Her persistent 18-month search led to a third ear, nose and throat doctor who discovered the curable cause
March 18, 2023 at 9:00 am EDT
(Cam Cottrill for the Washington Post) Comment on this story
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At 47, Marlene Schultz thought she was far too young to keep saying “What?” when she couldn’t hear what people were saying to her.
The Pennsylvania accountant found it increasingly difficult to understand the voices of her teenage sons, much to her irritation. At work, Schultz often had to ask people to repeat themselves, which she found embarrassing. And she began cranking up the volume on her TV, an adjustment she hadn’t felt necessary in years.
So in May 2018, Schultz consulted the same ear, nose and throat specialist in suburban Philadelphia that her mother had visited years earlier when she developed a hearing problem in her late 60s.
The otolaryngologist ordered audiological tests that revealed low-frequency hearing loss in both ears. When Schultz told the doctor that she had also developed ringing in her ears, a condition known as tinnitus, he told her that listening to loud music years ago was the likely cause of her permanent hearing loss. The only treatment, he advised, would be hearing aids.
“I was pretty upset,” Schultz recalled. Why, she wondered, would someone who’d only attended a handful of rock concerts and otherwise rarely been exposed to loud noise need hearing aids at such a young age? The doctor didn’t seem interested in pursuing this question.
But Schultz was. Her persistence led to an 18-month search involving an allergist, an endocrinologist and two other ENT doctors, the second of whom discovered the underlying and treatable cause of her problem. It was a finding that would greatly improve Schultz’s quality of life and have repercussions for her family as well as a colleague.
“When a diagnosis is wrong,” she said, “it’s important to get a second opinion — and maybe more.”
Based on tests that revealed that Schultz could not hear low-pitched sounds, the first ENT concluded that she had mild sensorineural hearing loss in both ears. Sensorineural hearing loss is common and is caused by damage to the inner ear, which allows the brain to hear and understand sounds. It typically impairs the ability to hear high frequencies, such as B. Female voices. The most common cause is old age, although loud music or a blow to the head can also cause it.
The other type of hearing loss – conductive hearing – typically affects the middle ear, which transmits sound to the inner ear. Conductive hearing loss can be caused by a perforated eardrum, fluid in the ear, trapped earwax, an infection, or a benign tumor. Depending on the cause, it may be curable. Some people have a mix of conductive and sensorineural hearing loss.
When she was ready, the ENT advised her, Schultz advised, that she could be fitted with hearing aids, which could improve but not restore her hearing.
“I didn’t have that much money,” Schultz said. The devices cost about $3,000 and were not covered by their insurance. She decided to muddle through and hope her hearing didn’t deteriorate.
But a year later the time had come. Not only were the sounds more muffled, her ears felt constantly blocked, like she had a bad cold. To make matters worse, Schultz had recently started a new job in an open plan office where her colleagues kept their voices low so as not to disturb others.
In July 2019, Schultz consulted a second ENT affiliated with a different healthcare system. She told him about the results of her audiology tests and asked if her ear congestion could be related to her deteriorating hearing.
The second specialist diagnosed postnasal drip and told Schultz that her Eustachian tubes, which connect her nose and middle ear, were blocked. The doctor suspected that an allergy could be to blame.
He prescribed her a steroid nasal spray to clear her ears, which could improve her hearing, and recommended that Schultz see an allergist if her condition didn’t improve.
A month later, she saw an allergist who performed skin tests for common allergens: trees, pollen, dust mites, mold, and animals. Every test was negative. The allergist concluded that Schultz had vasomotor rhinitis — a common condition of unknown cause that leads to nasal inflammation. Environmental triggers can include stress, temperature changes, spicy food, paint fumes, perfume, or certain medications.
“I was desperate.”
– Marlene Schultz
Another possible cause was bacterial infection. The allergist prescribed an antibiotic and recommended that Schultz continue using the nasal spray.
In an attempt to clear her blocked Eustachian tubes and regain some of her failing hearing, Schultz had developed her own remedy. Once an hour she put a finger in each ear to relieve the pressure. It worked, but only briefly.
“I was desperate,” she recalled, making an appointment with an endocrinologist to see if he had any ideas. He recommended two over-the-counter medications, but focused on her enlarged thyroid. In late October, he performed a needle biopsy on a peanut-sized nodule, which turned out to be benign.
Three weeks later, Schultz underwent an MRI scan of her brain, which doctors hoped could shed light on the cause of her ear fullness and possibly her hearing loss. It showed nothing unusual.
After more than a year of searching, her hearing was getting worse and Schultz was no further than when she started.
“I wasn’t sure what to do or where to go,” she recalls.
At the suggestion of a relative, Schultz contacted one of her cousins, an ENT in Boston.
He advised her to see a hearing specialist at one of Philadelphia’s major teaching hospitals. Schultz went through Penn Medicine’s website, reviewed descriptions from various ENT doctors, and made an appointment with a specialist whose expertise sounded promising.
Four weeks later, in December 2019, she met with Douglas Bigelow, a head and neck surgeon who heads the Department of Otology and Neurotology.
Bigelow ordered a new round of hearing tests that differed markedly from the original audiological results. This time, Schultz’s hearing loss was classified as a sound conduction, not a sound sensation. This meant that depending on the cause, their problem could potentially be fixed.
Her age, symptoms and test results, Bigelow said, pointed to a condition called otosclerosis, the leading cause of middle ear hearing loss in young and middle-aged adults.
Otosclerosis affects approximately 3 million Americans, mostly white, middle-aged women. Many cases are considered inherited. Hearing loss results from abnormal bone growth in the middle ear that affects the stapes, the smallest bone in the body located behind the eardrum. The stirrup becomes frozen in place and unable to vibrate, reducing the ability of sound to travel into the inner ear.
“I was so relieved to know what I had and excited that there was a way to fix it.”
– Marlene Schultz
Gradual hearing loss, typically starting in one ear, is usually the first symptom. Many people are initially unable to hear low-pitched sounds or a whisper. Some experience dizziness, balance problems, or tinnitus.
A patient with a normal eardrum and an inability to hear low-pitched sounds “is kind of a classic for otosclerosis,” Bigelow said, adding that “her hearing loss was clearly conductive when I saw her.” The initial finding of sensorineural hearing loss , which is not surgically treatable, “could be due to technical issues with the audiologist,” he noted.
“Most of the time, a good ENT will make the right diagnosis,” he said of the diagnosis. “She had other symptoms, including congestion and fullness in her ears, which may have led people in other directions.”
Otosclerosis can be treated with hearing aids, but stapedectomy surgery may provide better results.
The surgery involves placing a prosthesis in the middle ear to replace the stirrup and restore hearing. Some hearing loss may remain after surgery. And sometimes people who have the surgery have worse hearing.
Schultz, who had never heard of otosclerosis, said she’s excited that she might be able to solve the problem that’s “driving me crazy.”
“I was so relieved to know what I had and excited that there was a way to fix it,” she said. Subsequent CT scans confirmed that she had otosclerosis in both ears.
Bigelow, who estimated he has performed about 1,000 stapedectomies in his 30-year career, operated on Schultz’s left ear in June 2020. The operation on her right was performed a year later.
According to Schultz, the most difficult months were those leading up to the first operation. In the early days of the pandemic, while working from home, Schultz spent hours in Zoom meetings she dreaded, struggling to hear what others were saying. She often didn’t know when it was her turn to speak.
Schultz has since regained about 90 percent of her hearing in both ears. The feeling of congestion and constipation has disappeared. The tinnitus remains, but is mild.
Her diagnosis had other consequences.
Her mother, who had been told years earlier that her hearing loss was age-related, was diagnosed with otosclerosis but decided against surgery. And based on Schultz’s experience, one of her work colleagues was diagnosed with otosclerosis and successfully operated on.
“I hear most of the noise now and it’s wonderful,” Schultz said. “I remember sitting in my kitchen and hearing a low groan and realizing it was coming from the fridge and I hadn’t heard it in years. I thought, ‘This is great!’”
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