Health mystery: What was wrong with the professor's voice?

Catherine Cutter's voice was her livelihood. A professor of food science at Penn State University, the microbiologist routinely lectured to large classes about food safety in the meat and poultry industries. But in 2008, after Cutter's strong alto voice deteriorated into a raspy whisper, she feared her academic career might be over.

How could she teach if her students could barely hear her?

The classroom wasn't the only area of Cutter's life affected by her voicelessness. The mother of two teenagers, Cutter, now 52, recalls that she "couldn't yell — or even talk" to her kids and would have to knock on a wall or countertop to get their attention. Social situations became increasingly difficult as well, and going to a restaurant was a chore. Using the drive-through at her bank or dry cleaner was out of the question because she couldn't be heard.

"I just retreated," said Cutter, who sought assistance from nearly two dozen specialists for her baffling condition. The remedies doctors prescribed — when they worked at all — resulted in improvement that was temporary at best.

For two years Cutter searched in vain for help. It arrived in the form of a neurosurgeon she consulted for a second opinion about potentially risky surgery to correct a different condition. He suggested a disorder that had never been mentioned, a diagnosis that proved to be correct — and correctable.

Until then, "everyone had been looking in the wrong place," Cutter said.

— — —

The problem began with a lump in her throat. On vacation with her husband and children in Myrtle Beach, S.C., in June 2008, Cutter awoke one morning feeling that something was stuck in her throat. Her family had eaten Mexican food the previous night. "I thought maybe I'd swallowed a chip wrong," Cutter recalled.The vacation had been uneventful, filled with the water sports her family relished. A day earlier, Cutter, who was bodysurfing, had been slammed by a wave onto the ocean floor with such force that she saw stars. She knew she had wrenched her neck and "got up and out of the water and just decided to sit it out" for the rest of the day.

Back home in State College, Pa., the feeling that something was lodged in her throat persisted. Cutter consulted her internist, who suspected she had developed acid reflux and prescribed medicine.

After several weeks, her voice became increasingly raspy, and Cutter consulted a gastroenterologist and an ear, nose and throat specialist. Although an upper endoscopy to inspect her throat and upper gastrointestinal tract found no evidence of reflux, both specialists said they suspected it and prescribed stronger acid-blocking drugs. When Cutter protested that she didn't have heartburn, the doctors told her that reflux can cause a variety of symptoms, including hoarseness.

Two months later, no better, Cutter began feeling a spasming sensation when she spoke. She had also noticed something odd: Her voice was normal for the first hour after she awoke and if she sat down or propped her feet up, it got stronger.

She consulted a neurosurgeon. He ordered CT and MRI scans of her brain and spine, which found nothing amiss and seemed to rule out injury from the surfing incident; he was at a loss to explain her vocal problem.

Frustrated, Cutter decided to expand her search. She consulted a gastroenterologist in Hershey, a two-hour drive from her home, and an orthopedic surgeon in Philadelphia, four hours away. Neither had any new ideas.

Unable to teach standing up because her voice was inaudible and unpredictable, Cutter devised several strategies. She taught sitting down, with the aid of a microphone, and began to use online materials more frequently. Increasingly she worried about her ability to continue teaching, having spent years working hard to build an academic career.

In November she went back to Philadelphia to consult a third gastroenterologist, this one at the Hospital of the University of Pennsylvania. He definitively ruled out acid reflux through further testing and referred her to his colleague Kevin Leahy, a Penn otolaryngologist who specializes in voice disorders.

Based on her history and tests, Leahy suspected she might have abductor spasmodic dysphonia. A neurological disorder of unknown origin, it results in a strained, hoarse voice. (radio talk-show host Diane Rehm has said the malady nearly ended her career.) Leahy said that the quality of Cutter's voice, her age, sex and other factors suggested it.

"It wasn't a completely solid" diagnosis, Leahy recalled recently. "One of the weird things is that if she raised her feet above her chest, her voice got better," which would not be typical of spasmodic dysphonia.

At Leahy's suggestion, Cutter began working with a speech pathologist near her home in an effort to strengthen her vocal cords. She returned to Penn in February 2009 for the first in a series of Botox injections. The paralytic agent, which is used in cosmetic procedures to smooth wrinkles, is also approved to treat spasmodic dysphonia; given at three-month intervals, the drug works by preventing vocal muscles from spasming.

The first injections worked well, strengthening Cutter's voice enough so that she could give a speech in Portugal in May.

But by October, her overall health had deteriorated. The effects of Botox wore off in a matter of weeks, not months, and Cutter developed dull pains in her shoulders and neck that radiated down her arms. She began getting migraine headaches and felt an intermittent but intense pain — worse than an "ice cream headache" — while swallowing.

"It was scary," she recalled. Her time and energy were consumed by medical appointments, including eight-hour round-trip drives to Philadelphia. Doctors had ruled out a host of disorders including thyroid cancer, a brain tumor and myasthenia gravis, a neuromuscular disorder, but still they couldn't explain what might be wrong.

Believing that her arm and neck pain might be orthopedic, she underwent more tests. An orthopedist spotted herniated disks in her spine. That might explain her neck and shoulder pain, but not her voice problem. Cutter tried to relieve the upper-body pain with acupuncture and then steroid injections, neither of which helped.

In April 2010, a neurosurgeon in State College ordered another MRI of Cutter's brain. This time the test revealed a swollen artery that appeared to be pressing on a nerve.

The neurosurgeon suspected she had glossopharyngeal neuralgia, a condition caused by irritation of the ninth cranial nerve, which carries sensations from the throat to the brain. He recommended a microvascular decompression, brain surgery designed to relieve the head pain, and referred her to the Cleveland Clinic.

Cutter, who did not want to travel to Cleveland, asked Leahy for a referral to a neurosurgeon. Leahy was concerned about the prospect of such surgery; the operation, he said, can be risky and leave patients worse off. He sent Cutter to Penn neurosurgeon John Lee for a second opinion.

During the June 2010 appointment, Lee made a startling suggestion. Based on the neck pain after the bodysurfing accident, her symptoms, including the lump in her throat, and the improvement in her voice when she reclined, he told Cutter he suspected she had a rare condition called Eagle syndrome.

Eagle syndrome occurs when a piece of bone called a styloid process, which extends from the skull into the ear, presses on or irritates adjacent structures, including the glossopharyngeal nerve. About 4 percent of the population has an elongated styloid process — considered to be longer than about an inch — but only 4 percent of them develop a problem as a result. Pain can arise after trauma or a tonsillectomy, both of which can stimulate bone growth. In Cutter's case, the neck injury from surfing appears to have been the trigger.

"Her voice problems were a bit of a curveball" because they are not commonly seen in Eagle syndrome patients and were suggestive of spasmodic dysphonia, Leahy said.

To confirm the diagnosis, Cutter underwent yet another CT scan, this one in a different location than previous scans: It focused on the base of her skull. That scan revealed a styloid process that was just over an inch long. Other tests confirmed the diagnosis and suggested that she was a candidate for styloid surgery.

"I was absolutely elated," Cutter recalled. Finally she had an answer, as well as a diagnosis that explained all her symptoms.

Cutter underwent surgery at Penn in August, performed by Leahy and Jason Newman, an associate professor of otolaryngology. The hour-long operation, using a surgical robot, involved clipping the excess bone and removing Cutter's right tonsil; it was the subject of a case report in the August 2011 issue of the journal Otolaryngology Head and Neck Surgery.

"It is a very painful surgery," Leahy said. Cutter concurs. She lost 20 pounds and spent weeks in physical therapy after her jaw locked because it had been propped open during surgery.

But the pain, she said, was worth it. Her voice returned to normal and her other symptoms vanished. "I am incredibly indebted to them," Cutter said of Lee, Leahy and Newman.

After she recovered, Cutter said she sent a copy of the case report to many of the doctors who had treated her. "I was so mad at the ones who pooh-poohed it," she said, "and I wanted all of them to know that this is what it was."


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9 min read
Published 25 February 2015 5:00am
Updated 8 January 2016 10:17pm
By Sandra G. Boodman
Source: The Washington Post


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