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 treatable cause.

(Cam Cottrill for The Washington Post)


At 47, Marlene Schultz thought she was way too young to keep saying “What?” when she could not hear what was being said to her.

The Pennsylvania accountant found it increasingly difficult to distinguish the voices of her teenage sons, much to their chagrin. 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 necessary for years.

So in May 2018, Schultz consulted the same suburban 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 an audiology test, 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 otherwise had little exposure 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 prompted an 18-month search that involved an allergist, an endocrinologist and two more ENTs, the second of which 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 implications for her family as well as a co-worker.

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

Based on tests that found Schultz was unable to hear soft 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 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 produce it.

The other 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, impacted earwax, infection, or a benign tumor. Depending on the cause it may be treatable. Some people have a mix of conductive and sensorineural hearing loss.

When she was ready, the ENT advised Schultz, she might be a good fit for 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 get confused and hope her hearing didn’t get any worse.

But a year later it had. Not only were sounds muffled, her ears felt constantly blocked, like she had a bad cold. To make matters worse, Schultz recently started a new job in an open-plan office where her co-workers spoke in soft voices to avoid disturbing others.

In July 2019 Schultz consulted a second ENT affiliated with a different health system. She told him about the results of her audiology tests and asked if her ear congestion might be related to her worsening hearing.

The second specialist diagnosed postnasal drip and told Schultz that her Eustachian tubes, which connect the nose and middle ear, were blocked. The doctor suspected that an allergy might be to blame.

He prescribed a steroid nasal spray to unclog her ears, which might improve her hearing, and recommended that Schultz consult an allergist if her condition did not improve.

A month later, she saw an allergist who did skin testing 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 can include stress, temperature changes, spicy food, paint fumes, perfume, or certain medications.

“I’m desperate.”

— 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 her blocked Eustachian tubes and regain some of her fading hearing, Schultz came up with 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, and made an appointment with an endocrinologist to see if he had any ideas. He recommended two over-the-counter medications but was wary of 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 scan of her brain, which doctors hoped might shed light on the cause of her ear fullness and eventually her hearing loss. It revealed nothing abnormal.

After more than a year of searching, her hearing was worse and Schultz was no further ahead 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 her cousins, an ENT in Boston.

He advised her to see a hearing specialist at one of Philadelphia’s large teaching hospitals. Schultz scoured Penn Medicine’s website, examining 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 division 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 conductive, not sensorineural. That meant that depending on its cause, her problem might be fixable.

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 thought to be 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 stapes become frozen in place and cannot vibrate, thus impairing the ability of sound 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 usually starts in one ear, tends to be the first symptom. Many people are initially unable to hear soft sounds or whispers. Some experience dizziness, balance problems or tinnitus.

A patient with a normal eardrum and an 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 problems with the audiologist,” he observed.

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

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

The operation involves the insertion into the middle ear of a prosthetic device to replace the stapes, 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 that she might be able to fix the problem that “gets me out.”

“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 year was performed a year later.

The hardest months, Schultz said, were those leading up to the first surgery. During the early days of the pandemic 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 eventually recovered about 90 percent of her hearing in both ears. The congestion and stuffy feeling is gone. 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 found to have otosclerosis but decided against surgery. And as a result of Schultz’s experience, one of her work colleagues was diagnosed with otosclerosis and underwent successful surgery.

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

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