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SOUNDSupport Please send your ideas for stories to: [email protected] Connecticut Children’s Medical Center Audiology Department Newsletter Volume 5 Issue 1 Saying Good-Bye GOOD-BYE Bob Benoit will be leaving his position as full-time audiologist at Connecticut Children’s Medical Center. Many families know that he has been a pediatric audiologist here since the Medical Center opened nineteen years ago. Prior to that, he worked for a number of years at Newington Children’s Hospital. Indeed almost all of Bob’s professional career has involved pediatric audiology. Please share with us a few thoughts on your career. Bob: Pediatric Audiology has always been my passion. Nothing against adult audiology but I always found working with children and their families to be both challenging and rewarding. During my career I have seen amazing progress in how we diagnose and treat hearing impairment in children. I have been fortunate to have practiced during this time. Let me give you a few “then and now” examples: Then… When I started out as an audiologist we diagnosed hearing impairment in young children by observing their reaction or lack of response to loud sounds. This was very subjective and not very accurate. We often needed to see children again and again before we knew just what they could hear. And now… We routinely obtain accurate and reliable test results on infants using measurements of brainwave activity in response to sounds. This allows us to appropriately fit hearing aids to infants as young as a few months of age. Then… Some of my first hearing aid fittings used large pocket-style aids about the size of a cell phone. Wires from the aid went to button receivers held in place by earmolds. Those cumbersome aids were not very adjustable. We made crude fitting adjustments with a screw driver. And now… Of course, now all aids are digital and much smaller. Our hearing aids are programmed with a computer and can be fine tuned to fit most any configuration of hearing loss. Aids now have special circuitry to reduce feedback, reduce background noise, wind noise and reverberation. They can be set to remind the user when to change batteries. They can use Bluetooth technology to wirelessly connect to phones, computers, music players, GPS etc. Then… A child with very profound hearing loss never had access to sound even with powerful hearing aids. A child with a very severe hearing loss might get only limited help from hearing aids. And now… An exciting option for severe to profoundly hearing impaired children became available when the FDA approved cochlear implants in 1990. Since that time many children have benefited from cochlear implantation. It has been gratifying to see how many of these children developed speech and language because of their cochlear implants. Then… Early in my career many children born with hearing impairment were not identified until age two or three years. With the development of automated hearing screening devices that changed. In Connecticut we formed a group of interested professionals who advocated for mandated hearing screening. Connecticut legislation was eventually passed and went into effect in 2000. And now… Universal hearing screening is the law of the land. This has reduced the age of identification from years to months. So many children born hearing impaired receive habilitation services at a very early age. Any other thoughts that you would like to share? Bob: Yes! I must add that I have been fortunate to have worked with so many great colleagues who were dedicated to providing quality hearing health care to our patients and their families. And your plans for the future? Bob: Actually, You will still see me in the Audiology Department from time to time. I plan to see patients on a very part time basis, when needed. Otherwise, my wife and I will continue our international travels when not working in the garden, fixing up our Victorian home, or expanding our antique business. In this Issue: Kidz Corner Research Update: Curing Hearing Loss Technology Update Walk 4 Hearing Anatomy Quiz Kidz Corner Hi, my name is Emalee Patterson. I am 11 years old and I am in 5th grade. I was first diagnosed with hearing loss in June 2012 when I was 8, which was the summer before 3rd grade. I did not realize that my hearing loss was not normal, that birds DO chirp, and doors DO squeak. I was fitted for new hearing aids the day I was diagnosed with the hearing loss. When my PURPLE hearing aids, and sparkly molds finally arrived in August 2012 I was excited about what I was going to hear, and so were my parents. I did feel nervous, more excited to hear everything like everyone else. Connie (my audiologist) gave me and my parents all the information needed about how to care for them. Then Connie put them on me, I remember how at first things sounded different (including my parents), but I got used to it quickly. When we were leaving Connecticut Children’s that day in August, we were walking across the parking lot and I heard an unusual sound I had not heard before…the noise seemed to follow me. I realized that the sound was coming from underneath me, and I looked down to see what it was…. It was my flip-flops! I looked at my parents and it all made sense to me of why flip-flops had their name…it is the noise they make when you walk! Discovering my hearing loss, and now having hearing aids has helped me so much with what I could not hear before. I am a dancer and my aids have helped me hear the music and the instructions from my dance teacher as well. I am so happy I got my aids! Thank you Connecticut Children’s, and Connie for all your help! By: Emalee Patterson Research Update: Curing Hearing Loss Often we are asked the question: “Will my child always need hearing aids?” For sensory neural hearing loss the answer has been “Yes”. Ongoing research, however, offers the possibility of hearing restoration in the future- perhaps in the lifetime of today’s children. Basic research is underway to explore the possibility for regeneration of sensory cells in the inner ear. Before we discuss this research let’s review how the ear works. Sound vibrations entering the outer ear canal travel down to the eardrum. The eardrum then collects and carries the sound vibrations to the middle ear bones. The middle ear bones in turn carry the sound vibrations to the inner ear, also known as the cochlea. In the inner ear the vibratory energy is picked up by tiny sensory cells, called HAIR CELLS. The human ear has about 18,000 of these tiny structures. Inner ear hair cells perform a vital function. When stimulated with vibration they cause the hearing nerve to generate electrical signals that travel into the brain and give the sensation of hearing. Simply put, without any functioning hair cells there is no hearing. Most sensory neural hearing loss is due to damaged or missing hair cells in the inner ear. Hair cells are very fragile structures that are easily damaged or destroyed. Loud sounds, certain medications and chemotherapies and some viral and bacterial infections can damage hair cells, causing hearing loss. The aging process also causes some loss of hair cells. In the case of childhood deafness hair cells may be damaged by birth complications or may be due to genetic factors. In fact, about half of all severe to profound hearing loss from birth is due to a genetic condition. This brings us to the ultimate research question: Can we find a way to regenerate damaged or missing hair cells in humans and thereby cure hearing loss? LEARNING FROM ANIMALS… Many years ago scientists thought that once sensory cells of the inner ear were destroyed, irreversible deafness was the result. More recently, however, it was discovered that SOME animals spontaneously regenerate destroyed hair cells and recover hearing. For example some birds and fish can, in effect, grow new hair cells after suffering inner ear damage. In a recent study chicks were given an antibiotic that is known to cause hearing loss as a side effect. Researchers did see hair cell damage in the chicks. Within weeks, however, new hair cells had replaced the damaged ones. But we did say SOME animals can regenerate lost hair cells. Mammals, including humans, do not have the ability to spontaneously replace destroyed hair cells. A recently published study showed that some regeneration of hair cells in mammals is possible with use of a potent drug. In that study adult mice were deafened by exposing them to high By Robert Benoit noise levels. Then the drug was injected into the middle ear where it could cross into the inner ear. The researchers did find that the drug triggered some regeneration of hair cells. This produced a slight improvement in hearing, especially for lower frequency sounds. How does regeneration happen? Underneath the hair cells are “supporting cells” that hold the hair cells in place. In some animals when hair cells are destroyed the underlying support cells develop into new functioning hair cells. (SEE DIAGRAM) Much research, like the mice study, is exploring how to stimulate supporting cells to develop into working hair cells in the cochlea. Drug therapy is one avenue of research. Another is gene therapy. Here, a harmless virus would be injected into a patient. The virus would carry and deliver a gene that would stimulate regeneration of hair cells in the cochlea. INTO THE FUTURE… Unfortunately, a true cure for sensory neural hearing loss in humans is not just around the corner. Early studies on animals raise as many new questions as they answer. Much more basic research is needed. The Hearing Restoration Project is at the center of research on cures for sensory neural hearing loss. Right now there are over a dozen laboratories in the U.S. and Canada studying how to regenerate hair cells as a way of treating hearing loss. For more information please go to the website: HEARINGHEALTHFOUNDATION.ORG Technology Update There’s an accessory for that! Wireless capability is quickly becoming the standard, not a luxury, in everyday life. Wireless technology has long been available to traditional hearing aid users. In March, Cochlear Americas announced full market availability of wireless accessories for select cochlear implant and Baha recipients. Wireless devices “connect” with each other by sending information to a device it has been “paired” with. Once paired, the sound processor will be able to receive a signal directly from a sound source, like a smartphone, computer, or television. The Mini Microphone, Phone Clip, and TV Streamer use the 2.4 GHz wireless protocol that Bluetooth and Wi-Fi devices depend on to provide a whole new world of sound to cochlear implant and Baha users. Mini Microphone • Transmits speech directly to the sound processor from a range of up to 23 feet. • Ideal for use in situations where noise, distance, or poor acoustics may be an issue, such as restaurant, lecture halls, or in the car. • Portable audio streaming device via plug and play line input. Phone Clip • Lightweight clip on device that allows By Melissa Mathieu, Au.D. for hands free connection to friends and family through smartphone. • Sends sound from a Bluetooth enabled phone directly to the sound processor and the microphone on the device sends sound back to the mobile phone. • Perform basic phone operations (i.e. answering and ending calls, adjusting the volume). • Basic remote control for Baha 4 patients by controlling the volume and program changing. • Wireless music streaming TV Streamer • Sends sound from a television or other audio source (i.e. computer, stereo) directly to the recipient’s sound processor. • Can connect one streamer to many users for group listening. These accessories are available for purchase on the Cochlear Store or contact your managing audiologist for additional information. Currently this technology is limited to cochlear implant recipients using an N6 speech processor and for Baha recipients using the Baha 4 or 5 systems. For these recipients, a simple software upgrade will be required to gain wireless compatibility. Please contact the Audiology department at 860.545.9642 to schedule this appointment. Walk 4 Hearing Hartford’s Annual Walk 4 Hearing event will take place June 13 at Mortensen Riverfront Plaza, 300 Columbus Blvd, Hartford, CT. The event kicks off at 9AM with registration, music and a pep rally. The walk will start at 10 AM and cover a distance of 5K. Join our team, CONNECTICUT CHILDREN’S WE ARE HEAR, or start your own team and make Connecticut Children’s Medical Center your alliance partner. Come help raise funds and community awareness of hearing loss! For more information please go to the following website: HLAA.CONVIO.NET Then search for Hartford Walk or Connecticut Children’s We Are Hear. We look forward to seeing you there! Maintenance Tip An important component of any hearing aid or cochlear implant processor is the MICROPHONE. Even the best hearing aid or implant processor will never work well, if its microphone is damaged or clogged. Here are a few tips for keeping your microphone working properly. • Never spray anything in the area of the microphone. We know you want to look great before going out BUT remove your aids before applying hairspray. Likewise remove your hearing aids or implant processors before using spray-on sun block, insect repellant or any other type of spray. • S ome hearing aid models and implant processors have protective filters covering the microphone openings. If yours does, change filters on a regular basis, usually every three to four months. Contact your audiologist if you need a supply of filters. • To keep moisture from building up in the microphone use a “dry-aid” kit on a regular basis, especially during humid weather. Anatomy Quiz The human cochlea contains about 15,000 sensory cells, known as hair cells, and about 40,000 nerve cells as well as thousands of supportive cells. The diameter of a human cochlear is about the same size as a a. Baseball b. Golf ball c. Walnut d. Pea The human body has over 200 bones. The smallest bone in the human is the middle ear bone called the stapes, also known as the stirrup. This bone has a length of about a. Three inches b. Two inches c. One inch d. One-tenth of one inch ANATOMY QUIZ ANSWERS: Correct answer for both: d NONPROFIT U.S. POSTAGE PAID HARTFORD, CT PERMIT NO. 3745 Connecticut Children’s Medical Center 282 Washington Street Hartford, CT 06106 A few parting words about Bob Benoit from his fellow audiologists “I will miss Bob rounding the audiology team up for lunch to make sure we get a break from our desks, his jokes and his help clinically when I had a difficult case. Enjoy retirement Bob!” I’m the worst joke teller. I started writing them down when I heard them so I could share them with Bob. I’ll continue to write them down and maybe I can text them to him when he gets that smartphone he’s been talking about. We’ll miss you Bob! Enjoy all the things retirement brings you! -Connie Miller Au.D. “While Bob has been fantastic to work with due to his easy going nature and strong work ethic, mostly I will miss Bob’s ever present sense of humor and the way he hums to himself in the office!” -Meg LaPointe M.S. CCC-A “Bob would come into my office each night before he left and tell me a joke. I always laughed, but never had one to tell. -Melissa Mathieu Au.D. I will miss Bob’s “parting jokes”. He always has a joke for us when he leaves for the day, his humor will be missed. -Courtney Ewell, Au.D. Bob and I have been office-mates for the better part of a decade now. I’m really going to miss being able to talk to him every week and I’ll miss his easy-going nature and positive personality. Bob never had a bad word to say about anyone. I’ll also miss his wonderful, yet specific, brand of humor. When I was getting married, he gave my husband a pair of socks wrapped up with a bow. We could not figure out what this present meant until Bob explained that he didn’t want Mike to get “cold feet”! -Lisa Rylant, Au.D. Bob brings a sense of calm to any situation with his easy going personality and unique sense of humor. Bob is always there to talk over a complex case and share his wealth of knowledge. We will miss him greatly! -Julie Foulis, Au.D. DIABETES & ENDOCRINOLOGY UROLOGY For future editions of Sound Support please submit your questions, comments and ideas to [email protected] 282 Washington Street, Hartford, CT 06106, © 2015 Connecticut Children’s Medical Center. All rights reserved. New 4-15 WWW.CONNECTICUTCHILDRENS.ORG