Two days shy of her 20th birthday, Sam Marino faced a difficult choice: save her lungs or preserve her hearing.
It was 2021, and Marino, a college student who lives in Oxford, Conn., was battling a bacterial lung infection that required a particular antibiotic, which could cause hearing loss. The infection was a complication of cystic fibrosis, a progressive genetic disease that damages the lungs, digestive system, and other organs in the body.
The antibiotic Marino needed, amikacin, is commonly used to treat bacterial infections in patients with cystic fibrosis. However, it is associated with a risk of hearing loss that increases with larger doses or a longer duration of therapy. She had been on amikacin twice before without any serious side effects. But the infection was now taking over her airways, and a much longer course of the drug, delivered intravenously, was needed.
She understood the risk but didn’t believe it would affect her—until a few months later when she noticed a “ringing” in her ears (commonly known as tinnitus). Marino turned to her Yale Medicine Cystic Fibrosis Program team for guidance on whether she should stop taking amikacin. “They told me I needed my lungs to live and that my hearing could be addressed in other ways,” recalls Marino, now 22.
She stayed on amikacin and began to wear hearing aids. Still, the hearing loss progressed. Her doctors switched her to an inhaled antibiotic in hopes that it would slow down her hearing loss. It did not. The antibiotic was damaging and even killing the hair cells in the cochlea (inner ear) that translate vibrations from sound waves into electrical signals, which are then transmitted to the auditory nerve and the brain. As the hair cells continued to die, Marino’s hearing dramatically declined. Soon, the problem could not even be fixed with hearing aids, which amplify sound but can’t restore the electrical signals of the hair cells.
“I just couldn’t believe I had another serious medical issue. I was grieving a piece of myself, and it caused a huge identity crisis because I’m pursuing a career where hearing is vital,” says Marino, who is about to graduate from Post University in Waterbury, Conn., with a degree in psychology. She plans to pursue a master’s degree at Sacred Heart University in Fairfield, Conn., to become a mental health counselor for children.
Marino knew she had to take action. She couldn’t go off the antibiotic because it was helping her fight the lung infection, but the hearing aids were not enough. So, in the fall of 2022, she met with audiologist Meg Narron, AuD, and head and neck surgeon Nofrat Schwartz, MD, a neurotologist with special training in inner ear problems, in the Yale Medicine Hearing & Balance Program. They discussed the possibility of a cochlear implant, an electronic hearing device that is implanted under the skin of the ear and works with an external processor worn behind the ear. An electrode is placed in the inner ear to bypass the damaged hair cells and directly stimulate the auditory nerve.
"Cochlear implants,” Dr. Schwartz explains, “are the only tool that can restore hearing when someone has the type and severity of hearing loss Marino experienced.”
Cochlear implant surgery: How hearing is restored
In the meantime, Marino continued to struggle with hearing loss. This was compounded by the mask-wearing related to COVID-19. Because of the masks, she couldn’t rely on facial expressions or try to read lips, whether she was in class or working her part-time customer service job at a local grocery store. Plus, her ability to hear high-pitch sounds, which are the type that give clarity—or intelligibility—of speech, was impaired.
“I could hear my professors talking, but I couldn’t understand what they were saying,” Marino says.
Although Marino’s hearing loss was considered severe, she still had some natural, or acoustic, hearing, but it was limited to low pitches—also known as tones or frequencies, Dr. Schwartz explains.
For example, Marino could hear a barking dog but not a chirping bird. She could hear vowels but not consonants. The more Marino learned about cochlear implants, the better she felt about her decision to get them. “My doctors walked me through everything, and I knew what to expect,” she says.
A few days before Christmas in 2022, she had the surgery at Yale New Haven Hospital. Dr. Schwartz chose to operate on Marino’s right ear first.
“With adults, we typically do one ear at a time because we want to make sure they like it and have time to let the brain adjust to this new technology,” Dr. Schwartz says. “The second ear is usually done about six months later, if needed.”
The surgery, which took about an hour and a half, entailed putting Marino under general anesthesia, making an incision behind her ear, and then creating a small hole in the mastoid bone, which sits behind the ear, that reaches the cochlea. Dr. Schwartz inserted the small, thin receiver piece of the implant under the skin behind the ear, followed by the electrode (a thin wire) into the cochlea.
To preserve Marino’s low-pitch hearing, Dr. Schwartz did what’s called "hearing preservation surgery," which means she inserted the electrode through a natural opening of the inner ear. This “atraumatic” approach, which uses soft electrodes, minimizes tissue damage and results in high rates of natural hearing preservation.
In the past, it was assumed that cochlear implantation would damage any hair cells that remained, Dr. Schwartz explains. “But with advancements in surgical approaches and electrodes, we are able to mitigate this and allow the preservation of remaining hearing,” she says.
The surgery was a success, and, as with most people who undergo the procedure, Marino was able to go home that day.
Fine-tuning the cochlear implant’s processor after surgery
Two weeks after her surgery, Marino visited Narron to have the implant turned on and its sound processor adjusted. “They told me it was going to sound different from what I was used to and that it’s not the same for everyone. At first, there was static, and then I started to get used to it. But everyone sounded like Alvin and the Chipmunks to me,” Marino says.
Narron says Marino’s hearing possibly seemed “squeaky” because she hadn’t been able to hear high-pitch sounds for a significant amount of time. “That colors her experience because suddenly we are giving her high-pitch sounds, which can be overwhelming,” she says. “I’ve had people tell me I sound like every cartoon character, from Donald Duck to Mickey Mouse. I tell patients that the first day we turn on the implant will be their worst day of hearing; it will only get better with time.”
Indeed, Marino says familiar voices, including her family, now sound more normal, and other voices became less high-pitched about three months after her surgery.
“I also struggled with hearing music for a while, but I’m rounding the corner with that, too,” says Marino, who enjoys listening to country music, as well as walking her dogs, fishing, and line dancing.
Typically, patients meet with their audiologist about three times in the month after they receive their implant. Since that first month, Marino has met with Narron two times for adjustments and tweaks. “At this point, I’m not making big changes. We are just controlling how much electrical current goes from the device,” Narron says. “Once we reach the six-month mark from surgery, patients typically come to see me just once or twice a year.”
Marino, Narron says, has been a model patient. “She wears her implant consistently and does all the recommended practices, which include reading aloud to herself, streaming podcasts, and listening to music,” she says. “Bombarding the auditory system can only help her. She has taken that advice to heart and is doing fantastic. Her story is inspirational.”
As for the quality of her hearing, that, too, has been drastically improved. Before surgery, Marino’s ability to understand speech was 20% in her right ear while wearing a hearing aid. One month after surgery, she was at 78%, a number that will likely continue to improve before leveling off, Narron explains. Similarly, Marino’s ability to hear someone reading aloud in a quiet room went from 42% before surgery (with a hearing aid) to 97% after surgery.
Marino hopes to have cochlear implant surgery in her left ear this summer.
Now that she is addressing her hearing problems, Marino’s pulmonology team can continue to focus on the bacterial infection in her lungs. “At least we no longer have to worry about hearing loss as a side effect with any treatment options,” Marino says. “The goal, of course, is to clear the infection entirely. But these infections are extremely challenging to treat because they get stuck in the thick mucus of my lungs, which is caused by cystic fibrosis.”
In the meantime, Marino is grateful to have her hearing restored. She recently celebrated her first surgery by getting a rose tattoo over her surgery scar.
Roses, Marino explains, are often used to symbolize cystic fibrosis because children with the disease have a hard time pronouncing its name and instead say, “65 roses.” She also has a tattoo in her mother’s handwriting on her wrist that reads “Just breathe” next to a purple ribbon—the symbol for cystic fibrosis awareness.
Marino’s mother, she says, has been a guidepost in how she deals with her life challenges. “She always tells me to make the best of everything, and that’s what I try to do,” says Marino, who shares her story with others who have cystic fibrosis on the Cystic Fibrosis Foundation website and enjoys mentoring children with the disease. “Everyone else in my family is healthy. I’m the only one with this disease, and I believe it happened for a reason—so that I can help others.”