The Pennsylvania accountant often had to ask people to repeat themselves, which embarrassed her. And she began cranking up the volume on her television, an accommodation she hadn't thought would be necessary for years. Image Credit: Shutterstock

At 47, Marlene Schultz thought she was much too young to keep saying "What?" when she couldn't hear what people were telling her.

The Pennsylvania accountant found it increasingly difficult to make out the voices of her teenage sons, much to their irritation. At work, Schultz often had to ask people to repeat themselves, which embarrassed her. And she began cranking 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 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 audiology testing that found low-frequency hearing loss in both ears. When Schultz told the doctor 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 probable cause of her permanent hearing loss. The only treatment, he advised, was hearing aids.

Special Reports

"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 exploring that question.

But Schultz was. Her persistence fueled an 18-month quest that involved an allergist, an endocrinologist and two additional ENTs, the second of whom discovered the underlying and treatable reason for 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 sit right," she said, "it's important to get a second opinion - and maybe more."

Clogged ears

Based on tests that found Schultz was unable to hear low-pitched sounds, the first ENT concluded 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 ageing, 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 transfers sounds to the inner ear. Conductive hearing loss can be caused by a perforated ear drum, fluid in the ear, impacted ear wax, infection or a benign tumour. Depending on the cause it may be curable. Some people have a mixture of conductive and sensorineural hearing loss.

A patient with a normal eardrum and the inability to hear low tones is kind of classic for otosclerosis.

- Douglas Bigelow, a head and neck surgeon

When she was ready, the ENT advised Schultz, she could be fitted 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 muddle through and hope her hearing didn't get worse.

But a year later it had. Not only were sounds more muffled, her ears felt perpetually clogged, as though she had a bad cold. To make matters worse, Schultz had 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 whether her ear congestion might be related to her deteriorating hearing.

Is it postnasal drip or allergy?

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 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 performed skin testing for common allergens: trees, pollen, dust mites, mould 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.

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 flagging hearing, Schultz had devised her own remedy. Once an hour she put a finger in each ear to relieve the pressure. It worked, but only briefly.

"I was getting desperate," she recalled and made an appointment with an endocrinologist to see if he had any ideas. He recommended two over-the-counter drugs but zeroed in 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 scan of her brain, which doctors hoped might 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 worse and Schultz was no further along than when she started.

"I wasn't sure what to do or where to go," she recalled.

Where to turn?

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 perused the website of Penn Medicine, scrutinizing 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 audiology 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.

A condition called otosclerosis

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 becomes frozen in place and cannot vibrate, thereby impairing the ability of sound to pass into the inner ear.

I was so relieved to know what I had and excited there was a way to fix it.

- Marlene Schultz

Gradual hearing loss, which typically starts in one ear, tends to be 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 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 observed.

"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 feelings of fullness in her 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 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 wind 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 was "driving me nuts."

Finally, hope at the end of the tunnel

"I was so relieved to know what I had and excited there was a way to fix it," she said. Subsequent CT scans confirmed 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 most difficult months, Schultz said, were those leading up to the first surgery. During 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 congestion and clogged feeling have disappeared. The tinnitus remains but is mild.

Her 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 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 wonderful," Schultz said. "I remember sitting in my kitchen and hearing a low groaning sound and realizing it was coming from the refrigerator and that I hadn't been able to hear it for years. I thought, 'This is great!'"