Peggy Guillou enjoys watching her grandchildren put on “shows” in the family room. They sing and dance for their captive audience.
This simple pleasure of family life is particularly special because siblings Matthew, 16, Marcus, 15, Michelle, 13, Maria, 10, Miley, 8, and Marcia, 6, were born deaf. Over the past eight years, all the children have been fitted with bilateral cochlear implants, allowing them to hear for the first time and develop communication skills.
“They are doing wonderful; it is absolutely a miracle what these implants have done for these children,” said Guillou of Cutler Bay, who raises the six children who call her mama.
Cochlear implants are used in patients who suffer from severe hearing loss due to a genetic defect, as the Guillou children do, or whose inner ear has been damaged and hearing aids are ineffective. The implants come in two parts, a surgically implanted internal device and an external speech processor worn near the ear.
Dr. Fred Telischi, chairman of otolaryngology at the University of Miami Health System, performed all 12 surgeries, one for each ear of the six Guillou children. Since its founding in 1990, the UM Ear Institute’s cochlear implant program has performed about 2,000 of these surgeries, about half of them on children, the youngest being 7 months, Telischi said.
When properly evaluated, more than 90 percent of child patients should have successful outcomes with the implants, he said.
“Most severe hearing loss is related to the inner ear hearing organ, called the cochlea,” Telischi explained. “The cochlear implant is designed to bypass the cochlea by putting electrodes in this organ, and the electrical currents stimulate the hearing nerve directly. That’s how you bypass the hearing organ that is malfunctioning.”
The surgery is only one small part of the treatment plan, and it’s a team effort: UM specialists in otology, audiology, speech pathology, psychology and educational services work together to address the patient’s communication and educational needs, as well as psychological adjustment issues.
While the Guillou family is an extreme case — only about 1 to 3 in 1,000 are born with severe hearing loss, said Telischi — undiagnosed hearing loss or disorders of any magnitude can result in learning and communication issues. Thus, it’s important that parents know when to see an audiologist.
“Even mild hearing loss or impaired hearing in one ear can cause a child to miss as much as half of a classroom discussion,” Domitille Lochett, an auditory-verbal therapist with UHealth, said in a UM report.
Cochlear implant surgery, also available at Nicklaus Children’s Hospital and other health systems across South Florida and the country, will likely be recommended for the most severe cases of hearing loss, but other therapies and technology, including hearing aids, can help.
“The important part of having hearing over the first several years of life, 0 to 3, is to develop speech and language … so that’s why it is important to identify these kids early,” Telischi said.
Among the Guillou children, Maria was the youngest to begin receiving therapy. She was under 2 when she received her implants and started at the Debbie School, an institute for children with disabilities that’s part of UM’s Mailman Center for Child Development; she moved into a mainstream school at 5.
She’s making A’s and B’s and has been on the honor roll. Now 10, Maria also participated in a summer university program for reading.
“She’s into it; she’s doing very well,” her grandmother said.
It’s never too late to get help.
Eight-year-old Miley received her implants at age 4, and unlike the others, she had not had any communication therapy or treatments because she didn’t get the help she needed in her former home. The others had at least received sign language instruction and hearing aids, Guillou said.
“She cried in silence, and she did not make any sound at all, nothing. It was very frustrating for her.”
But as soon as Peggy Guillou received custody of Miley, Guillou wasted no time introducing her to the wonderful world of sound. Guillou was awarded custody of the children because the parents were deemed unfit to care for them, she said.
“She is doing fantastic for a little girl who had nothing for four years. She is able to talk, she’s able to sing, she’s able to enjoy music,” Guillou said. “Sometimes they call us over; they are doing a show in the family room, some ballet, some musicals. She’s able to read and write and do what the other kids can do.”
Now, Miley and three of her siblings attend auditory schools; Maria and Michelle attend regular classes at Our Lady of the Holy Rosary — St. Richard Catholic School in Cutler Bay. Once a child gets implants, there’s much work to be done to help the child develop language and communication skills, Telischi said.
Speech therapy is critical, and families also typically need an education liaison to ensure the appropriate school environment, a social worker and deaf-specific psychological counseling for children and families, as well as equipment programming and maintenance, Telischi said. UM maintains hearing aid and cochlear implant equipment loaner banks for families in need.
In the Guillou household, in addition to schooling, all the children receive speech therapy at home three times a week and tutoring and deaf-specific counseling as needed. Then there’s the daily reading.
“I push a lot of reading,” Guillou said. “They must all read every day one or two hours and write a report. Then we read it over and go over it with them. It helps them with comprehension and on their vocabulary and their writing.”
Guillou remembers fondly when the children heard sounds for the first time.
“After Matthew got the implants, he said, ‘Mama, what is that noise?’ and I said, ‘Matthew, that’s the birds.’ To me that’s one of the things that made me feel so great, that he could finally hear the birds chirping.”
To be sure, cochlear implant technology has improved greatly since 1984, when the implants were first approved by the FDA for adults. Over the years, scientists have shown that profoundly deaf children who receive a cochlear implant at a young age develop language skills at a rate comparable to children with normal hearing, according to the National Institutes of Health, which has supported research on the implant technology dating back to the 1970s.
Critical research is continuing at UM and institutions around the country. Today, kids are typically screened at birth and in school for hearing loss, and more pediatricians are screening, too.
But it is important for parents to know the signs.
“One of the easiest ways to identify kids who have hearing loss is their speech doesn’t develop. By 6 months, they should be babbling, and by 1 or 1 1/2 [years], they should be forming words and word combinations. If they aren’t, that’s really one of the very sensitive signs of hearing loss,” Telischi said. “We want to make sure that all kids who don’t develop their speech properly have their hearing tested.”
Guillou also advises parents to seek help as early as possible.
“I know what you are facing. But don’t give up hope — continue fighting and get whatever the child needs. It’s a lot of work and a lot of money we spend, but it is necessary to help them.
“I want to see them be successful in life. I want them to be happy. I want them to hear the birds and hear the rain — and sing.”
Signs of hearing loss
The following could be signs of hearing loss, based on the child’s age:
▪ Shows a lack of attention to sounds (birth-1 year)
▪ Does not respond when you call their name or does so inconsistently (7 months-1 year)
▪ Does not follow simple directions (1-2 years)
▪ Shows delays in speech and language development (birth-3 years)
▪ Pulls or scratches at their ears
▪ Has difficulty achieving academically, especially in reading and math
▪ Is socially isolated and unhappy at school
▪ Has persistent ear discomfort after exposure to loud noises
If your child is displaying any of these behaviors, visit a certified audiologist, who should perform a hearing screening.
SOURCE: University of Miami Health System