In 2006, Laura Barbieri was delighted with her role as a clarinetist with the Houston Grand Opera Orchestra, when she noticed one day that the hearing in her right ear seemed to be blocked. “It was shortly after I had the flu,” she recalls, “so I just assumed it was a lingering symptom.” But the problem persisted, and in 2008 she experienced progressive hearing loss in that ear as well as tinnitus. A brain scan revealed a benign tumor the size of a grape growing on the nerves that control hearing and balance. In April 2009, the tumor was removed by a surgeon in Los Angeles, but by then Barbieri had completely lost his hearing in his right ear.
Fearing further damage to his hearing, Barbieri consulted several neurosurgeons, including John G. Golfinos, MD, chairman of the department of neurosurgery at NYU Grossman School of Medicine. Dr. Golfinos told her he suspected an underlying condition known as neurofibromatosis type 2 (NF2). A genetic disease that affects approximately 1 in 25,000 people, NF2 causes tumors to grow on the nerves of the peripheral nervous system.
Dr. Golfinos professional instinct proved correct. A follow-up scan showed another budding tumor on the nerves of his left ear. “I went to a ton of doctors,” she recalls, “but Dr. Golfinos was the only one who discerned that I had NF2.” For Barbieri, a classical musician who had spent two decades honing her skills to earn a coveted spot in a major orchestra, the diagnosis was “shattering”. She describes music as her first language. “Acting,” she says, “was the thing that fulfilled me the most, the thing that I felt I had the most to offer the world.”
In 2010, Barbieri moved to New York for a job, settling in the Prospect Heights neighborhood of Brooklyn. She continued her care at the Comprehensive Neurofibromatosis Center at NYU Langone, the largest neurofibromatosis clinic in the United States. The connection not only offered him the highest level of multidisciplinary expertise, but also led to unprecedented surgery to stem the growing damage caused by NF2.
By January 2021, Barbieri had become completely deaf and began noticing tingling on the left side of her face, as well as weakness that prevented her from smiling or raising an eyebrow. A tumor compressing the brainstem can affect facial movements, swallowing, and vocalization, among other things. Barbieri was forced to communicate with a text-to-speech app on her phone.
With Barbieri’s auditory nerve now irreparably damaged, J. Thomas Roland, Jr., MD, chair of the department of otolaryngology-head and neck surgery at NYU Grossman School of Medicine, recommended an implant Auditory Brainstem (ABI), a device that can recreate the electrical pathways that normally stimulate the auditory nerve and allow the brain to interpret sound. NYU Langone is one of only three East Coast medical centers to offer the device. Since its approval by the United States Food and Drug Administration (FDA) for patients with NF2 in 2000, in part due to Dr. Roland’s pioneering work on the clinical trial, Dr. Roland and his team of neurotologists have successfully treated more than 150 patients. “I can’t cure this disease surgically,” says Dr. Roland, “so my goal is to restore some of the hearing that the patient has lost.”
A complex case like Barbieri’s would typically involve three separate surgeries – one to remove the tumor, another to implant the ABI and a third to resuscitate the facial nerves – each requiring a lengthy recovery. But Dr. Roland devised a surgical plan — never before attempted at NYU Langone or, to his knowledge, at any other medical center — in which all three operations could be performed sequentially in a single day. He knew Barbieri was up for it. “Laura is a remarkable person,” he says. Barbieri had already completed 3 Ironman triathlons, a dozen Half Ironman competitions and 24 marathons – feats which, she says, “made me feel like I was stronger than the disease.”
Dr. Roland devised a surgical plan—never attempted before at NYU Langone or, to his knowledge, at any other medical center—in which all three operations could be performed sequentially in a single day.
In March 2021, 15 years after neurofibromatosis began to rob Barbieri of her hearing and her livelihood, she walked into an operating room at NYU Langone to regain some of what she had lost. After Dr. Golfinos and Dr. Roland removed the tumor, they began the process of implanting the ABI. Then Adam S. Jacobson, MD, a head and neck surgeon who is director of the Center for Facial Paralysis and Resuscitation, performed transplants to rewire Barbieri’s facial nerves and provide new electrical power to weak muscles that , over time, would restore tone and movement to the left side of his face. “Teamwork is what allows us to tackle the toughest cases with confidence,” notes Dr. Golfinos. “It is unlikely that we will run into any issues that we haven’t seen before, but if we do we will be able to manage them.”
Barbieri, now married, reports that she has already regained some facial tone and movement. Dr. Jacobson expects her to continue to improve for up to two years after surgery. “My doctors at NYU Langone have been absolutely amazing,” Barbieri says. Although she is still unable to hear music, she credits the ABI for keeping her connected. “It refreshes my memory of music and allows me to imagine what might be going on in my mind,” she says. “If there is music, at least I hear the rhythm, the percussion. These little things make a big difference. Barbieri’s recovery inspired her to pursue a new professional direction. Formerly in sales, she now thinks of working with people who have suffered from hearing loss. “As someone who’s been through this and been a musician, I think I’m in a unique position to help people through this process,” she says.