She disagreed with her early hearing loss diagnosis. She was right.

Her persistent 18-month search led to a third ear, nose and throat specialist who discovered the curable cause

(Cam Cottrill for The Washington Post)


At 47, Marlene Schultz thought she was far too young to keep saying “What?” when she couldn’t hear what people were telling her.

The Pennsylvania accountant found it increasingly difficult to distinguish the voices of his teenage sons, much to his annoyance. At work, Schultz often had to ask people to repeat themselves, which embarrassed her. And she began turning up the volume on her television, an accommodation she hadn’t thought would be necessary for years.

So in May 2018, Schultz consulted the same Philadelphia ear, nose and throat specialist her mother had seen years earlier when she developed a hearing problem in her late 60s.

The otolaryngologist ordered audiological testing, which 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, she wondered, would someone who had attended only a handful of rock concerts and was otherwise only exposed to loud noise need hearing aids at such a young age? The doctor didn’t seem interested in investigating that question.

But Schultz was. Her persistence fueled an 18-month search involving an allergist, an endocrinologist, and two additional ENTs, the second of whom discovered the underlying and treatable cause of her problem. It was a finding that would greatly improve the quality of Schultz’s life and have ramifications for her family as well as a colleague.

“When a diagnosis doesn’t fit,” she said, “it’s important to get a second opinion—and maybe more.”

Based on tests that showed Schultz was unable to hear low 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 enables the brain to hear and understand sounds. It typically affects the ability to hear high frequencies, such as women’s voices. The most common cause is aging, although loud music or a blow to the head can also cause it.

The second type of hearing loss – conductive – typically affects the middle ear, which transmits sounds to the inner ear. Conductive hearing loss can be caused by a perforated eardrum, fluid in the ear, affected earwax, infection or a benign tumor. Depending on the cause, it can be cured. Some people have a mix of conductive and sensorineural hearing loss.

When she was ready, ENT Schultz advised, 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 her insurance. She decided to mess around and hope her hearing didn’t get any worse.

But a year later it did. Not only were the sounds muffled, her ears felt perpetually blocked, as if she had a bad cold. To make matters worse, Schultz had recently started a new job in an open office environment where her colleagues spoke in soft voices to avoid disturbing others.

In July 2019, Schultz consulted another ENT associated with another health system. She told him about the results of her audiology test and asked if her ear blockage could be related to her deteriorating hearing.

The other specialist diagnosed postnasal drip and told Schultz that her Eustachian tube, which connects the nose and middle ear, was blocked. The doctor suspected that an allergy might be to blame.

He prescribed a steroid nasal spray to unclog her ears, which could improve her hearing, and recommended that Schultz consult an allergist if her condition did not 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 results in nasal inflammation. Environmental triggers may include stress, temperature changes, spicy food, paint fumes, perfume, or certain medications.

“I was getting desperate.”

— Marlene Schultz

Another possible cause was a bacterial infection. The allergist prescribed an antibiotic and advised Schultz to continue using the nasal spray.

In an attempt to clear her blocked Eustachian tube and regain some of her fluttering hearing, Schultz had devised his own remedy. Once an hour she stuck a finger in each ear to relieve the pressure. It worked, but only briefly.

“I got desperate,” she recalled, and made an appointment with an endocrinologist to see if he had any ideas. He recommended two over-the-counter medications, but addressed her enlarged thyroid gland. In late October, he performed a needle biopsy on a nodule the size of a peanut, which turned out to be benign.

Three weeks later, Schultz underwent an MRI scan of her brain, which doctors hoped would shed light on the cause of her ear fullness and possibly her hearing loss. It revealed nothing abnormal.

After more than a year of searching, her hearing was failing and Schultz was no further along than when she 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 an audiologist at one of Philadelphia’s major teaching hospitals. Schultz reviewed Penn Medicine’s website, reviewed the descriptions of various otolaryngologists, and scheduled 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 significantly from the original audiology results. This time, Schultz’s hearing loss was classified as conductive, not sensorineural. This meant that depending on the cause, her problem could possibly be solved.

Her age, symptoms and test results, Bigelow told her, pointed to a condition called otosclerosis, the most common cause of hearing loss in the middle ear in young and middle-aged adults.

Otosclerosis affects about 3 million Americans, mostly middle-aged white women. Many cases are believed to be hereditary. Hearing loss is caused by abnormal bone growth in the middle ear that affects the stapes, the smallest bone in the body, located behind the eardrum. The stapes becomes frozen in place and cannot vibrate, impairing sound’s ability to pass 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, which typically starts in one ear, is usually the first symptom. Many people are initially unable to hear low sounds or a whisper. Some experience dizziness, balance problems or tinnitus.

A patient with a normal eardrum and 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 that cannot be treated surgically “may be due to technical problems with the audiologist,” he noted.

“Most of the time, a good ENT will come up with the correct diagnosis,” he said of the diagnosis. “She had other symptoms, including congestion and a feeling of fullness in her ears, which might have led people in other directions.”

Otosclerosis can be treated with hearing aids, but stapedectomy surgery can provide better results.

The surgery involves inserting a prosthetic device into the middle ear to replace the staples, restoring hearing. Some hearing loss may continue after surgery. And sometimes people who undergo the surgery end up with hearing loss.

Schultz, who had never heard of otosclerosis, said she was excited that she might be able to solve the problem that was “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.

The hardest months, Schultz said, were the ones leading up to the first surgery. 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 did not know when it was her turn to speak.

Schultz has since regained about 90 percent of his hearing in both ears. The congestion and stuffy feeling is gone. Tinnitus remains but is mild.

Her diagnosis had other consequences.

Her mother, who had been told several years earlier that her hearing loss was age-related, was found to have otosclerosis, but decided to have surgery. And as a result of Schultz’s experience, one of her work colleagues was diagnosed with otosclerosis and underwent a successful operation.

“I hear most of the sounds now, and it’s wonderful,” Schultz said. “I remember sitting in my kitchen and hearing a low moaning sound and realizing it was coming from the fridge and I hadn’t been able to hear it for years. I thought, ‘This is amazing!’”

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