relied on by physicians to treat meningitis, bacteremia and respiratory infections in cystic fibrosis, aminoglycosides kill the sensory cells in the inner ear that detect sound and motion.

Infants in neonatal intensive care units, or NICUs, are at particular risk. Each year, approximately 80 percent of 600,000 admissions into NICUs in the United States receive aminoglycosides. The rate of hearing loss in NICU graduates is 2 to 4 percent compared with 0.1 to 0.3 percent of full-term births from congenital causes of hearing loss.

When Steyger and colleagues gave healthy mice a low amount of aminoglycoside, the rodents experienced a small degree of hearing loss. If the mice had an inflammation that is typical of the infections treated with aminoglycosides in humans, the mice experienced a vastly greater degree of hearing loss.

The study lays the groundwork for improving the standard of care guidelines for patients receiving aminoglycosides. To shield patients’ hearing, the researchers called for the development of more targeted aminoglycosides and urged clinicians to choose more targeted, non-ototoxic antibiotics or anti-infective drugs to treat patients stricken with severe infections.

Due to their widespread availability and low cost, aminoglycosides are used frequently worldwide. Clinical use of aminoglycosides is limited due to the known risk of acute kidney poisoning and permanent hearing loss, yet are crucial life-savers in cases with potentially fatal infections.

Scientists who contributed to the OHSU study, “Endotoxemia-mediated inflammation potentiates aminoglycoside-induced ototoxicity,” include: Steyger; Ja-won Koo, M.D., Ph.D.; Lourdes Quintanilla-Dieck, M.D.; Meiyan Jiang, Ph.D.; Jianping Liu, M.D., Ph.D.; Zachary D. Urdang, B.S.; Jordan Allensworth, B.S.; Campbell P. Cross, B.A.; and Hongzhe Li, Ph.D.

This research was supported by: National Research Foundation of Korea grant 2011-0010166; Seoul National University Bundang Hospital 03-2011-007 (J.K.W.); National Institute of Deafness and Other Communication Disorders R01 DC004555, R01 DC12588 (P.S.S.), R03 DC011622 (H.L.), and P30 DC005983; and the Department of Otolaryngology at OHSU (L.Q.D.).

*Peter S. Steyger, Ph.D., is a prior Hearing Health Foundation board member and previous head of our Council of Scientific Trustees.

The above post is reprinted from materials provided by Oregon Health & Science University.

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Playground Lessons

By Scott Swanson

It's 1982. I'm 4 years old, and the proud new owner of two behind-the-ear hearing aids. These things are monsters. Huge. A result of life-saving surgery I had two years earlier. My mother had to put them in for me every morning. At age 4, I had no clue that this stinks. 

In 1983 I started kindergarten. In the Pacific Northwest, where I live, rain is a way of life. You don't duck for cover just because there's precipitation in the air. This especially goes for recess. At my school, there was this woman we called "Grandma" whose job it was to oversee the playground. She was old, and she laid down the law. She also rewarded kids who picked up toys with sugar-free candies. Those things were the worst! But some of us enjoyed the nice gesture.  



One fine September day it was raining and as usual, we got sent to the playground. I was getting pretty deep into a game of Foursquare, or maybe it was Red Rover. Either way, I find myself being sent inside by Grandma. Back to my classroom I went. I'm pretty sure I thought I was in trouble. Was I hogging the ball? Being mean? I couldn't figure it out.

School ended later that day, and I walked home in the rain. I remember when I walked through the front door my mom and dad were chuckling. They asked me how my day was. I gave them a generic response: "Fine.” "How was recess?" "Dumb." "Why?" "Grandma sent me back to my classroom for no reason." More chuckles, and then my mom told me to have a seat.

She told me that the school called to say they very concerned for my safety. She explained to me that Grandma noticed my hearing aids. She also noticed the rain. She put two and two together. When water and electronics mix, people get electrocuted. Grandma must have thought she saved my life. My parents told the school that although they appreciated them erring on the side of caution, I was not in danger of being electrocuted. A little rain would at worst damage my hearing aids but definitely not cause me to lose my life. 

I still see Grandma from time to time in the grocery store. I think back to that day and I doubt she remembers. She was old to us then, let alone 25 years later. Most importantly, I know she had my safety on her mind, and for that I'm thankful. I'll always remember her as much as any teacher I had at that school. Gross sugar-free candies, and all!

Scott has moderate to severe hearing loss. At age two, while undergoing life-saving surgery, Scott was administered ototoxic antibiotics, which have a side effect of hearing loss. By the age of four Scott had enough hearing loss to require hearing aids. He initially wore behind-the-ear (BTE) hearing aids, but currently wears completely-in-canal (CIC) hearing aids. Scott is the only one in his family that lives with hearing loss.

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The Link Between Your Kidneys and Your Hearing

By Judy Huch, AuD and Laura Friedman

March is National Kidney Month and today, March 12th, is World Kidney Day. Why is this important? For years we have been aware of rare syndromes involving renal disorder and hearing loss, such as Alport, MYHIIA, Muckle-Wells, Brescheck, and Bartter syndromes.1 But in October 2010, a study done in Australia showed a link between chronic kidney disease (CKD) and hearing loss, which was published in the American Journal of Kidney Diseases.

This study examined the “medical records of 2,564 people aged 50 and over, 513 of whom had moderate chronic kidney disease. Some 54.4% of all the patients with chronic kidney disease had some degree of hearing loss, as compared to only 28.3% of those who had no kidney problems.” Even more interesting, 30% of the CKD patients had a severe hearing loss compared to just 10% in those patients without CKD.

So what is the correlation between the CKD and hearing loss? According to researchers, "The link can be explained by structural and functional similarities between tissues in the inner ear and in the kidney. Additionally, toxins that accumulate in kidney failure can damage nerves, including those in the inner ear." Also, some treatments for kidney ailments are ototoxic, meaning they cause hearing loss. 

In the U.S., there are 31 million adults living with kidney disease, 7.5 million of whom have moderate forms of CKD.  Based on the recent findings it is important that these patients be aware that their hearing is also at risk. If you have patients or know anyone with chronic kidney disorder, please urge them to have their hearing tested annually to monitor any changes to their hearing status.

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1Toriello, H. V., Reardon, W., & Gorlin, R. J. (2004). Hereditary hearing loss and its syndromes. (Second ed., pp. 267-289). New York, NY: Oxford University Press.

A portion of this post originally written by Judy Huch, AuD, Editor of Hearing Health @ Hearing Health & Technology Matters.Other content was contributed by Healthy HearingThe American Speech-Language-Hearing Association, and Oregon's Deaf and Hard of Hearing Services.

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Emerging Research Grants for 2014 Announced

By Tara Guastella

HHF is proud to announce that 10 leading hearing scientists have been an awarded an Emerging Research Grant. It was an incredibly competitive funding cycle and it is a true honor for these investigators to have risen to the top and received this award.

Six grantees are first-year grant recipients and are studying areas such as noise-induced hearing loss, tinnitus, ototoxicity (hearing loss caused by certain drugs and medications), age-related hearing loss, and hearing aids.

Four previous grantees are receiving a second year of funding for their work. This group is researching such areas as central auditory processing disorder (CAPD), auditory physiology, cochlear implants, genetic hearing loss, and Usher syndrome.

One first-year grant recipient, whose work is funded by the continuing support of the General Grand Chapter Royal Arch Masons International, is aimed at developing better ways to assess auditory processing disorders. Here is an excerpt on his work:

Srikanta Mishra, Ph.D.

New Mexico State University

Medial Efferent Mechanisms in Auditory Processing Disorders

Many individuals experience listening difficulty in background noise despite clinically normal hearing and no obvious auditory pathology. This condition has often received a clinical label called auditory processing disorder (APD). However, the mechanisms and pathophysiology of APD are poorly understood. One mechanism thought to aid in listening-in-noise is the medial olivocochlear (MOC) inhibition— a part of the descending auditory system. The purpose of this translational project is to evaluate whether the functioning of the MOC system is altered in individuals with APD. The benefits of measuring MOC inhibition in individuals with APD are twofold: 1) it could be useful to better define APD and identify its potential mechanisms, and 2) it may elucidate the functional significance of MOC efferents in listening in complex environments. The potential role of the MOC system in APD pathophysiology, should it be confirmed, would be of significant clinical interest because current APD clinical test batteries lack mechanism-based physiologic tools.

Read more about the research all of the 2014 grant recipients are conducting.

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