Devastating Diagnosis 

Dr. Gelvez knew something more was going on due to the cloudiness of the spinal tap, so he prescribed multiple antibiotics for Adalyn. An MRI revealed she had experienced multiple seizures and a series of mini strokes. She was diagnosed with a rare strain of bacterial meningitis and transferred to the PICU. Most antibiotics were resistant to the strain Adalyn had so doctors put her on a wide variety. 

“She went down very fast,” Alexa said. “We are so thankful Dr. Gelvez ordered the spinal tap so we could get a quick diagnosis. Initially we were in shock and trying to comprehend it all. It felt like whammy after whammy with no good news. I was begging for her to live hour by hour and that turned into day by day.” 

Alexa said the doctors knew just how to talk with parents of a sick baby. Blake Nichols, M.D., pediatric specialist in the PICU, took the time to explain every detail of Adalyn’s treatment plan. 

“I remember they really wanted us to understand exactly what was going on with her and it felt like she was in the very best hands,” Alexa said. 

Audiology Emergency 

After a couple of days, they noticed that Adalyn was not responding to voices anymore. 

Bacterial meningitis comes with a high risk of varying degrees of hearing loss. Twelve days after being admitted, Adalyn underwent hearing tests. 

“Anytime we have a child with bacterial meningitis, we consider it an Audiology emergency and the child’s hearing is tested quickly,” said Lisa Vaughan, Audiology program manager at Cook Children’s. 

Patricia Purcell, M.D., MPH, pediatric otolaryngologist at Cook Children’s, said catching it in time is vital – a month after the diagnosis the cochlea hardens, and implants are not possible. 

Adalyn underwent her first hearing test and results were consistent with severe to profound hearing loss – the infant’s only option to be able to hear would be cochlear implants. 

Finding Funding for Cochlear Implants
Pediatric Otolaryngologists Dr. Purcell and Allison Chisholm, M.D., specialize in cochlear implants at Cook Children’s. In 2023, they performed 27 implants and since the program started 17 years ago, more than 600 implants have been inserted. 

The FDA has not approved cochlear implants for infants under 9 months old, so private insurance does not cover the procedure or devices. Vaughan quickly sprang into action, and within 72 hours, funding was secured. The cochlear implants, procedures and all related costs would be covered by generous donors through the Cook Children’s Health Foundation and the Cook Children’s Health Plan. Alexa’s three children have always used the Cook Children’s Health Plan, and Alexa said it has streamlined everything in the Cook Children’s network. They have also learned about resources to help Adalyn such as a deaf and hard of hearing teacher who now comes to their house. 

“Having it all under one umbrella has made a night and day difference,” Alexa said. “It has not only taken away the financial stress but has helped us with booking appointments through our healthcare manager. We are so thankful to have that stress taken off us.”
 
Dr. Purcell came up with a plan before Adalyn was discharged. She had another MRI to be sure she was a candidate for implants, and after a month in the hospital, Adalyn was on the way home.

“We really appreciated that they were on the ball and proactive in giving us information so we could prepare,” Alexa said. “We had come so far from where we started not knowing if she was going to make it. We were ready for the next challenge, and I was already looking up how to learn sign language.” 

Adalyn’s Chance to Hear Again 

A couple of weeks later, at 6 months old, Adalyn was wheeled to the operating room for her first implant. She would need two procedures – one for each ear – due to inflammation from an ear infection in her left ear. 

During the procedure, Dr. Purcell had to be careful not to damage adjacent structures to Adalyn’s brain, facial nerve and carotid artery. Dr. Purcell said technology like CT scans allowed for preoperative planning and a safe procedure. After three hours, the implant was complete. 

“Adalyn was a trooper the whole time,” Alexa said. “She wasn’t sick at all and healed quickly.” 

“We absolutely love Dr. Purcell. She went above and beyond and always returned our calls herself. She took time to clearly explain everything.” 

Home for Christmas

One week later, Dr. Purcell performed the procedure on Adalyn’s left ear, and she made it home just in time for her first Christmas. Alexa said she will never forget the holidays that year.
 
“We didn’t have much time to think about Christmas with Adalyn being in the hospital,” Alexa said. “Cook Children’s had gifts waiting for all of us, including our boys Brandon and Levi, and we were so grateful.” 

Alexa said that when they took Adalyn home, it was like having a newborn all over again. 

“Before Adalyn got sick, she could fully roll over and hold her head up,” Alexa said. “We were starting at ground zero again.” 

‘We had our Smiling Baby Back’ 

On January 12, less than a month after Adalyn’s procedures, Kristi Reed, Audiology program clinical coordinator at Cook Children’s, turned on the processor for the first time and initially activated the implants. She spent time with Alexa going over all the equipment.

Within a few hours of adjusting to hearing again, they had their babbling and smiling baby back. 
“It was like a switch went on, and we saw her personality again,” Alexa said. “We felt relieved and joyful to have overcome that first big hurdle.”
 
Adalyn was back to giggling and playing peek-a-boo – her favorite. 

During the first six months after surgery, Adalyn visited Audiology frequently as they increased the range of the implants. Adalyn mapped soft and loud noises and quickly made progress. She also saw an Audiology verbal therapist who specializes in working with children who have experienced hearing loss. Alexa learned how to facilitate language and listening at home.
 
“I’m amazed at how far she has come,” Alexa said. “Before the implants, a jet plane could take off and she wouldn’t hear it. Now, she even hears the shaking of a bell and can tell where it’s coming from.” 

A Thriving Toddler

Seven months ago, Adalyn graduated from feeding therapy and started baby food. Now 17 months old, Adalyn continues weekly auditory and verbal therapy and physical and occupational therapy as well as in-home physical therapy and sign language lessons.
 
“From the beginning I said she was our little warrior, and she is showing us every day how strong she is and continues to work toward and achieve her milestones,” said John, Adalyn’s dad. 

Alexa said they look forward to their visits with Physical Therapy at Cook Children’s in Mansfield where they come up with new ideas to try at home. Adalyn has made significant progress the last couple of months and now rolls all over the place and is working toward crawling. Her joyful personality continues to inspire those around her. Alexa said she babbles all the time and as soon as they put on her “ears” in the morning she lights up and smiles.
 
“It’s a miracle to sit back and see it all unfold,” Vaughan said. “This changes the trajectory of these kids’ lives forever.” 
 

It is a heartbreaking position for any parent to be in — knowing that a potentially life-saving drug treatment may also cost your child the ability to hear. This was the difficult reality for Brant and Ashley James after their son, Pierce, now 3, was diagnosed with a rare cancer. 

In his nearly 4 years of life, Pierce James, who his family calls Fierce Pierce, has faced more challenges than many adults experience in an entire lifetime. He was born at the edge of viability at 25 weeks gestation and spent 83 days in a neonatal intensive care unit. Even so, Pierce thrived as an infant and toddler despite some delays due to his prematurity.

But four days before his 2nd birthday, the James family received devastating news.

Just as Brant and Ashley were on the mend from a bout with COVID, Pierce seemed to be coming down with the virus as well. So Brant took his son to an urgent care close to their home in Rockwall to get checked out. He was soon told it wasn’t COVID. Instead, physicians suspected something much more serious and arranged for Pierce to be transferred to a nearby hospital. 

“About four hours later, I received a phone call from the doctor and my husband, and the words out of the doctor’s mouth was, ‘We have some bad news to tell you,’” Ashley said. “At that moment, they told me that they were pretty confident that Pierce had cancer.”

In fact, Pierce had a rare liver cancer called hepatoblastoma. It was classified as stage 4, metastasized to his lungs and required aggressive chemotherapy to treat. 

Risk and Reward

Throughout the course of his treatment, Pierce experienced several setbacks, some of them life-threatening. But one complication was particularly difficult for Ashley to reconcile. 

Pierce’s chemotherapy protocol included a drug called cisplatin. While it is effective at killing cancer, it is also an ototoxic medication, meaning it can damage the inner ear and cause hearing loss. Thanks to the chemotherapy protocol, Pierce is now cancer-free, but he does have permanent and profound hearing loss. 

“The cancer is gone and we're believing it's never ever coming back,” Ashley said. “But the hearing loss, without a miracle from God, is irreversible. It's not going to change and it’s just very hard. I've come to terms with it, but it's still very hard for me that my baby has lost his hearing because, I'm like, hasn't he suffered enough?”

Once the cancer was under control, Brant and Ashley turned their attention to dealing with Pierce’s hearing loss. As a then 2-year-old, Pierce was at a critical point for speech and language development. His parents wanted to be as persistent with audiology interventions as they were with chemotherapy. The family turned to Cook Children’s Pediatric Audiology services for help.

“There are certain chemotherapies that cause hearing loss in children and adults. So we're very involved with Hematology and Oncology,” said Lisa Vaughan, AuD, manager of Cook Children’s Audiology services. “A lot of times we do testing prior to the kids starting chemo. We get a baseline hearing test for every kid before they start, and then we monitor them throughout all of their treatment.”

Sometimes, if hearing loss is suspected, treatment can be adjusted. Other times, as in Pierce's case, hearing loss is the lesser of two evils. 

“It's really hard because it's just one more thing for these families,” Dr. Vaughan said. “Your child has cancer and we now have to watch their hearing. You're going to go through some really horrible treatments, and then when we're done with this and things are better, we may be saying your child’s hearing is permanently damaged. It’s a hard conversation because these families have been through so much.”

Cook Children’s Audiology services moved quickly to help Pierce. He completed his final round of chemotherapy in September 2022 and, by the end of that month, also had his first set of hearing aides.  

The New Age of Sound Waves

Each year, one to three out of every 1,000 babies are born with hearing loss. Others experience hearing loss from trauma or as a result of medical treatment. 

 Because of developments in audiology technology there is hope for hearing-impaired children.  Dr. Vaughan says with early intervention, children with hearing loss perform as well as their hearing peers academically. 

“We can do the surgical cochlear implant as early as nine months to get a baby hearing great so that they can compete with their hearing peers,” Dr. Vaughan said. “The other big thing on the horizon is unilateral loss and being able to implant a cochlear device in just one ear. In the past we've thought hearing from one ear will suffice. But what we've realized over the last 15 years is that kids with hearing loss in one ear have lots of trouble in the classroom because of background noise and localization. Now we can make both ears equal.”

Hearing aids have come a long way, too. Gone are the days of manually turning the volume or frequencies on hearing aids up and down using a screwdriver. Now, hearing aids are sleeker, come in multiple colors, are equipped with bluetooth technology and are digitally programmed to allow individuals to hear everything from the tiniest frequency to the biggest sound.

Cook Children’s Audiology services offer these interventions, as well as bone conduction implantable hearing devices and a full range of auditory testing. They also provide protective devices such as custom earplugs, like those that help protect the hearing of musicians while also allowing them to hear each musical note. 

“Musician plugs have a filter on the end that allows musicians to hear the sound but at a lower volume. They do not distort or affect the quality of the music,” Dr. Vaughan explained. “We see a lot of performers now with monitors in their ears. Those are also to protect their hearing so they can perform longer. These are great for kids in high school bands or those that like listening to loud music.”

Early Ear Intervention

Protecting your child’s hearing and having your newborn screened for hearing loss are the two most important things parents can do for their child’s audiology health and for their speech and language development. 

Newborn hearing screenings are required at any birthing center with 50 or more births per year. If a baby fails the screening at birth, they can be rescreened at one month. If they fail the second screening, a diagnostic evaluation can confirm hearing loss by three months and intervention can begin by six months, giving a child the best chance at language development on par with their hearing peers. 

“Without that, when we wait until they are two or three and discover their words aren’t sounding great or they aren’t talking at all, then their brains have missed two to three years of learning,” Dr. Vaughan said. “It’s really hard to backtrack to correct. Newborn hearing screening is so very important. If we do the hard work on the front end and get things moving, hearing impaired children perform at equal levels to fully hearing children.”

The James family’s urgency to have Pierce fitted for hearing aids as soon as possible following cancer treatment set their once-hearing child on a course to recover as much speech and language development as possible. Since the completion of cancer treatment one year ago, Pierce has relearned to crawl and walk, and recovered many fine motor skills. He’s beginning to vocalize a number of sounds, too, including the sweetest sound to every mother’s ears — her baby calling for “mama.” 

Related Stories:

Exploring pediatric audiology and advancing development for children with hearing loss | CookChildrens.org

Let's begin by looking at the facts:

• About one to three babies out of every 1000 will be born with a permanent hearing loss.
• Infant hearing loss is the most common health condition identified through routine newborn screens.
• Nine out of 10 cases of genetic hearing loss occur in families where both parents have normal hearing.

The earlier children with hearing loss are identified and start getting help, the more likely they will reach their full potential. Hearing loss can cause children to fall behind their peers in speech, learning, reading, and social-emotional development, which can result in lower educational and employment levels into adulthood.

1-3-6 Goal

In fact, the Centers for Disease Control recommend the following 1-3-6 Goal:

1 — All infants get a hearing screen before 1 month of age.

3 — Hearing loss is confirmed before 3 months of age.

6 — Hearing loss intervention begins before 6 months of age.

So now that we know it’s better to identify hearing loss as a baby, just how are babies tested anyway? There are two types of tests used to screen hearing in babies and both are safe and painless.

Types of Hearing Screens

Otoacoustic Emissions (OAE)

For this test, a small earphone and microphone are placed in the ear, sounds are played, and a response is measured in the ear canal. If a baby hears normally, an echo is reflected back and measured by the microphone. When a baby has a hearing loss, no echo can be measured on the OAE test.

Auditory Brainstem Response (ABR)

For this test, sticker electrodes are placed on the baby's head to detect responses while sounds are played in the baby's ears. This test measures how the hearing nerve responds to sounds and can identify babies who have a hearing loss.

The two tests can be used separately or together. Both tests require a quiet, if not sleeping baby. If a problem is found, follow-up testing should happen as soon as possible.

What if My Baby Fails a Hearing Screen?

If your baby fails a hearing screen, you will be referred to an outpatient facility where an Audiologist will complete a more in depth ABR. It will measure the softest level that the ear responds to sounds using different pitches or tones. If testing is done before a baby is 4 months old, the baby can usually sleep naturally for the testing. For older or very active babies, sedation may be needed to help them sleep during testing. It is important that babies are sleeping for ABR testing.

The newborn hearing screen is one of the most important first tests your newborn takes. Babies start learning how to use sound as soon as they are born and listening prepares them to speak. Therefore, if a baby has hearing loss it is important that services begin right away. If you have any questions or concerns about your child’s hearing, contact your Pediatrician and ask about seeing an Audiologist.

See below for additional resources on newborn hearing screening:

• Cook Children's Audiology
• CDC information on hearing loss/screening
• Babyhearing.org
• Joint Committee on Infant Hearing

About the author

Christina Graham is an audiologist at Cook Children's. Cook Children's audiologists focus on diagnosing specific types of hearing loss for kids. They work closely with the child, his/her family, speech pathologists, school personnel, specialists, and physicians to create a plan that will enhance a child's functional hearing and help them learn and develop. To learn more, click here. To make an appointment, click here.