Teen Undergoes Skull Base Surgery for Acoustic Neuroma

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Meghan Shaw was no stranger to the otolaryngologist (ear, nose, and throat doctor). For as long as she could remember, she suffered from frequent sinus infections. She got surgery at age 14 but, by 16, she was back in the otolaryngologist's (ENT) office. This time, it was for something new. 

Meghan and her mom were working with an otolaryngologist (ENT) to try and figure out seemingly random symptoms, like why her hands and feet always felt like they were falling asleep. Her mom thought it could all be related, and her doctor was concerned enough to pull her out of class that day and send her to Primary Children’s Hospital for more testing.

As Meghan was filling out a form about symptoms, she told doctors she may have hearing loss in her left ear—not really bothersome, she said, but definitely noticeable. “I had just started this new job where I was cooking a lot,” she said, “and I was burning the food all the time because I wouldn’t hear the timers over the sink and the fridges.”

A hearing test revealed she had about 40 percent hearing loss in her left ear and 5 percent in her right ear. Doctors recommended she return for a follow-up the next year, but during that time, it got worse. Her local ENT ordered a CAT scan and referred her to renowned hearing specialist Dr. Richard Gurgel at University of Utah Health’s Division of Otolaryngology. He was immediately suspicious that something was wrong because Meghan was so young and had hearing loss on just one side. 

“The two ears should be balanced,” Gurgel said. “They’re the same age, and they’ve been exposed to the same amount of noise. So when one ear goes down—particularly when it’s an inner-ear or nerve-damage type of hearing loss—we’ll typically get imaging.”                                                  

He ordered an MRI which, interestingly, didn’t reveal any issues in her left ear. They did, however, find a large tumor called a vestibular schwannoma growing on the right side. These tumors usually occur later in life or are very small, so people don’t experience many symptoms.   

“We don’t know why an individual gets these,” Gurgel said. “There are no known risk factors. It happens to people who are older, younger, men, women, all different backgrounds.” 

Like most vestibular schwannomas, Meghan’s was benign, but she was much younger than a typical patient with this condition. Several treatment options exist for someone diagnosed with acoustic neuroma, so it’s important to see a specialist with a lot of experience.

“It’s not a one-size-fits-all approach for treating these patients,” Gurgel said. “We have the option to do surgery to remove the tumor, we can treat with a focused radiation, and for some patients, we just observe with yearly scans. There are also medical treatments for patients with more rare syndromes. Finding the right treatment for any patient requires a very personalized approach. 

Left untreated, Meghan’s tumor was likely to grow to the point that it could cause significant damage to her hearing and balance. It could even become deadly as it started pushing on her brain stem. Gurgel recommended that she have surgery because of her young age and large tumor size.

It felt a bit like a roller coaster to Meghan: experiencing hearing loss in the left ear, then being diagnosed with a benign tumor on the right side, and finally learning she needed brain surgery.

“That’s something you never think will ever happen to you,” she said. “Brain surgery is just a whole other level, so that was kind of scary.”  

As part of a multidisciplinary skull base surgery team, Gurgel worked with Dr. William Couldwell, Chair of the Department of Neurosurgery at University of Utah Health and an internationally recognized expert in skull base surgery who specializes in removing brain tumors.

The surgery began with an incision behind Meghan’s ear. Gurgel started the approach by first clearing a path to the tumor. Couldwell and his team removed the tumor with extremely precise microsurgery.

The biggest risk during surgery is damage to the facial nerve, which could leave Meghan with a sagging or drooping face on one side. Another common concern after surgery is balance problems. As vestibular schwannomas grow on the balance nerve, these tumors can cause dizziness and unsteadiness, although the body will often adapt. Once the tumor is removed, the brain and body have to compensate again as the balance returns to normal.

“If they had a large tumor or are unsteady, they may go to rehab for a period of time after surgery to help them with walking and gait,” Couldwell said. “We want to make sure people aren’t going to fall when they go home."

Meghan had a couple of strange setbacks during her recovery, including two trips to the ER for chemical meningitis related to withdrawal from the steroid she was taking, which means she had inflammation around the brain and all the symptoms of bacterial meningitis without actually having a true infection. She also had to get a spinal tap, which was “the literal worst pain I have ever experienced in my life,” she said.

The surgery also had other lasting impacts. Meghan is completely deaf in her right ear and still, for unknown reasons, has mild hearing loss in her left ear. So she got the BoneBridge, a cutting-edge implantable hearing device, surgically placed by Gurgel to compensate. She will have regular follow-ups throughout her life to ensure that the tumor has not returned, even though there is very little risk it will come back.

Today, Meghan is in Australia completing a business and public relations internship with a non-profit that provides primary health care to the remote island nation of Papua New Guinea.

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