Jamie's story

     Acoustic neuroma

 

As a physical therapist, Jamie is attentive to the ebbs and flows of human health. Body parts work, they stop working, they recover. Diseases also can intervene, throwing a permanent wrench into one's otherwise high-functioning biological machine.

A few years ago, Jamie began to sense that she wasn't hearing as well as she used to. Her left ear seemed to be failing her at times. She mentioned it to her primary care physician, who looked for signs of infection but saw nothing amiss.

In the fall of 2017, with her hearing getting worse, Jamie's intuition kicked in. "I thought I should probably have it checked out," she recalls.

Jamie scheduled an appointment with an audiologist, who sounded the alarm. Jamie had significant hearing loss in only one ear, a red flag that something serious was going on. A week later Jamie was in the office of Tasneem Shikary, MD, an ear, nose and throat specialist with TriHealth. Dr. Shikary listened to Jamie's medical history, ordered an MRI, and prepared Jamie for the possibility that she might have a benign brain tumor called an acoustic neuroma. Formally known as a vestibular schwannoma, an acoustic neuroma grows from the nerves responsible for balance and hearing.

"I saw Dr. Shikary on Sept. 28 and had the MRI four days later on Oct. 2, first thing in the morning," Jamie says. "By 11 a.m. I got a call from her that I did have a vestibular schwannoma that was nearly 3 centimeters in size."

Jamie was referred that very day to Vincent DiNapoli, MD, PhD, a neurosurgeon and brain tumor specialist with Mayfield Brain & Spine, and Joseph Breen, MD, an ENT surgeon with UC Health. Jamie saw Dr. DiNapoli four days later. "He confirmed what Dr. Shikary had told me, but it felt very different going in to see a surgeon knowing this diagnosis," Jamie says. "I had a long list of questions, and Dr. DiNapoli was very patient and informative. He spent nearly 45 minutes with me and my mom, first getting my history and asking about hearing tests I had had. He asked about my symptoms, did a neurological screen, and looked at my balance."

Because her tumor was larger than 2 centimeters, Dr. DiNapoli told her, it needed to be surgically removed. The surgery would be extensive because the tumor was shoulder-to-shoulder with two important cranial nerves: the vestibulocochlear nerve, which is responsible for sending hearing and balance signals to the brain, and the facial nerve, which is responsible for moving muscles in the face. Dr. DiNapoli also told Jamie that because of the tumor's size, there was less than a 2 to 5 percent chance of saving her hearing. "We don't promise anything regarding hearing preservation when the tumor is that large," Dr. DiNapoli explains.

Nevertheless, Dr. DiNapoli was reassuring. He would operate in tandem with Dr. Breen, another skilled surgeon who was fellowship-trained in otology.

"He went through all the risks and benefits," Jamie says. "He was very comforting and compassionate about meeting a mother who works and does things in the community and has a young one at home. This was something that would have a huge impact on my life."

A retrosigmoid surgery

Jamie underwent surgery at Good Samaritan Hospital. The procedure lasted 11 hours.

"The surgery overall went very well," Dr. DiNapoli says. "It took a long time because of the size of the tumor and the way that it was stuck to her facial nerve. When a tumor gets to be that size, the facial nerve becomes almost like a translucent, thin rim of paper around the outside edge of the capsule of the tumor, so we have to find that nerve using a stimulator and spend a lot of time carefully pealing the tumor off of that nerve, which is less than a millimeter thick in some places. You have to be careful not to damage the facial nerve; you want to make sure the face is still functional."

All told, Dr. DiNapoli was able to remove more than 98 percent of the tumor. "There remains a small piece of the capsule that is stuck to her facial nerve," he says. "It is essentially an enhancement, or scar, along the nerve. We're watching to make sure it doesn't change over time, but it's unlikely to."

Says Jamie: "They told me ahead of time that if part of sheath remained on the facial nerve, they would leave it rather than risk damaging the facial nerve."

Jamie spent one night in the intensive care unit, was up and walking the next day, and was discharged after spending three nights in the hospital. "I didn't have many side effects from the surgery other than headaches, which I was told to expect," Jamie says. "I had a little dizziness the first few days, which is normal because of the anesthesia, and some minor facial swelling."

Those symptoms resolved within a few weeks, and Jamie returned to work 5 weeks after her surgery. As was expected, however, she now has nearly complete hearing loss on her left side.

"Because the tumor arises from, and is adhered to, the vestibular nerve, the chances of getting it away from the nerve and saving the hearing are very rare," Dr. DiNapoli says. "I think this has something to do with the vascular supply of the nerve. Because even if you preserve the nerve anatomically, which we did in Jamie's case, the nerve still doesn't work. We think that's because it was so stretched and splayed and attached to the tumor that you're basically defunctionalizing it when you remove the tumor."

The inability to hear "in stereo," with sound being processed through both ears, is challenging, she says, but she is coping well with the change. "It's most difficult when I'm in crowded areas, where there's a lot of noise, and not being able to locate where sound is coming from," she says. "You spend so much of your energy concentrating on what you should be hearing. At the same time, I feel very fortunate. As a healthcare provider, we teach our patients how to accommodate change, and that was beneficial to me. Being a fairly young, active individual who never sat still before surgery, I had every desire to get back to functioning and not let this slow me down."

Looking ahead, Jamie will have six-month follow-up MRI's. If the residual tumor hasn't changed 18 months following surgery, she will undergo follow-up MRI's only as needed.

Jamie's advice to others who are diagnosed with an acoustic neuroma is not to be afraid to ask questions. "Make sure your take the time to know your surgeon and are comfortable with that relationship and the skill set of your surgeon. This is just a bump in the road! If you can be comfortable and confident in your surgeon's abilities and come to terms that it's not going to be a lifelong, debilitating disease, you can be in control of what you want your future to hold."