Hear! Hear! to productive life

Cochlear implants have revolutionized the world of the profoundly deaf, allowing for normal, productive lives.

By
February 4, 2006 21:50
Hear! Hear! to productive life

hearing aids 88. (photo credit: )

 
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If one is fated to be born with a serious handicap, the "best" disability is deafness. So says Rochele, a young haredi Jerusalemite mother of nine who has a lot of experience. Four of her children - aged two to 15 - inherited a pair of mutant recessive genes that caused their handicap. Although she and her husband underwent genetic tests before they married, deafness genes - such as Connexin 26, which is responsible for a third of all congenital deafness and 90% of all deafness in hearing parents - were then not testable. But her children have normal lives and study in regular schools, thanks to their cochlear implants. This is an electronic device - essentially a bionic ear - with an external segment that attaches by magnet to an internal segment surgically implanted in the inner ear. The external section contains a microphone and sound processor that collects important sound data, codes it and turns it into electrical pulses, sending them via a wire to an external transmitter. This in turn transmits them through the skin to the internal receiver, which transmits them to 24-channel electrodes inside the cochlea (the snail-shaped structure in the inner ear) to stimulate the auditory nerve along a whole range of frequencies. Candidates for implants have a severe-to-profound hearing loss (cannot discriminate words) and don't benefit from high-powered hearing aids. Studies have shown that implanting these devices, which costs about $20,000 apiece, is among the most cost-effective medical procedures, ranking above interventions such as coronary artery bypass. Fortunately, a few years ago the Health Ministry recognized the cost effectiveness of cochlear implants in young people, so health fund covers the cost of the device, surgery and hospitalization up to the age of 18. But there is little or no financial assistance for adults who need an implant. THE EAR normally translates sound waves into electrical impulses, which the brain perceives as sound. Implants bypass the dysfunctional inner ear and mirror the natural mechanical-to-electrical translation - a different process than hearing aids, which simply amplify sounds. Cochlear implantation is aimed at people of any age - from one-year-olds to octogenarians, those born deaf and those who became deaf with age or from injury, toxic reactions or noise. The implants work best for people who lost their hearing after they acquired speech, and are even more effective in those with recent hearing loss, but also work wonders for those born deaf, especially before the age of seven. In many cases, two cochlear implants are better than one. Human bodies were created with two ears because only stereophonic hearing allows us to localize sources of sound, hear and understand in noisy situations and feel complete in the world, says Elaine Matlow Tal-El, chairman of a voluntary self-help parents' organization called AV-Israel that fights for cochlear implants, advocates integration of hearing-disabled children into regular schools, and offers professional rehabilitation. Founded 10 years ago, AV-Israel (www.avisrael.org.il) has facilities in Jerusalem (Rehov Pierre Koenig 33 in Talpiot) and Ra'anana (POB 391). "We use the auditory-verbal approach that has helped thousands of children around the world and many in Israel to become fully integrated in the regular education system," says Tal-El, mother of four daughters, two of whom (twins) were diagnosed as profoundly deaf at the age of two. "The problem wasn't diagnosed immediately," she recalls. "We were told they might be having problems speaking because we are bilingual at home. They were born full term, and we have no family history of deafness. We still don't know what caused it." Her daughters Dana and Tamar began with hearing aids, but these were inadequate. Today they are 16, have cochlear implants and are doing very well in the 10th grade of Jerusalem's elite High School for the Arts and Sciences. "They blab on cellphones all day and are involved in youth movement activities," says their proud mom. The first implant was performed in New York 13 years ago at the expense of the family, which has spent a total of $100,000 on the devices and the concomitant rehabilitation. Her deputy chairman, Josie Eizner, is the mother of two children, now 29 and 26, both of whom were born deaf and who used hearing aids which were replaced by implants. The elder is married and the mother of four healthy children, while her son is married and studying architecture. "For deaf children, learning language and attending schools - where they must hear and comprehend in noisy surroundings, especially in Israel - are critical to success," continues Tal-El, a former immigrant from the US who married an Israeli. "The latest trends in rehabilitation of the deaf show that bilateral implantation brings deaf children to previously unattainable levels of functioning." BUT DESPITE this, the basket of health services doesn't recognize this possibility. "Families who have provided their children with a second implant have had to fight the system to do so. Israeli professionals have yet to reach a consensus regarding the real value of bilateral implementation, and the issue is still hotly debated," says Tal-El. Because of this, she and her colleagues organized a recent conference at the capital's Variety Center where noted cochlear implant rehabilitation experts Drs. Diane Brackett and Antonia Brancia Maxon of the New England Center of Hearing and Rehabilitation addressed policymakers, practitioners and parents. Getting just one of the expensive implants was considered a "big deal" only a few years ago, but having two, especially in young people, is now considered an even greater blessing. In 2001, New York University Medical Center was one of the pioneers in bilateral implantation when its surgeons inserted a multi-channel cochlear implant in each ear of a 52-year-old secretary. Five years ago, only 13 patients in the US had bilateral implants. NYU otolaryngology Prof. Susan Waltzman and co-director of its cochlear implant center said then that it wasn't clear how much better patients hear with two. Today, the answer is clearly "Much!" The potential demand for cochlear implants is much greater than the actual number used in Israel today, says former French immigrant Haya Levi, veteran head of Hadassah University Medical Center's speech and hearing center in Jerusalem's Ein Kerem. One in 1,000 children born today has serious hearing difficulties or complete deafness," she explains. "Among Arabs, because of consanguinity [marriage between first cousins], the rate is even higher. There are another two to four individuals per 1,000 - of all ages - who become deaf and do not function well with hearing aids. Cochlear implants can help them, even if they are 80. Even patients who don't have a functioning auditory nerve can be helped with a cochlear implant attached to the brain stem, although functioning is usually not as good as when connected to the auditory nerve." Last week, Prof. Yosef Elidan - head of Hadassah's otolaryngology department - and his staff performed their 226th cochlear implant since they began 11 years ago. "In 2005," says Miriam Adler, a New York-born communications specialist who coordinates Hadassah's implant program, "we have done more than 50 - most of them children." Not every ear-nose-and-throat specialist is qualified to perform the delicate surgery, as it requires long training and much experience. In addition to Elidan, there are a handful of other surgeons who do it, in Sheba Medical Center at Tel Hashomer, Schneider Children's Medical Center of Israel in Petah Tikva, Bnai Zion Medical Center in Haifa and Soroka University Medical Center in Beersheba. Although there is a small risk because the two-to-four-hour operation is carried out under general anesthesia, the procedure is regarded as safe, with rare complications, mostly infections of the skin flap. The Hadassah center doesn't recommend implants for all hearing-disabled children. "Our experience shows that implants offer better functioning in difficult listening situations, such as noisy environments" says Adler, "but even with them, patients do not have normal hearing." But getting the device and undergoing surgery is not the end of the story. Batteries have to be replaced every day or two, and since the health funds pay little or nothing of this cost, it can reach hundreds of shekels a month, especially if a patient has two implants. Some batteries - which constantly release current - are rechargable, but even these have a limited life. In addition, patients must go for rehabilitation after surgery, as just having an implant is not enough to hear. "During the first year, the patient needs mapping of impulses eight times, and in subsequent years two to five times," says Adler."We don't know why, but the nerves' reactions change over time. It's not like a static hearing aid. You have to program the implant. Programming of a second implant is even more complicated, as you have to suit it to the first." Hadassah has begun offering a cheaper, but effective, French model to families not eligible for health fund subsidies because they want a second one, or if the patient is over 18. With existing models, listening to music is difficult because the signal has to be coded by the processor, which operates best in the range of human speech. Users can perceive rhythm and lyrics, depending on the tempo, but have trouble perceiving melody. But the small number of companies that design and manufacture implants are working furiously to improve their performance; there may eventually be an implant located solely inside the head, as well as batteries that last as long as those in a pacemaker. "We really hope the Health Ministry will add second implants to the basket of services, especially for children," Levi and Adler agree. "The cost is high, but we would love to see implants provided to suitable hearing-disabled adults as well." At that point, the number of clinical communications specialists with expertise in implants will be inadequate and more will need to be specially trained. The government, they conclude, should take the long view. Deaf adults who become hearing enabled can contribute more to society. Deaf children who are mainstreamed in regular schools save money, because fewer places will be needed in special education schools. "Deaf kids," adds Adler, "are usually normal kids with normal intelligence. Their potential, once you tap it, is unbelievable. We in this field have a great deal of satisfaction, because we know many of the kids from when they were babies. We see the kids developing with their implants and integrating themselves into society."

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