“You can’t see the ceiling, can you?” the man asked his 31-year-old wife. She grimaced, then shook her head. She was lying in bed looking toward the familiar shadows and shapes cast by the wintry morning sun. But she couldn’t see them. It was as if a dense white fog lay between her and those daily shifting patterns. Squinting didn’t help. Opening her eyes as wide as she could didn’t, either. All her life she had perfect vision. It was a secret source of pride. She’d never even seen an eye doctor. But that morning changed everything.
She first noticed the trouble in her eyes six months earlier. She is a professional violinist and a teacher and that summer took her students to Italy to experience the sacred music and art. As she gazed up at the frescos decorating the ceiling of a favorite cathedral, a shimmering shape with jagged, irregular edges appeared out of nowhere. The points seemed to twinkle as the starlike image slowly enlarged. Inside the glittering outline, the colors were jumbled, like the crystals in a kaleidoscope. It was beautiful and terrifying. She dropped her head, closed her eyes and rubbed her aching neck.
When she opened her eyes, the star burst, with its glimmering edges, was still there, distorting all that lay beyond it. It grew so large that it was almost all she could see. Then slowly it began to fade; after nearly a half-hour, the world started to resume its familiar look and shape. There had been similar, if less severe, experiences: Every now and then, when she would get up quickly after sitting or lying down, she would feel an intense pressure inside her head, and when it released, everything briefly looked faded and pale before returning to normal hues. These spells only lasted a few seconds and happened only a handful of times over the past few years. She wrote it off to fatigue or stress. After that day in Italy, those glistening star bursts appeared weekly, then daily.
Stranger still, straight lines developed weird lumps and bumps when she looked at them out of the corner of her eye. Doorways, curbs and table edges seemed to waver, growing bulges and divots. When she looked at the object full on, it would obediently straighten out but resumed its aberration once it was on the sidelines again.
Days after her morning whiteout, the young woman went to an optometrist in nearby Fort Lee, N.J., Dr. Paul Shahinian. If the star bursts were worrisome to the young woman, Shahinian’s reaction to her exam was terrifying. She needed to see a neuro-ophthalmologist, he told her — a specialist in eyes and brains — and she needed to see one soon. All the information collected by sight is transmitted to the brain through a thick cable of fibers at the back of the eye called the optic nerve, the doctor explained, and the nerve in her left eye was hugely swollen. As she sat in his office, Shahinian called the specialists himself. The first two offices he called had the same answer: She couldn’t get an appointment for months. Then he reached out to a neuro-ophthalmologist who was new to the area, Dr. Kaushal Kulkarni, and, after explaining the urgency, arranged for the patient to be seen the following week.
Kulkarni listened to the patient describe her strange visual abnormalities. Although her vision was still 20/20, the intermittent star bursts and the bent lines seen in her peripheral vision — a phenomenon known as metamorphopsia — suggested something was wrong with the way the brain was getting and processing her visual information. Kulkarni shined a narrow bright light into the young woman’s right eye. As expected, both pupils constricted. He moved the light over to the left, and both pupils immediately dilated. Moving it to the right, her pupils again constricted; returning to the left, again they suddenly widened. Clearly the signal on the left wasn’t getting through. The swelling was cutting off the flow of information from the eye to the brain. It seemed to be a one-way problem, however: The fact that the left pupil constricted when the light was shined in the right eye indicated that information from the brain was still getting through.
There are many causes of this kind of optic-nerve injury. Shahinian had thought that, given the patient’s age, this was likely to be multiple sclerosis, an autoimmune disorder in which the immune system mistakenly attacks nerve fibers that link the brain to the body. Kulkarni agreed that was a possibility and ordered an M.R.I. to look for evidence of that or other abnormalities. Was this a tumor? Or a stroke? Other autoimmune diseases also had to be considered. It could also be the result of an infection: Lyme disease could do this; so could cat-scratch fever, an infection caused by the bacterium Bartonella henselae; even syphilis, often called the great imitator because of its ability to manifest in so many ways, could cause this kind of injury.
He sent the patient to the lab for testing. The results of the blood tests came in quickly. It wasn’t Lyme or Bartonella or syphilis. None of the inflammatory markers suggestive of an autoimmune disease were elevated. It was the M.R.I. that held the answer. Kulkarni didn’t see the bright white smattering of dots and dashes that would suggest M.S. Instead, a large round object, a mass about the size of a plum, dominated the middle portion of the left side of her brain.
Kulkarni called the patient and told her that the M.R.I. was abnormal. She came back a couple of days later. He couldn’t tell her based on the pictures just what kind of tumor she had. The most common would be a meningioma, a tumor of the tissue that lines the brain. An acoustic neuroma was rarer but also possible. This is a slow-growing tumor originating in the tissue that connects the ear to the brain. She was a little young for that; these tumors usually appear in men and women over 40, and they usually cause problems in hearing and balance rather than in vision. Whatever it was, the tumor was so large that it blocked the circulation of the spinal fluid through the brain, causing the nerve to swell. It would have to be removed.
Kulkarni knew the patient didn’t have health insurance. He called around to the neurosurgeons he knew, trying to figure out how to get this woman the care she needed. The only answer seemed to be for her to get in through the emergency department. Thanks to a law called the Emergency Medical Treatment and Labor Act, all emergency departments are required to provide stabilizing treatment to any patient who comes in, regardless of insurance status or ability to pay. The care provided isn’t free; patients are billed, but they have to be treated.
She went to the emergency department at NewYork-Presbyterian Hospital/Columbia University Medical Center, and the mass was removed. The pathologist confirmed that the tumor was an acoustic neuroma. Reading up on this type of growth, the patient noted that hearing loss is a common symptom. She had hearing loss in her left ear, but she hadn’t made a connection between that problem and the one with her eyes. She thought that the constant exposure to the sound from her violin might have caused a little damage. She figured it was just the price of doing what she loved. After the surgery, her hearing didn’t change, but the strange star bursts completely disappeared. Straight lines still have the tendency to buckle in her peripheral vision, though.
Before her hair had even grown enough to hide the scar, the bills began to roll in. The numbers were even more frightening than the images of the tumor. The ultimate accounting totaled around $650,000. She and her husband worried that they would have to declare bankruptcy. They had some money — they were saving for a rainy day, but this was a tsunami. Salvation came, unexpectedly, when a friend of a friend asked if they had talked to the hospital about financial help. It turned out that Columbia Presbyterian did have a financial-aid program. In fact, all nonprofit hospitals are required to provide financial assistance to people who need it; it’s mandated by the Affordable Care Act. They didn’t have to bankrupt themselves. They are grateful that the ordeal ended well. And, two years after the surgery, they are beginning to rebuild their savings — because you never know when it might rain again.
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share, write her at Lisa.Sandersmdnyt@gmail.com.