His persistent 18-month search led to a third ear, nose and throat specialist who discovered the curable reason.
So in May 2018, Schultz consulted the same ear, nose and throat specialist in suburban Philadelphia that her mother had seen years earlier when she developed a hearing problem in her late 60s.
The otolaryngologist ordered an audiology test that found 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 earlier was the likely cause of her permanent hearing loss. The only treatment, he advised, was hearing aids.
“I was pretty upset,” Schultz recalled. Why, he wondered, would someone who had attended only a handful of rock concerts and otherwise had little exposure to loud noise need hearing aids at such a young age? The doctor did not seem interested in exploring this question.
But Schultz was. Her persistence prompted an 18-month search involving an allergist, an endocrinologist, and two additional ENTs, the latter of whom discovered the underlying, treatable reason for her problem. It was a finding that would greatly improve Schultz’s quality of life and have implications for his family as well as a co-worker.
“When a diagnosis doesn’t fit,” he said, “it’s important to get a second opinion, and maybe more.”
Based on evidence that found Schultz unable to hear high-pitched sounds, the first ENT concluded he 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 usually affects the ability to hear high frequencies, such as women’s voices. The most common cause is aging, although it can also be caused by loud music or a blow to the head.
The other type of hearing loss—conductive—usually affects the middle ear, which transfers sounds to the inner ear. Conductive hearing loss can be caused by a perforated eardrum, fluid in the ear, impacted earwax, 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 Schultz, she could be fitted with hearing aids that could improve, but not restore, her hearing.
“I didn’t have that kind of money,” Schultz said. The devices cost about $3,000 and were not covered by his insurance. He decided to do it and hope his hearing wouldn’t get any worse.
But a year later, yes. Not only were the sounds muffled, but his ears felt perpetually clogged, as if he had a bad cold. To make matters worse, Schultz had recently started a new job in an open-plan office, where his co-workers spoke in soft voices to avoid disturbing others.
In July 2019, Schultz consulted a second ENT affiliated with a different health system. He explained the results of the audiology tests and asked if the ear congestion could be related to the hearing impairment.
The second specialist diagnosed a postnasal drip and told Schultz that her Eustachian tubes, which connect the nose and middle ear, were blocked. The doctor suspected an allergy might be the culprit.
He prescribed 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 did 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 causes nasal inflammation. Environmental triggers can include stress, temperature changes, spicy food, paint fumes, perfumes, or certain medications.
– Marlene Schultz
Another possible cause was a bacterial infection. The allergist prescribed an antibiotic and recommended that Schultz continue using the nasal spray.
In an attempt to clear his blocked Eustachian tubes and regain some of his hearing, Schultz had devised his own remedy. Once an hour he put a finger in each ear to relieve the pressure. It worked, but only briefly.
“I was freaking out,” she recalled, and made 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 that was found to be benign.
Three weeks later, Schultz underwent an MRI of his brain, which doctors hoped might shed light on the cause of the ear fullness and possibly his hearing loss. It revealed nothing abnormal.
After more than a year of research, his hearing got worse and Schultz didn’t get any further than when he started.
“I wasn’t sure what to do or where to go,” she recalled.
At the suggestion of a relative, Schultz contacted one of his cousins, an ENT in Boston.
He advised her to see a hearing specialist at one of the large teaching hospitals in Philadelphia. Schultz scoured Penn Medicine’s website, examining the descriptions of several otolaryngologists, and scheduled an appointment with a specialist whose expertise seemed promising.
Four weeks later, in December 2019, he met with Douglas Bigelow, a head and neck surgeon who heads the division of otology and neurotology.
Bigelow ordered a new round of hearing tests that differed markedly from the original audiology results. This time, Schultz’s hearing loss was classified as conductive, not sensorineural. This meant that depending on the cause, your problem could be fixed.
Her age, symptoms and test results, Bigelow told her, pointed to a condition called otosclerosis, the most common cause of middle-ear hearing loss in young and middle-aged adults.
Otosclerosis affects about 3 million Americans, mostly middle-aged white women. Many cases are believed to be inherited. Hearing loss is the result of abnormal bone growth in the middle ear that affects the stylus, the smallest bone in the body, located behind the eardrum. The stylet freezes in place and cannot vibrate, thus impairing sound’s ability to pass to the inner ear.
“I was so relieved to know what I had and I was excited that there was a way to fix it.”
– Marlene Schultz
Gradual hearing loss, usually starting in one ear, is usually the first symptom. Many people initially cannot hear deep sounds or a whisper. Some experience dizziness, balance problems or tinnitus.
A patient with a normal eardrum and the inability to hear low tones “is kind of 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 correct diagnosis,” he said of the diagnosis. “He had other symptoms such as congestion and a feeling of fullness in the ears that might have led people in other directions.”
Otosclerosis can be treated with hearing aids, but stapedectomy surgery may offer better results.
The operation involves inserting a prosthetic device into the middle ear to replace the stylet, restoring hearing. Some hearing loss may persist after surgery. And sometimes people who undergo the operation end up with worse hearing.
Schultz, who had never heard of otosclerosis, said she was thrilled to be able to fix the problem that was “driving me crazy.”
“I was so relieved to know what I had and I was excited that there was a way to fix it,” she said. Subsequent CT scans confirmed that he 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 his right was done a year later.
The hardest months, Schultz said, were those leading up to the first surgery. In the early days of the pandemic while working from home, Schultz spent hours in dreaded Zoom meetings, struggling to hear what others were saying. He often didn’t know when it was his turn to speak.
Schultz has regained about 90 percent of his hearing in both ears. The congestion and feeling of obstruction are gone. The tinnitus remains but is mild.
His diagnosis had other ramifications.
Her mother, who had been told years earlier that her hearing loss was age-related, was found to have otosclerosis but chose not to have surgery. And as a result of Schultz’s experience, one of his co-workers was diagnosed with otosclerosis and underwent successful surgery.
“I can hear most sounds now and it’s wonderful,” Schultz said. “I remember sitting in my kitchen and hearing a low whine and realizing it was coming from the fridge and I hadn’t heard that in years. I thought, ‘This is great!'”
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