Billy Joel has canceled his upcoming concerts because of a brain disorder affecting his hearing, vision and balance, the singer-songwriter announced on Friday.
The condition, called normal pressure hydrocephalus, or N.P.H., is estimated to affect hundreds of thousands of older Americans. Here’s what to know about it.
What is normal pressure hydrocephalus?
N.P.H. occurs when excess cerebrospinal fluid accumulates in the brain, causing difficulty walking, trouble controlling one’s bladder and memory problems. Those symptoms together suggest the disorder.
The bladder symptoms can include incontinence and waking up at night to urinate with increasing frequency, said Dr. Charles Matouk, a neurosurgeon at Yale University and director of the university’s Normal Pressure Hydrocephalus Program.
A statement posted to Mr. Joel’s social media accounts on Friday said his condition had been “exacerbated by recent concert performances.”
N.P.H. is rare, but risk increases with age. Dr. Matouk estimated that it might affect less than 1 percent of the population ages 65 to 80, but likely 5 percent or more of people over 80.
Experts say the condition is likely underdiagnosed because its symptoms can easily be dismissed as normal effects of aging. Dr. Matouk urged people to see a doctor if they experienced trouble walking, controlling their bladder and remembering things.
How is it diagnosed?
When a patient shows up with gait, bladder and memory problems, the first test may be a CT scan or M.R.I. In patients with N.P.H., that imaging will show enlargement of the brain’s fluid-filled ventricles.
But the conclusive test is a spinal tap: Because that procedure removes cerebrospinal fluid, patients with N.P.H. experience a temporary alleviation of symptoms, confirming the diagnosis, Dr. Matouk said.
How is it treated?
Treatment typically involves a surgery called ventricular shunting.
The disease occurs when the channel that cerebrospinal fluid normally drains through is blocked. To treat it, a surgeon drills a hole in the skull and inserts a shunt that bypasses the blocked channel and lets fluid drain into a part of the body that can absorb it, like the lining of the abdomen or the lung.
Dr. Matouk leads a group of researchers working on a less invasive procedure in which a shunt is inserted into a blood vessel and threaded up to the base of the brain, allowing fluid to drain into the bloodstream.
The researchers recently completed a small, preliminary trial that Dr. Matouk said showed the procedure to be safe. However, the results have not been published yet, and the procedure is not approved by the Food and Drug Administration. They are now enrolling patients for the next clinical trial stage.
Some patients may also opt for physical therapy to improve their gait stability, especially after surgery. Mr. Joel’s statement said he was undergoing physical therapy, “and has been advised to refrain from performing during this recovery period.”
But physical therapy alone cannot halt the progression of the disease, said Dr. David Shprecher, the movement disorders director for Banner Research. Its benefit, he said, comes in conjunction with surgery. (Mr. Joel disclosed in March that he had undergone surgery, without identifying his condition at the time.)
What’s the prognosis?
It depends on when the disease is diagnosed. If it’s caught early enough, surgery can largely or fully reverse the symptoms in many patients.
Gait and bladder issues tend to respond better to treatment than cognitive symptoms, Dr. Matouk said, but cognitive symptoms can still improve. Dr. Shprecher added that prognosis was affected by whether the patient has N.P.H. alone or also has other conditions, which can make cognitive symptoms harder to treat.
For some patients, especially in later stages, symptoms can become irreversible.
“If you get to me in a wheelchair, we can only expect the shunt to help so much,” Dr. Matouk said. “The earlier it’s diagnosed, the better patients will do.”
Still, patients can benefit significantly from surgery — even, in some cases, when the disease has progressed.
Gary Chaffee, 78, of Flower Mound, Texas, began experiencing symptoms of hydrocephalus when he was in his 50s. His job as a pharmaceutical executive required him to travel frequently, but he couldn’t navigate an airport or follow the conversation in meetings. Eventually, he went on medical leave.
Dorothy Sorlie, 83, of Eau Claire, Wis., learned she had N.P.H. in 2018, after more than two years of worsening symptoms. By the time she received a diagnosis, she was in a wheelchair and had symptoms of dementia.
But both Mr. Chaffee and Ms. Sorlie improved after receiving ventricular shunts. Though Mr. Chaffee was not able to return to his job, he volunteers, spends time with friends and walks daily. Ms. Sorlie said she was doing “fine” aside from age-related arthritis.
Amanda Garzón, chief operations officer for the Hydrocephalus Association, said she hoped Mr. Joel’s decision to speak publicly would bring more attention both to N.P.H. and to other forms of hydrocephalus, including forms that are congenital or caused by traumatic brain injuries.
More attention could lead to more funding for new treatments or even preventive measures.
“Billy Joel could really change the entire trajectory of the condition,” Ms. Garzón said.
