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Cochlear Implant Program gives gift of sound  by Heather Woolwine Public Relations Tim Newsome admits he and his wife, Corey, weren’t prepared to learn their son, Cooper, had severe hearing loss.  “He passed his newborn hearing tests, but he never really babbled or made many sounds,” Tim said. “When he was evaluated at six months and didn’t exhibit some of the verbal and behavior developments that we should’ve seen, we realized what was happening. After a lot of crying and the shock, we came together to figure out what the best thing for him was going to be and did it.” Tim and Cooper Newsome take a break from playing during the annual picnic, April 2. The Newsomes did their homework and ended up in MUSC’s Cochlear Implant Program, directed by Ted Meyer, M.D., Ph.D., assistant professor of otolaryngology.  Paul Lambert, M.D., Otolaryngology chairman, fit Cooper with hearing aids to determine how much auditory benefit he would receive. After several months of hearing aid use, Cooper was demonstrating little-to-no benefit from the hearing aids so the decision was made to implant Cooper. “We tried the hearing aids but they just didn’t work,” Tim said. “So we set a date for the surgery this past December, right before Christmas.” At just a year old, little Cooper was implanted with one of three FDA-approved cochlear implants on the market today. More than three months later, he now babbles, says “mama,” and discovers knew sounds daily. “It may sound strange but I noticed he was making progress the other day when I sneezed and he began to cry,” Tim said. “I mean, no one wants their child to cry, but in this case, I knew that he was scared because he’d been able to hear the noise for the first time. Every time he makes a sound we repeat it back to him, and he also has a 3-year-old sister who contributes to his language and hearing development all the time,” he joked. Like Cooper, patients who receive cochlear implants are gradually reintroduced to a world with diverse sounds after surgery.  Abby Connell, audiologist and clinical coordinator of the program, gets to know her patients well through the numerous follow-up visits after implantation. In addition to the implant, most patients continue to wear hearing aids for an added hearing advantage. “Some patients, however, will stop using the hearing aid after a while because the implant works so well,” Meyer said. “There are many physicians in Europe right now that believe in providing two implants for each patient with profound hearing loss, although most insurance companies won’t cover two devices here in the states. There is ongoing research that having two implants provides a patient with some advantages, including better signal detection within noise and better ability to localize sounds.” Meyer mentioned the improvements made in terms of the actual device itself within the last few years, now rendering them more flexible and faster at processing sounds. In addition, Connell noted changes in FDA guidelines for who is eligible to receive an implant, recently loosened so that those with some residual hearing have a better shot at receiving an implant if hearing aids just aren’t doing the trick. “There are no real cut offs for age within the guidelines,” Meyer said. “We have implanted children as young as one year and adults as old as 92. What’s important to determine in each potential implantation case is that other means of hearing were unsuccessful and we’re doing what’s best for each patient.” In terms of implanting young children, Meyer sees an advantage to patients with profound hearing loss receiving an implant so young because implants work best in terms of oral communication development. “Most children with a profound hearing loss who receive a cochlear implant can be mainstreamed into regular schools, although there is still a need for speech and language therapists as well as other health professionals to help care for those children,” he said. “And some parents decide not to mainstream and will send their child to a school for the deaf. There is still controversy within the deaf community concerning implants but ultimately our role is to listen to the child’s parents and accommodate the child’s needs based on what the parents believe is best for their child. Our standpoint is that we want to make communication easier for people with profound hearing loss.” Current research suggests that language development after implantation in children who receive implants at earlier ages parallels that of language development in children with normal hearing. For adults, the need for an implant can come after years of hearing aids and gradual hearing loss or even after a sudden hearing loss, with any number of medical causes.  Time is usually of the essence and especially in the situation where hearing aids fail a patient. “The implant doesn’t work as well for long-term deafness but most adults with good hearing and normal speech and language development earlier in life have up to a 75 percent chance of being able to talk on the phone after surgery,” Meyer said. Meyer and Connell recognize there are some misconceptions about implantation. “Most people come in the door expecting that an implant is going to totally restore normal hearing and I have to explain to them that it is not an ‘Extreme Makeover’ kind of process,” Connell said. “It’s not a quick fix. It takes a lot of patience to make it work and will not fix things that aren’t related to hearing loss. If your child had ADHD before, he’s going to have ADHD afterwards. But with hard work and effort involved on everyone’s part, implantation works out well.” Like any process, implantation adheres to guidelines that must qualify patients in the first place. Patients must receive less than a 50 percent score on word recognition tests that Connell administers and both children and adults must have bilateral, severe-to-profound hearing loss.  Aside from federal implantation regulations, Connell and Meyer don’t consider candidates who aren’t motivated or who have unrealistic expectations. Parents of implant candidates must be willing to carry over everything needed for the child’s development at home, in addition to providing for extensive speech, language and listening therapy. “We do try to bring in other professionals to deal with denial issues and conduct psychological evaluations to reinforce our stance,” Connell said. For patients with extreme mental deficits or multiple handicaps, the goal of developing spoken language after implantation must be kept in mind. “While we must consider whether or not the procedure will really improve the quality of life for a person with severe mental or physical problems, we do implant more children with these issues now than in the past, allowing many of these patients to become more aware of their world and maximize their potential, whatever that may be,” she said. As implantation technology continues to maximize its own potential with smaller, shorter implants, surgeons like Meyer and Lambert are working on modified procedures to minimize insertion trauma to the cochlea during surgery and retain more residual hearing. Meyer and his colleagues are also working on projects to better understand mechanisms of speech perception to improve the knowledge about how implants affect communication and hearing.  He also works closely with John King, Ph.D., assistant professor and director of the Vestibular and Balance Program, and both are interested in improving the ability to evaluate the vestibular system for patients with hearing loss. A frustration for Meyer and Connell is that some insurance companies still consider implantation a hearing device when it's actually defined as a prosthesis. Receiving an implant typically costs about $50,000 and is usually an outpatient procedure for adults and a night or two in the hospital for pediatric patients. Bilateral implantation is not routine in the United States yet, but the program is currently considering a candidate for bilateral implantation. “It’s a personal decision for each individual, especially if an insurance company will not provide funding for the second device,” Meyer said. After a month, the patient returns to MUSC and Connell begins programming the implant to receive sound, when “the real work starts.” During the successive visits, Connell ensures that the device works, changes levels, introduces new programs, and conducts lots of testing. “The ear that has the most memory of sound will do best with the implant, and sometimes that’s difficult for patients who worry that they are willingly doing something to alter their ‘better ear,’” Meyer said. “Sometimes we see candidates drop out because they won’t take a chance on the better ear. But if you don’t use the better ear, then the patient is less likely to be a successful implant user.” A tightly knit group, the Cochlear Implant Program and its patients get together for annual events, including a recent picnic at Charlestowne Landing on April 2.  For more information on the Cochlear Implant Program, visit http://www.muschealth.com/medical_services/specialty_listing/spec_otolaryngology/cochlear.htm  or call 876-1308. Children who benefit the most from the implants: have been deaf for a short period of time have been in a good auditory-oral training program have families who are strongly committed to the training process The goal for children with a cochlear implant system is to help them to learn to listen and to speak. Adults who benefit the most from the implants:  have already developed spoken language  are motivated to listen again and receive the most benefit from the cochlear implant Post-lingually deafened adults usually understand an average of 85 percent of simple sentences without lip reading with the cochlear implant system.   The  MUSC Cochlear Implant Program: Ted Meyer, M.D., Ph.D.; Paul Lambert, M.D.; John King, Ph.D.; Abby Connell; Kimberley Snyder; Nevitte Swink; Pamela Teachey. “I received my implant two years ago and it has been the most tremendous experience. When I was 6 years old I had the measles that resulted in the loss of most of the hearing in my left ear. After I had my second child, I suffered gradually hearing loss in my right ear. I was referred to MUSC by my audiologist as a possible candidate for an implant. I can’t even tell you what a huge difference it has made … I hear more sounds and I’m more aware of what’s going on around me. I can understand speech better and don’t have to rely on speech reading like I used to. I can even hear people behind me! Everything is really normal and just tremendous. There is an element of risk to it, that it won’t work, but I’m glad I took the chance and would highly recommend it.”  —Kathy Marrah, Summerville “When Jonathan was born, he had no hearing in his right ear and when he was 6 years old, the left ear went too. While we were figuring out what to do and talking with our ear doctor about what was best for him, Jonathan attended regular school and did well. Our doctor recommended us to MUSC and we were committed to doing whatever it took to help our son. It’s been awesome and everyone at the hospital was wonderful. Now 8 years old, he still asks if he has to wear it forever and we tell him yes, but we think he’s starting to understand that wearing the implant means that he’s able to hear and that’s better than the alternative.” —Gayle and Rodney Hinson, Ridgeville       Friday, April 15, 2005 Catalyst Online is published weekly, updated as needed and improved from time to time by the MUSC Office of Public Relations for the faculty, employees and students of the Medical University of South Carolina. Catalyst Online editor, Kim Draughn, can be reached at 792-4107 or by email, catalyst@musc.edu. Editorial copy can be submitted to Catalyst Online and to The Catalyst in print by fax, 792-6723, or by email to petersnd@musc.edu or catalyst@musc.edu. To place an ad in The Catalyst hardcopy, call Community Press at 849-1778.