Hear, and Now: Restoring Sound Perception After Hearing Loss

The Caruso family was caught up in a movie at home earlier this year when daughter Gianna, 15, made the announcement that would change her father’s life.
“Dad and Mom, I hear the rain.”
Rick Caruso ’80 smiled. “Yeah, it’s raining,” he said, turning his attention back to the movie.
“No, Dad,” said the teen. “I hear the rain.”
Her words clicked. Born with inner-ear hearing loss, Gianna had just done what was once impossible for her. Rick and Tina Caruso realized that their daughter could hear even the faint tapping of raindrops, thanks to a recently implanted hearing device.
Such life-changing leaps happen daily within the USC Tina and Rick Caruso Department of Otolaryngology – Head and Neck Surgery, renowned for its innovative treatments and research addressing hearing loss in children and adults.
Keck Medicine of USC physicians’ efforts to break the sound barrier are gaining added momentum from a transformative $25 million gift from the Caruso family. Their gift in June endowed and named not only the USC Caruso Department, but also the USC Caruso Family Center for Childhood Communication, a treatment center affiliated with Children’s Hospital Los Angeles.
“This is a very personal mission for us,” says Caruso, a USC Trustee who graduated with honors from the USC Marshall School of Business.
For physician John K. Niparko, the support bolsters what he calls a “talented, dedicated team” in the department.
“The fact that we have partners like the Caruso family is beyond expectation and quite humbling—it inspires us all the more,” says Niparko, chair of the USC Caruso Department and the Leon J. Tiber and David S. Alpert Chair in Medicine at the Keck School of Medicine of USC.
Two years ago, working in tandem with audiologist Kristina Rousso, Niparko introduced Gianna to a new, in-the-ear device, which sits deep in both of her ear canals. When it was activated, Gianna began to cry. It was the first time she’d heard her parents’ voices clearly. “It was a great day—one of the most amazing days in our lives,” Gianna’s father says.
That experience motivated the Carusos to ask an important question, he says: “What more could we do to help kids who don’t have the ability to get this kind of medical support or technology?”
The need is there. About one in 1,000 children has profound hearing loss at birth, and by 5 years, that increases to one in 500. The impact can be dramatic. “Early sound exposure is absolutely essential for a child’s development in so many dimensions,” Niparko says. “We have an opportunity to engage the brain circuits of the developing child in the first few years—circuits highly dedicated to language learning.”
Niparko is a leading authority on implantable technologies and principal investigator on a 14-year National Institutes of Health (NIH)-funded study on cochlear implants. He’s also passionate about music and art—both of which are, “at their core, about communication,” he says.
Andy and Jennifer Garcia of Long Beach, California, wanted to help their daughter Amanda access that world after she was born with mild to moderate hearing loss caused by a virus. At first, Amanda did well with a single hearing aid. Then, a serious ear infection left her deaf in one ear. As Amanda’s hearing deteriorated, so did her speech and her self-confidence, recalls her mother. The family turned to Niparko for help, and at age 9, Amanda received a cochlear implant. The moment it switched on, “her eyes got big!” Jennifer Garcia says.
The cochlear implant differs from a hearing aid—essentially an amplifier—by bypassing the damaged cochlea, part of the inner ear with nerve endings essential for hearing, to directly stimulate the auditory nerve. With training, children learn to recognize the sounds of language. For many, that work takes place at the USC Caruso Family Center, where audiologists, speech-language pathologists and education experts from Keck School of Medicine and Children’s Hospital Los Angeles care for some 5,000 children and young people annually. Expect that number to expand, especially as interactions between the USC Caruso Family Center and its partner on the same campus—John Tracy Clinic—continue to grow.
The Lundie family went through the journey with their son Shane, who was born profoundly deaf. When he was about a year old, Shane underwent a successful cochlear implant procedure, allowing him to hear sound for the first time. But the journey didn’t end there. The Lundies worked closely with the USC Caruso Family Center for Childhood Communication to ensure that he continued developing his hearing and communication skills.
At the USC Caruso Family Center, families like the Lundies and Garcias first enter a sound booth equipped with state-of-the-art audiometers that focus on frequencies in the speech range. Young children use puzzle pieces to signal when a sound registers. Even babies can be evaluated: When they turn their heads at a sound, they’re rewarded with the sight of a toy bear playing the drums. Speech-language pathologists also use play-based activities to assess a child’s language skills. Just five months after hearing sound for the first time, Shane was able to say a new word: “Mama.” It was, as his mother Kelly puts it, the “best Christmas gift ever.”
“We want each child to have the richest possible language experience,” says pediatric audiologist Margaret Winter. To that end, the USC Caruso Family Center has also developed its own literacy-training program—“Come Read With Me”—for families who speak English as their second language. Children, teachers and parents participate. Analysis shows major gains in children’s reading and writing years after training. Following this summer’s program, one mother noted that her daughter “now prefers books over Barbies,” Niparko says.
Other research explores new technologies. For children missing an auditory nerve—necessary to transmit sound from inner ear to brain—the answer may lie in what’s called an auditory brainstem implant, or ABI. The FDA approved a Phase I clinical trial for ABI in children under 5 years old through the Keck School of Medicine, working with the House Clinic.
In the five-year clinical trial—the only one in the U.S. to gain NIH funding—House Clinic and Children’s Hospital Los Angeles surgeons will implant ABI devices in as many as 10 children, who will be monitored for three years. With the first procedure in 2014, it’s too soon to tell how well the ABI may help children develop speech over time. “That’s a question we all hope to answer,” says Keck Medicine audiologist Laurie Eisenberg, study co-leader.
Over the course of her career, Eisenberg has seen the field of assistive devices flourish. “Forty years ago, no one could have predicted where we are today,” she says.
Others in the department turn to human biology to target hearing loss. In his quest to understand the embryonic development of the inner ear, USC researcher Takahiro Ohyama recently identified a key molecular sequence that may someday open up avenues for gene therapy.
And scientist Neil Segil studies cellular reprogramming to try to regenerate hair cells, delicate structures in the inner ear that are critical for hearing.
Some animal species, including birds, frogs and fish, can regenerate damaged or lost hearing by growing new hair cells. Mammals can’t. But Segil’s team was the first to show that what are called supporting cells—neighbors to hair cells—could be reprogrammed in mammals to generate hair cells at an early stage of life. Now Segil, a professor of research, is tapping into stem cell techniques developed at the Keck School of Medicine that trigger skin cells to turn into other types of cells. Preliminary data suggest that hair cells develop directly from a type of skin-related stem cell, Segil says. It’s early, but he’s cautiously excited about the possibilities.
Today Gianna, Amanda, Shane and others like them are beneficiaries of decades of advances in hearing research. Gianna can now hear her coaches when she competes in her equestrian events, and she wants to volunteer at the USC Caruso Family Center. Amanda, 10, felt confident enough to talk to her Girl Scout troop about her cochlear implant. Two-year-old Shane loves to dance to the radio. And he’s catching words “even at a whisper—mind-boggling!” says his mother. Every time Shane hears a new sound, he points to his ear and says another word Mom loves to hear.
“On.”
By Candace Pearson