Hearing Tests for NewbornsEarly detection offers hope

From the earliest days, babies begin to develop their speech and language skills. They learn by listening to and interacting with voices and the variety of sounds in their environment. Hearing loss, however, is easily missed during these first stages of life — a critical phase of language development. Newborn screening tests to detect hearing loss and follow-up preventive intervention programs are now available to help to prevent speech development delays that could affect a newborn’s future social, emotional and educational development. According to recent information published by the Centers for Disease Control and Prevention in Atlanta, approximately one or two out of every 1,000 infants born in the United States suffers from some degree of hearing loss. Dr. Lynn Spivak, director of the Hearing and Speech Center at Long Island Jewish/Schneider Children’s Hospital in New Hyde Park and assistant professor of otolaryngology at Albert Einstein College of Medicine in the Bronx, states that hearing loss can result from a number of medical conditions. “About 50 percent of congenital hearing loss is genetic and 50 percent is acquired,” she says. “The most common causes of acquired hearing loss come from in-utero or postnatal infection, premature birth, or diseases such as rubella and meningitis.” Other babies who may be born with or develop hearing loss include those with birth defects or exposed to drugs in the womb. But recent research indicates that infants diagnosed before the age of six months and treated through intervention programs can develop language skills (spoken and/or signed) on a level with their hearing peers. In 1999, the American Academy of Pediatrics issued a statement recommending that hearing screening tests be implemented nationwide. Approximately 35 states currently mandate newborn hearing screening tests be performed by hospital technicians prior to discharge. New York State’s regulations became effective October 20, 2001. According to a statement issued by the New York State Department of Health, tests should be conducted prior to discharge, and results made available to parents and documented in the infant’s medical record by the specialist who performed the exam. If an infant should fail, a repeat test must be performed prior to discharge. Two types of hearing screening tests are performed on infants: • Otoacoustic Emissions (OAE). Otoacoustic emissions are sounds made by the cochlea, the fluid-filled part of the inner ear that converts sounds/vibrations into electrical signals that are then sent to the brain. The OAE requires the placement of a tiny earphone/probe into a newborn’s ear canal. Sounds are played and the baby’s responses are measured. If an infant suffers from hearing loss, no echo will be detected. • Auditory Brainstem Response (ABR). The ABR test consists of small electrodes attached to the newborn’s head. Soft clicking sounds are presented into the ear through small earphones. These sounds cause brainwaves, which are picked up through the electrodes and recorded by a computer. Both OAE and ABR can be used separately or in combination. Each is performed while the newborn is asleep, and there are no risks involved. Some hospitals prefer to perform the OAE test first, and if an infant fails, the ABR is used to follow up. If a newborn fails the first test, this does not automatically mean that he/she has a hearing loss. “There are several reasons why your baby may have failed: vernix, or fluid, may be present in the ear canal, which can interfere with the test,” explains Dr. Jay Dolitsky, director of pediatric otolaryngology at the New York Eye and Ear Infirmary in Manhattan. Other factors for failure include movement, fussiness and/or crying during the screening. “Babies who fail in the hospital are screened against about four weeks. If they fail during this prescreening, they should be scheduled for full diagnostic evaluation,” states Dr. Spivak. Both audiologists and otolaryngologists agree that infants whose hearing loss is diagnosed before six months demonstrate better language skills than those whose hearing loss is diagnosed later. “The goal of early detection intervention is to avoid speech and language delays and possibly the need for special education and services,” Dr. Spivak says, but notes that each child’s situation is different. “The more severe the hearing loss, and the later it is detected, the more likely it is that the child will need special services in school.” Babies can be fitted with a hearing aid as early as four weeks of age. If hearing loss is so severe that surgery must be performed, Drs. Spivak and Dolitsky both agree that a child can undergo a cochlear implant after one year of age. “The child must go through a hearing aid trial first,” says Dr. Dolitsky. Dr. Spivak adds, “The child must be 12 months of age before being implanted with a cochlear implant except in some special cases.” The Individuals with Disabilities Education Act (IDEA) ensures that children who suffer from permanent hearing loss receive free early intervention programs from birth to 3 years old, and throughout their school years until they reach 21.

Resources For further information about hearing loss, screening tests and intervention programs, the following may provide useful resources: • National Association of the Deaf (NAD): www.nad.org • National Campaign for Hearing Health: www.hearinghealth.net • American Speech-Language-Hearing Association (ASHA): www.asha.org • American Society for Deaf Children (ASDC): 1-800-942-ASDC or www.deafchildren.org • American Academy of Audiology: 1-800-AAA-2336 or www.audiology.org • Centers for Disease Control and Prevention’s Healthline: 1-888-232-5929 or http://www.cdc.gov/ncbddd/ehdi • March of Dimes: www.modimes.org

SIDEBAR

Signs of possible hearing loss in infants and young children 1. Failure to startle or jump at loud sounds. 2. Failure to turn toward the sound of a voice or to imitate sounds after six months of age. 3. Lack of babbling at nine months of age. 4. Not using single words by 18 months of age 5. Several ear infections in infants/toddlers. Every child develops at his/her own pace. However, if anything concerns you and/or your partner, consult your pediatrician/caregiver for further information)

Photo Caption for “Newborn/hearing” photo: Newborn screening tests to detect hearing loss and follow-up preventive intervention programs are now available to help to prevent speech development delays that could affect a newborn’s future social, emotional and educational development.

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The Silent Ear Disease By Terri Pilcher

Teddy wears hearing aids. He was born with permanent hearing problems, so when his hearing worsened, his mother, Michelle Webster, wasn’t worried. “We already knew he had hearing loss, so we didn’t seek immediate attention,” she recalls. He stopped responding to sounds, his speech worsened, and he began growling the way he had prior to receiving hearing aids. After four months, he saw an audiologist for a routine ear and hearing aid check. The test showed that his eardrum wasn’t vibrating properly, a cause of hearing loss not related to his birth defect. Michelle took Teddy to his pediatrician who diagnosed a bulging eardrum caused by an effusion. An effusion is a large amount of uninfected fluid behind an eardrum. Effusions last for days or weeks after acute ear infections. The physician referred them to an ENT specialist. When decongestants failed to loosen the fluid behind Teddy’s eardrum, the specialist inserted tubes to allow the fluid to drain. After months of fighting ear infections, adjusting the hearing aid, and inserting tubes, Teddy (when wearing his hearing aids) now hears the same sounds as a normal child.

Ear infections are the number one reason for visits to the emergency room and the number two reason for visits to a physician’s office. Deborah Allen, an audiologist, says that half of their patients are children. Of those children, more than 90 percent of them have hearing loss because of an ear infection. These problems are preventable when the infections are caught early, but this responsibility is left to the parent. Unless a parent thinks their child has a hearing problem or an ear infection, most physicians won’t perform hearing tests or check for an infection. The single exception is the required audiometry performed before a child enters kindergarten. According to pediatrician Dr. Beverly Tyler, parents often don’t realize that their children have ear infections, because they don’t have fever or pain. Says Dr. Tyler, “There are no hard and fast rules as to what symptoms must accompany an ear infection.” To find effusions and painless ear infections, Dr. Tyler suggests that parents look for changes in their child’s hearing. Children who start saying, “What? What? What?” or “Huh? Huh? Huh?” may have hearing problems. Other warning signs Dr. Tyler recommends looking for include sitting close to the TV, turning up the TV, and only responding when face-to-face. Smaller children may develop irritability, crankiness or a lack of attention. Deborah Allen recommends a simple test. “Parents can try to whisper behind the child’s back. Something that’s very common and they know. Something like, ‘Would you like some ice cream?’‚ or ‘Would you like to go out to play?’ For an accurate test, the child must not be able to see that the parent is speaking.” “Be aggressive if you think there’s a problem,” advises Michelle Webster. “I know a mother who was poo-pooed by her doctor for a long time because she thought her child had a hearing problem. It took a lot of visits before someone listened to her and did the tests that showed he had a hearing impairment.” When parents suspect a hearing problem in their children, they should go to the pediatrician. “If a pediatric patient complains of earache and the eardrum is inflamed, with or without drainage from the canal, it is treated with antibiotics,” says Dr. Tyler. “Ear infections don’t have to be very bad before therapy is started. Ear infections can be very painful, and if not appropriately treated can have serious consequences.” Don’t be surprised if your physician doesn’t follow these guidelines, however. According to Dr. Tyler, “There is a lot of controversy about when to treat, but most experts will recommend the physician use their best clinical judgment.” Any time a doctor’s treatments don’t result in improvement within several days, “it is advisable that the patient return to the office for reevaluation,” recommends Dr. Tyler. If an ear infection is not visible, don’t give up. Dr. Tyler tells parents, “Don’t second guess your feelings.” If you think your child has a hearing problem, insist on testing. Ask for a tympanogram, which determines whether fluid is behind the ear. Most pediatricians and all ENTs keep the equipment in their offices. Other tests are also available to check for more serious causes of hearing loss. Hearing losses due to fluid in the inner ear are always temporary. Once the fluid drains, the hearing returns, but by then damage to speech development may have occurred. The first two years of life are the critical time for language development as well as the time when most ear infections occur. “If the infection is undetected and it’s through those critical language learning periods, it really can delay children’s development,” says Deborah Allen. Marie Noplok, a speech therapist, explains, “Although only four or five speech sounds are mastered by age 4, toddlers need exposure to all of the sounds.” The most noticeable differences in young children with speech delays due to hearing infections are “the lack of use of plurals, articles, and possessives.” Noplok and her therapists provide a language and speech evaluation and, depending on the severity of the delay, provide treatment. Parents are then given homework to help their children catch up with peers. Even without speech therapy, children’s speech and language will improve once the fluid in their ears clears. Says Noplok, “They’ll catch up with their peers eventually, although they’ll be delayed.” How many ear infections harm hearing? Half of all ear infections with effusion. Parents must remain vigilant to prevent children, especially those under the age of 2, from developing hearing loss due to ear infections and effusions.