Nerve Deafness: Cochlear Implant Gives Hailey New Opportunities
"Today, Hailey is unstoppable."
Hailey Ems was diagnosed with bilateral sensorineural hearing loss (nerve deafness) when she was 14 months old. At 20 months, Hailey underwent cochlear implantation surgery. Today, thanks to what her mom describes as "a miracle of technology," paired with frequent visits to Cincinnati Children's Hospital Medical Center for intensive auditory and speech and language therapy, Hailey experiences the equivalent of mild hearing loss.
Mary and Daniel Ems never planned on a home birth for their third child, Hailey. Yet on April 14, 2000, Mary's labor progressed so quickly that Hailey was born on the floor of the family's bathroom while the emergency responders discussed how to get a stretcher up their narrow staircase.
Apart from her surprise arrival, Hailey appeared to be a typical infant. In the emergency room, she was evaluated for signs of head injury, internal injuries and hypothermia. Her exam, most unfortunately, did not include a newborn hearing test. After 48 hours in the hospital, Hailey joined older siblings Daniel and Alexandria at home in Cincinnati.
First Signs
But at just 6 months old Hailey began to show signs of delayed development. "Her midsection was like a rag doll. She couldn't sit up at 6 or 9 months," says Mary. At first, medical providers suspected cerebral palsy and tests were run to assess Hailey's physical and neurological development. To address her lack of muscle tone, Hailey began occupational and physical therapy three times a week at Cincinnati Children's.
The real turning point came when Hailey was 14 months old. It was then that the family experienced a collective "ah ha" moment. They were watching television in the living room when 4-year-old Alexandria blew her dad's shrill basketball whistle and everyone but Hailey winced. Unsure of what they'd seen, the family conducted an experiment. Her 10-year-old brother, Daniel, stood behind Hailey and blew the whistle again. This time Hailey turned toward Daniel and grinned. While dad felt certain that Hailey had heard the whistle and responded, Daniel didn't think so. He suspected that she was reacting to the feel of his breath on the back of her head.
Daniel's next move was inspired. He held a piece of paper up between the whistle and Hailey's head and blew again. Nothing. That's when they knew. Hailey didn't flinch or move or react in any way to the piercing sound of the whistle. She didn't seem to hear it at all.
The True Test
After an inconclusive screening with Hailey's neurologist the following day, Mary requested a referral for a more definitive screening to confirm what the family suspected. An auditory brain stem response (ABR) was performed on Hailey at Cincinnati Children's. This time the test came back conclusive: Hailey had a bilateral moderatelysevereto-profound sensorineural hearing loss.
"When the audiologist gave me the test results I had no idea what it meant. She said, 'Hailey is profoundly deaf in both ears,' but I couldn't believe what she was saying. As we walked though the parking lot, I scooped Hailey up and carried her to the car. Then I realized, I'm already treating her differently, like a fragile china doll," recalls Mary."When we got home my husband and children greeted us at the door. We all took turns holding and hugging Hailey. Dan and I went through the packet of information and tried to make sense out of the endless pieces of paper. Each time we read a new piece of paper, the reality sunk in just a bit more.".
Additional tests confirmed that there was damage to the hair cells in both of Hailey's ears, resulting in permanent hearing loss. The hair cells are the crucial part of the inner ear that stimulate hearing nerves. Following confirmation of the hearing loss, Hailey was fitted with hearing aids and enrolled in auditory and speech-language therapies.
The Work Begins
At their first visit, Hailey's primary aural rehabilitation therapist, Amanda Fitz, MA, CCC-A, explained that there are two types of therapy for hearing impaired children: speech-language therapy and auditory therapy. Speech and language therapy helps the child learn to talk and understand language, whereas auditory therapy helps the child learn to listen and make use of the many sounds in their environment, including speech and language. "The kindness and warmth given in her explanation told me that this was the place to be and that Hailey was in good hands," says Mary.
Mary accompanied Hailey twice a week to aural rehabilitation and speech-language therapies. She resigned from her job in order to accommodate Hailey's new therapy schedule while Dan worked double shifts to make up for the new costs associated with her disability.
After three months, the Ems sat down with Amanda to talk about Hailey's progress. "She told us that Hailey's auditory skills had not improved with the hearing aids and that by now she should have shown some progress. Before I had a chance to even think about what she had told me, Amanda said that Hailey still might have the chance to hear. That's when she asked if I was familiar with cochlear implants," says Mary.
An Opportunity to Hear
Amanda explained how the cochlear implant works for children with profound hearing loss. Using a model, she demonstrated the three components of the implant: the internal device that is surgically implanted into the skull; an external microphone that attaches to the outer part of the head directly over the internal device; and a processor that is worn against the body and attached by a harness.
"Dan and I sat motionless as Amanda effortlessly explained the mechanics of the cochlear implant. After she talked about the implant itself, she went through the steps necessary for Hailey to become an implant recipient," says Mary. Because the surgery is invasive and permanent, a strict application process is required.
More Tests
Mary and Dan went home to talk over the possibilities of the cochlear implant but only after signing a mountain of paperwork to start the process. "I knew I wanted this for Hailey. We would do anything to help her learn to communicate," says Mary. With the paperwork started, the Ems initiated other steps toward approval. Hailey had an MRI to confirm that her cochlea was completely intact. Then, as her medical file was reviewed by an eight-member cochlear implant panel, Mary and Daniel met with a social worker at Cincinnati Children's.
"At first I was surprised that they wanted us to meet with a social worker. But after we talked, I was so glad we went," says Mary. "She talked to us about the choice we were making and helped us understand that while Hailey was a great candidate, not everyone in the deaf community would support our decision. We didn't realize that to some, we would be seen as rejecting deafness. In our minds, this was a chance for Hailey to hear. We weren't going to let that opportunity pass us by."
Challenging Insurance
Initially, the Ems were told by their insurance company that the cochlear implant was not "medically necessary." Unwilling to accept this limitation, Mary quickly became an advocate for her daughter and appealed the company's decision. After letters and phone calls, the insurance provider reversed its position and Hailey was approved for her surgery. The entire process took less than three months, but it was an exhausting time for the entire family.
But the battle wasn't over. Five years later, in 2006, when medical providers determined that Hailey would benefit from a second implant, the Ems were denied coverage again.
"I became an excellent advocate. I looked at the company's coverage for amputees and I challenged them," says Mary. "Surely they wouldn't say one arm is enough if you've lost both. I would have testified in front of congress if it had been necessary."
Meanwhile, the Audiology Division had become Mary's base. "They couldn't do it for me, but they knew how to connect me to the right people and organizations." A referral led the family to the Let Them Hear Foundation who provided Mary and Daniel with the tools and support they needed to make Hailey's case. "Their help was invaluable," says Mary.
Once the initial insurance hurdle was cleared, Mary and Daniel needed to choose which model of cochlear implant would work best for Hailey. Again, the audiologists helped to guide their decision. "They asked us to think about what features were most important to us. We chose a model that gave an alert signal when it comes off," says Mary. Since initially Hailey wouldn't be able indicate when her implant wasn't transmitting, having an alarm was a top priority."Just as we were getting used to Hailey being deaf, we were now trying to get used to the fact that Hailey might soon be able to hear."
More Surgery for Hailey
On December 3, 2001, Hailey received her first cochlear implant. The procedure took approximately seven hours. "Dan and I both took books, crossword puzzles and magazines to the hospital so we would not have to leave the waiting room. But we didn't look at one single thing," says Mary. "Shortly after Hailey was taken in for surgery, Dan fell asleep from exhaustion and worry and I talked to my mother-in-law about anything and everything in order to keep my mind off of Hailey."
Hailey sailed through surgery. Although it is an invasive procedure, Hailey and her family were able to leave the hospital 24 hours after surgery. "I could not believe how alert Hailey was by the next day; it was almost as if she did not have surgery at all. It was truly amazing," says Mary.
In Her Own Words
Hailey had to wait four weeks for her incision to heal before the external component of her device could be activated.
"On January 10, Hailey was scheduled to be 'turned on.' We were told beforehand that Hailey may or may not respond to sound at the first visit and not to be upset if nothing happens. It does not mean the implant is not working," says Mary. "By that time, Dan and I were used to the debriefing process and decided we would go to this appointment with hope but not be disappointed if things did not work out," recalls Mary.
"The first visit was a lengthy one. The mapping audiologist had to use software from her computer to program Hailey's new processor. This was a process of trial and error since the only way to tell what levels to program the device was to watch Hailey's reactions.
"Unfortunately Hailey did not respond when she was turned on, as we had hoped. All we could do was continue with auditory and speech-language therapy and wait for a sign from Hailey that the implant was working. Everyone involved in Hailey's well-being was confident the implant was working and that it was only a matter of time before Hailey would respond to sound. Dan and I kept telling ourselves the same thing but we were not seeing the results we had hoped for and we wondered if the device was working at all," says Mary.
"Each week I took Hailey to therapy, hoping for a reaction to sound but each week the result was the same. By March, Hailey was 12 months old and still not walking or responding to sound. I was becoming discouraged.
"Then, on March 25, Hailey woke up and did two miraculous things: she took five steps and made three sounds. I could not believe what I was seeing or hearing. At first I thought it was a fluke until she did it throughout the day and again the next day and the next. Her next therapy appointment could not come fast enough for me to share the news. The audiologist told me Hailey was starting to realize that she can make the same sounds with her voice that she hears, which is the hard part, and that she will now begin to make more sounds and eventually speak. We were so excited and happy for Hailey because everything was finally coming together and all of her hard work was starting to pay off," says Mary.
Moving Forward
Since making the decision to go forward with the cochlear implant, the Ems haven't looked back. At age 7 Hailey loves sports, particularly t-ball, swimming and soccer, and while mom worries about Hailey wearing her processor on the sports field, Hailey is unstoppable. Mary describes Hailey as a confident and independent little girl.
This year Hailey will enter second grade, mainstreamed into a public school classroom. Several years ago Mary returned to school to become a special education teacher and now she uses what she's learned as the mother of a deaf child to help others.
