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Kira was safely awakened during brain surgery so neurosurgeons could map her brain in real time. (Photo provided by Seattle Children's)

This 19-year-old sang her way through brain surgery

About four years ago, Kira Iaconetti, 19, began noticing something weird that would happen when she was singing or listening to music.

“It was like a light switch turned off in my brain,” said Iaconetti, a talented self-taught musician who has been performing in musicals since she was 6 years old. “Suddenly, I was tone deaf, I couldn’t process the words in time with the music and I couldn’t sing.”

At first, she thought the two-minute glitches were common, bound to happen to any serious performer at some point. Once it passed, she’d usually be able to go on singing even though her energy was zapped.

Worried that it would eventually affect her performance on stage, she followed up with a neurologist she had previously visited near her home in Lynden, Washington, when the episodes started becoming more pronounced.

“Forcing myself to sing after one of these glitches was extremely difficult by this point. I would become incoherent, slurring and stuttering my words,” she said. “That was good enough reason to go back to the neurologist.”

Music-triggered epilepsy

The neurologist determined Iaconetti’s episodes were actually seizures that were particularly difficult to pinpoint because they were often only triggered by music. The cause of her seizures remained unknown until a magnetic resonance imaging (MRI) scan at Seattle Children’s revealed a marble-sized mass in the right temporal lobe of her brain.

“Her tumor was discovered because of a very unusual type of epilepsy she had called musicogenic epilepsy,” said Dr. Jason Hauptman, a neurosurgeon in Seattle Children’s Neurosciences Center. “These seizures are triggered by listening to music or singing, which is an unfortunate problem for Kira since she is a performer who likes to sing.”

Hauptman described how the calcified tumor was pressing up against her auditory cortex, which likely explained the occurrence of the seizures during her performances.

Novel surgery to preserve a passion

Iaconetti would need brain surgery to remove the tumor. Because of the tumor’s location, the Seattle Children’s epilepsy surgery team took an innovative, personalized approach to planning her surgery.

“In a sort of twisted joke from the universe the tumor was right inside the area of my brain that controls my hearing and singing ability,” Iaconetti said. “Messing with it could permanently affect my voice, and because Dr. Hauptman knew how important it is to me to continue singing and acting, he wanted to be very careful when removing the tumor. He didn’t want to interfere with my ability to sing.”

With input from colleagues from a range of specialties, Hauptman decided to have Iaconetti wake up during surgery and complete musical tasks while mapping the areas of her brain that were used. When removing the tumor, Hauptman would use the perimeters defined during the mapping to steer clear of the areas of Iaconetti’s brain that gave her the ability to produce and interpret music.

“Our focus was not only on taking care of the tumor, but making her life better. We wanted to preserve the things she cares about like her passion for pursuing a career in musical theater,” Hauptman said.

Seattle Children’s is a leader in performing the advanced neurosurgical procedure Iaconetti would undergo, which is known as an awake craniotomy. Most often the child performs language tasks to preserve vital cognitive functions during an awake craniotomy. An awake craniotomy that tested for musical ability, including rhythm and intonation, had never been done at Seattle Children’s before.

Bringing together a team of experts

To prepare for the first-of-its-kind surgery, neuropsychologists, Drs. Hillary Shurtleff and Molly Warner, in conjunction with David Knott, music therapist, worked with each other and Iaconetti to develop the musical tasks she would complete during surgical planning and in the surgery itself.

Shurtleff and Dr. Andrew Poliakov, an expert in using innovative imaging techniques to understand neurological conditions in children, created unique stimuli to activate music-related areas around her tumor. With an advanced imaging technique called functional magnetic resonance imaging (fMRI), they mapped the critical areas of Iaconetti’s brain activated when she performed the set musical tasks in pre-surgical appointments. The fMRI scans provided Hauptman an initial roadmap to use as a guide during the intraoperative awake mapping exercises.

Knott would decipher whether Iaconetti’s musical ability was interrupted when certain areas of her brain were probed by Hauptman during surgery.

“We’ve never had a patient sing in the operating room before, and Kira is such a talented musician,” Shurtleff said. “Her voice is so beautiful and her willingness to do something new helped make the whole process interactive, collaborative and exciting.”

Awake and ready to sing

On the day of the surgery, the surgical team, including Hauptman, Shurtleff and Knott gathered around Iaconetti on the operating table. After putting her to sleep to remove a small window of her skull to access the area of the brain where the tumor resides, the anesthesiologists woke Iaconetti up.

More quickly than one might expect, she was conscious and responsive.

Then the team led Iaconetti through the awake mapping. As Shurtleff coached her through the three musical tasks they had prepped the week prior, Hauptman stimulated her brain with gentle electrical pulses. Knott listened closely to make sure she was accurate and on pitch. If any of the areas Hauptman stimulated interrupted her ability to either listen or sing, he considered that area integral to her singing function and avoid it when removing the tumor.

Before surgery, Iaconetti and Knott had gone through a list of songs handpicked from her playlists. Knott selected a song he thought she could sing during surgery. For Iaconetti, it was a song that reminds her of her family and her birthplace in Hawaii.

As Hauptman started to remove the tumor, Iaconetti gradually warmed up into the main chorus of the selected song, Weezer’s 2001 hit, “Island in the Sun,” while still on the operating room table.

Then, on her second time through the song, something magical happened. Members of the surgical team began softly singing along with her.

Several smiled as they reached a line in the song that states, “I can’t control my brain.”

As if on cue, Iaconetti replied, “Literally.”

A step closer to getting back onstage

With the tumor removed, Iaconetti was put back to sleep for the final parts of the surgery. Just 48 hours later, Iaconetti was playing guitar and singing with Knott from her inpatient hospital bed.

“After taking some time to warm up, she nailed the pitches and her tone sounded great,” Knott said. “She was projecting her voice. It was very encouraging to see her sing and communicate musically in such a strong way so soon after brain surgery.”

The pathology results identify the mass removed by Hauptman as a low-grade glioma. According to Hauptman it’s unlikely that Iaconetti will need any further treatment for the tumor. Her prognosis is good.

“At Seattle Children’s, we deal with the incredible every day,” Hauptman said. “Kira is a remarkable young lady who had a terrible problem. We came together and developed a very novel way to approach her problem that we’re hoping will have a positive impact for the rest of her life.”

As for Iaconetti, she already has her sights set on her next audition.

“My biggest fear before the surgery was that the seizures would get in the way of performing,” she said. “Now, I want to get back to the stage, to performing as soon as I can.”

For kids with complex neurological disorders who have few or no other treatment options, neurosurgical innovations can offer new hope, and even a cure, which is one of the reasons Seattle Children's launched It Starts With Yes: The Campaign for Seattle Children’s. It Starts With Yes is a $1 billion initiative with a bold vision to transform children’s health. With your help, they will continue to provide financial assistance for families in need; expand necessary healthcare and research facilities; and invest in clinical and research programs to advance pediatric medicine. Learn more or join It Starts With Yes to improve how Seattle Children's cares for kids like Iaconetti and transforms childhood health for generations to come.