Hearing Loss
2/26/14
Mother’s Day is fast approaching, and for Lisa Clayton of Killen, AL, it promises to be a day of celebration with her husband, Ryan, and their three daughters – 4-year-old Ella Marie and 2-year-old identical twins Isabella and Sophia. But before the joy, there were also times of fear and anguish. “Shortly after Ella Marie was born, she was diagnosed with profound hearing loss,” explains Lisa. “I was told she was deaf – that she would not be able to hear.” Ella Marie had failed the hospital’s newborn hearing screening due to a mutation of Connexin 26, a protein found on the (GJB2) gene. This mutation is the most common cause of congenital sensorineural hearing loss – the kind of deafness that occurs as a result of damage to the cochlea. “I was on an emotional rollercoaster thinking that Ella Marie would never hear me say ‘I love you,’ wondering if she would have difficulty making friends in the future, wondering if I had done something wrong to cause this,” Lisa says. “And wondering, too, just how I could communicate with my own child if she couldn’t hear me at all.” When she was just 3 months old, Ella Marie began weekly visits to the HEAR Center at Children’s of Alabama in Birmingham, where further testing revealed she was a candidate for cochlear implantation. A cochlear implant is a complex electronic device consisting of an external component that sits behind the ear and a second that is surgically placed under the skin. Cochlear implants bypass damaged portions of the ear to directly stimulate the auditory nerve. Signals generated by the implant are sent by way of the auditory nerve to the brain, which recognizes the signals as sound. “We have the best cochlear implant surgeon in the world at Children’s – Dr. Audie Woolley,” Lisa says. “When I first met him, I remember telling him I had prayed for a miracle – that further testing would reveal Ella Marie was not deaf. He looked at me and said, ‘Lisa, this is your miracle. Ella Marie will be able to hear with cochlear implants. The sky will be the limit for her.’” Ella Marie, who is bilaterally implanted, received her cochlear implant when she was 17 months old and her second one two days after her second birthday. “Her parents came to the first office visit with us having acquired substantial knowledge about hearing loss and cochlear implants on their own,” says Dr. Woolley, medical director of the Cochlear Implant Program at Children’s. “Lisa is a teacher, and she was ready to do whatever it took to bring about hearing for her daughter and to help her achieve spoken language. The Claytons were diligent both in therapy at the HEAR Center and also with work at home with Ella Marie.” Fast forward two years to the Claytons’ decision to have a “second” child. “Because of our experience with Ella Marie and Connexin 26, we knew any future child of ours would have a 25 percent chance of hearing loss,” Lisa explains. “But after a lot of discussion, we decided ‘Yes, we want a second child.’ Then, at my first prenatal visit, we were treated to a big surprise – we were going to have twins!” Although she was excited by the news, Lisa says the fears also crept back in: What if the twins were deaf, too? How could they deal financially with three children who have a disability? Or, what if one twin was deaf and one was not – would that be hard on them? What if Ella Marie was their only deaf child? Lisa was well aware of the time and energy involved in caring for just one child with hearing loss – the doctor visits, audiology tests, surgeries and auditory verbal therapy sessions. Could she manage that for three children – along with all the everyday activities in which every mom is involved? As it turned out, the answer to that question was: “Yes, she could.” Isabella and Sophia did not pass their newborn hearing tests. They, too, were deaf. And they, too, would receive cochlear implants at Children’s of Alabama. “Isabella and Sophia were among Alabama’s youngest cochlear implant recipients,” Lisa notes. “They also are Dr. Woolley’s only set of twins to receive implants. Their surgeries were performed on the same days – the first at age 8 months and the second at age 10 months.” Lisa says she often thinks about holding Ella Marie in her arms at the hospital shortly after she was born and hearing Ryan say, “This is a special little girl who is going to accomplish special things.” Today, the Claytons say they have been blessed not only with one little girl who accomplishes great things, but with three girls who amaze them daily. “I have seen my fears morph into the smiles, the blessings and the joy our daughters bring to our lives,” Lisa says. “Their silent world has transformed into a world where there is lots of laughter, language and love. Every day we celebrate something new the girls are doing – a new word that Isabella and Sophia have added to their vocabulary or a long conversation with Ella Marie.” Today, Ella Marie is a preschooler for whom, as Dr. Woolley predicted, “the sky is the limit.” She is involved in gymnastics, dance, tee-ball and soccer – and she lives life to the fullest. “Every time I ask her if she would like to try something new, she quickly responds, ‘Yes,’” Lisa says. “She is very kind and very social and she has many friends. Both Isabella and Sophia have such spunky personalities, and they are always looking for fun. They are already starting to put sentences together in their speech. They love to sing songs, dance, listen to books being read aloud to them, dress up like princesses and play outside.” “We knew they would do well with all the family support and love they receive at home – not only from their parents, but also from their extended family and friends,” says Dr. Woolley, who keeps a photograph of the Clayton sisters in his office. “Each visit with them brings new surprises and delights – and that is what makes our job so rewarding. This family works hard and they achieve results. I love hearing updates about the girls, and I look forward to following their progress throughout their lives.” “This Mother’s Day, I know that I am ‘deafinitely’ blessed,” Lisa adds. “The fear of my children not being able to hear me say ‘I love you’ has subsided. Not only do they hear me, but they say it back!”
