Natalia Zariella Guerrero is excited to turn 7 soon. A happy child who adores her brother—older by just 19 months—Natalia loves to color, play sports, swim and compete against her brother in video games. Her mother, Zenia Villarreal, describes Natalia as inquisitive, fearless (she loves rollercoasters) and a truly special little girl.
“She is beautiful and unique—one of a kind,” Zenia said.
One of a Natalia’s unique qualities is that she is deaf, and she communicates using only American Sign Language.
Natalia was born on October 26, 2015, at Children’s Memorial Hermann Hospital in the Texas Medical Center. Zenia, who works for Memorial Hermann Health System as an OR lead coordinator, was thrilled to welcome her beautiful baby girl into the world. But that joy turned to fear when her daughter was rushed to the NICU for severely low blood sugar. The neonatal intensive care unit (NICU) at Children’s Memorial Hermann Hospital has 118 beds and is classified as a Level IV NICU by the Texas Department of State Health Services (DSHS)—the highest level of care available for premature and critically ill newborns.
“They were able to stabilize her, but she was there for almost a week,” Zenia said.
While she was in the NICU being treated for her low blood sugar, Natalia also underwent all the standard newborn testing, including a newborn hearing screening. It was after that test when Zenia first learned there may be a problem with her daughter’s ability to hear.
“I remember they told her dad and me that she ‘barely passed,’ and that she needed to see a specialist,” Zenia recalled.
During a regular new baby visit, Natalia’s pediatrician, Dr. Rachel Baker, at Children’s Memorial Hermann Pediatrics West University, referred her to Dr. Sancak Yuksel, an associate professor in the Department of Otorhinolaryngology at McGovern Medical School at UTHealth Houston and a pediatric otorhinolaryngologist affiliated with Children’s Memorial Hermann Hospital and UT Physicians Otorhinolaryngology.
In the U.S., it is recommended that every newborn be screened for hearing loss. According to the Centers for Disease Control and Prevention (CDC), approximately 1 to 3 per 1,000 children have hearing loss.
As it turns out, Natalia was one of them.
“When a child fails a newborn hearing screening, he or she needs to be evaluated by an audiologist to get a full hearing evaluation as soon as possible,” Dr. Yuksel explained. “This is important because when it comes to intervention, the earlier the better. Babies begin to learn and develop immediately after birth, so we want to diagnose any issues as soon as possible.”
Dr. Yuksel added that in some cases, babies may pass the newborn hearing test, but other red flags may indicate an issue. He said that if a baby doesn’t startle at loud noises, does not turn their head toward sound, has balance or vision problems, or is not saying simple words by the time they reach one year of age—such as “mama” or “dada”—it is important parents seek out an evaluation in a timely manner.
Dr. Yuksel explained that when a child is referred to Pediatric Audiology at Children’s Memorial Hermann Hospital, a specialist will perform a full hearing evaluation to determine what may be causing the hearing loss.
“In some cases, it could be a simple cause like effusion, or fluid, in the middle ear canal, which can accumulate during delivery,” Dr. Yuksel said. “We can treat this by placing tubes in the ears, which drains the fluid and will restore hearing.”
If the root cause is not effusion, a child’s hearing loss may be characterized as sensorineural hearing loss which is caused by damage to nerve fibers in the inner ear that carry signals to the brain. This type of hearing loss is congenital (meaning present at birth) and often has a genetic cause.
These cases are diagnosed through an Auditory Brainstem Response (ABR) test, which is a special hearing test that measures how well sound is transmitted to the brain—an objective hearing evaluation tool that does not require the patient’s subjective input. In this test, electrodes are placed on the earlobes and forehead to measure responses to clicking sounds, which can detect interruptions in sound transmitted to the hearing nerve.
Typically, children are first fitted for hearing aids. If this treatment does not improve hearing, a cochlear implant—which is a surgically implanted neuro-prosthesis that can help a person withhearing loss experience sound perception—may be considered. Dr. Yuksel emphasized how important it is to diagnose these conditions in the first months of life.
“It is best to determine a diagnosis by the time a child is at least 3 months old, so that they can be fitted for a hearing aid by the age of 6 months,” Dr. Yuksel said. “If a child does not benefit from a hearing aid, then we would explore a cochlear implant by 12 months of age.”
After her initial evaluation1, it was determined that Natalia did in fact have some fluid in her middle ear, so Dr. Yuksel placed tubes in hopes that they would drain the effusion and improve her hearing. During the procedure, however, he could not find any effusion.
“That was the first red flag that there may be something more—that she may have sensorineural hearing loss,” Dr. Yuksel said. “After we put the tubes in, the first thing we did was repeat the hearing test, and unfortunately, she failed that one as well.”
The next step was to do a full workup, including a CT scan, which helps determine any abnormality in the ear structure, and the ABR evaluation. In the meantime, Natalia was also being evaluated by Dr. Ian Butler, a professor of pediatrics at McGovern Medical School at UTHealth Houston and a pediatric neurologist affiliated with Children’s Memorial Hermann Hospital who also sees patients at UT Physicians. Dr. Butler worked concurrently with the audiology team to fully evaluate Natalia’s unique symptoms.
The result of the ABR was definitive: Natalia had profound hearing loss.
“I remember that being a very sad and challenging day,” Zenia said. “When we got the results, we were told that Natalia was profoundly deaf in the right ear and had auditory neuropathy disorder in the left ear. I remember my mind going blank and not being able to process anything else—I was in shock.”
According to the CT scan, Natalia’s right inner ear canal was absent, indicating that she would never be able to hear on that side, even with a hearing aid or cochlear implant. The left side was also abnormal, with the inner ear canal being severely underdeveloped, causing an interruption with the ear sending sound to the brain.
Because of the nature of Natalia’s condition, hearing aids were not beneficial. The final option was a cochlear implant in her left ear, but even that was unlikely to be successful. Still, Natalia’s parents were willing to try anything, so in 2017, when Natalia was 20 months old, Dr. Yuksel performed the cochlear implant procedure at Children’s Day Surgery at Children’s Memorial Hermann Hospital. Unfortunately, it was unable to restore the sound delivery system to her brain.
Natalia’s condition is rare and complex, with a likely genetic component to her hearing loss. As Natalia’s parents worked closely with specialists to exhaust all options, they also had their daughter evaluated for therapies to help her overcome her challenges. Working with highly specialized therapists for both physical and speech therapies at TIRR Memorial Hermann, Natalia is doing just that. Continually recognized as one of America's Best Rehabilitation Hospitals by U.S. News & World Report, TIRR Memorial Hermann is a national leader in medical rehabilitation and research.
“Natalia started physical therapy a little after she turned 8 months because she could not sit up or hold her head upright,” Zenia said. “She never crawled like a normal baby, but she instead used her upper body to drag her lower body—what we call ‘army crawling.’ One of her many tests showed that she is missing cranial nerve 7 and 8; one has to do with her balance and the other with facial expressions. But we are working through all of that.”
After her diagnosis, Natalia’s parents dove into resources for the Deaf community. In addition to accessing any necessary follow-up care from Children’s Memorial Hermann Hospital, they enrolled Natalia in school and the entire family is learning American Sign Language. Today, Natalia is thriving. She has just started first grade—her first year in a mainstream classroom—and she cannot wait for everything she will learn in the year ahead.
“She will have an interpreter in class to help her, but otherwise she will be learning just like all the other kids,” Zenia said.
Natalia’s parents hope that by sharing their daughter’s story, they can help spread awareness about congenital hearing loss, including the importance of seeking out specialists for diagnosis and treatment, as well as the many resources available to the Deaf community.
“I have already learned so much and we are continuing to learn and grow together,” Zenia said. “But she is one of my miracles and is perfect the way she is. She amazes me every single day.”
1 Affiliated physicians evaluate patients at UT Physicians clinic locations and perform all inpatient procedures and treatments at Children’s Memorial Hermann Hospital.