Born deaf in her left ear, little Hazel Goerger went through numerous tests to figure out the cause of her hearing loss. Because she appeared to be otherwise healthy, her doctors suspected a genetic or structural issue was to blame.

But when Hazel also showed signs of a delay in her gross motor development at 2 months of age, a special education teacher who works with infants with hearing loss wondered whether cytomegalovirus (CMV) was instead responsible. One of every 100 babies born in the United States is infected with CMV, according to the Centers for Disease Control and Prevention. But only about 20 percent suffer long-term consequences such as hearing loss, vision loss, developmental delays, cerebral palsy, and seizure disorders. 

CMV is a tricky infection, and its effects vary widely. Most people contract it in their lifetime, says University of Minnesota Medical School infectious disease specialist Mark Schleiss, and for most it will do little harm. 

Babies born to women who become infected for the very first time during pregnancy, however, are the most likely to develop disabilities. And since CMV is transmitted through bodily fluids such as saliva from a sloppy kiss, pregnant mothers who have young children in day care have a high risk of becoming infected.

Hazel’s pediatrician thought CMV was unlikely to have caused her hearing loss. But her parents pushed to have her tested. At 10 months, through retrospective testing of a blood sample given at birth, they learned Hazel had been born with CMV.  

Hazel’s ear, nose, and throat doctor referred her parents, Heather McElroy-Goerger and Dan Goerger, to Schleiss at University of Minnesota Masonic Children’s Hospital. Schleiss is internationally known for his laboratory research on CMV as well as his efforts to raise awareness of the infection and its potential consequences. 

Testing at the U revealed white-matter damage in Hazel’s brain consistent with CMV, in addition to her hearing loss. It was a blow—and a surprise—to the family. “Unfortunately, it’s hard to say what this means for her,” McElroy-Goerger says. “We don’t really know exactly what it’s going to look like.”

MINIMIZING DAMAGE

Together, Schleiss and the Goergers decided to put Hazel on a yearlong course of antiviral medication in hopes of preserving the hearing in her right ear and minimizing the wrath of CMV on her developing body.

“For a baby who has congenital CMV diagnosed in the first three weeks of life, if they’re treated with an antiviral medicine and they have a symptomatic infection, we know that six months of treatment improves their outcome,” Schleiss says. “It improves their hearing, and it improves their neurodevelopmental outcome. We don’t really know whether treatment of an older baby or toddler would provide a benefit.”

A National Institutes of Health study aims to determine whether antiviral medication also might help older babies and toddlers. But investigators won’t have the final data for years.

That’s why it’s so critical not to miss the diagnosis, Schleiss says. “The earlier you intervene, perhaps the more likely you are to limit the damage,” he says. 

Schleiss has created and implemented a new standard of care in the Fairview Health Services system that involves CMV testing for all newborns who do not pass a hearing screening before they leave the nursery.  

The protocol was developed with support from a three-year grant from the Vikings Foundation, a longtime partner of the Medical School’s Department of Pediatrics. Hence, the initiative’s football-inspired slogan: “If you don’t pass, screen.”

GOING FURTHER

Schleiss isn’t limiting his efforts to screen newborns for CMV to Fairview. He’s also advocating for CMV testing to be added to the Minnesota Department of Health’s newborn screening program. 

In a pilot study with the health department, Schleiss and his team have screened more than 7,000 newborns for CMV, regardless of whether they passed their hearing test. 

Those found to have an asymptomatic CMV infection will be followed for signs of hearing loss or developmental delays.“My ultimate goal would be to see Minnesota become the first state to adopt universal newborn screening for cytomegalovirus,” he says.

Hazel Goerger’s family would like to see that happen as well. “If Hazel had been tested, we could have started her on antivirals immediately and very likely have prevented further hearing loss,” McElroy-Goerger says.  

So far, 2-year-old Hazel is meeting developmental milestones and will be monitored closely as she grows. With the help of weekly speech therapy and regular audiology visits, Hazel has amassed a substantial vocabulary and is working hard on articulation. She loves chasing her 4-year-old sister around the house, helping in the kitchen, and singing “Baa Baa Black Sheep” at the top of her lungs. 

“I don’t know what we would have done without Dr. Schleiss being an invaluable intellectual and emotional resource,” McElroy-Goerger says. “We feel like we’re working with the best possible information when we make tough decisions for her.”

Nicole Endres is a contributing editor for Legacy magazine.