Health and Wellness

She was not happy with her diagnosis of early hearing loss. She was correct.

After a persistent search of 18 months, she finally found a third ear and nose specialist who was able to identify the cause.

(Cam Cottrill at The Washington Post).


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

Much to the annoyance of her teenage sons in Pennsylvania, the accountant found it more difficult to hear their voices. Schultz was often asked to repeat herself at work by people, which embarrased her. And she began cranking up the volume on her television, an accommodation she hadn’t thought would be necessary for years.

Schultz saw the same suburban Philadelphia ear and nose specialist that her mother had seen many years ago when she suffered from hearing problems in her late 60s.

Audiology testing was ordered by the otolaryngologist. It revealed low frequency hearing loss in both of her ears. Schultz also mentioned to the doctor that she suffered from ringing in her ears (a condition called tinnitus). He said that her hearing loss was likely due to her having listened years ago to loud music. He advised hearing aids as the only way to treat her condition.

“I was pretty upset,” Schultz recalled. Schultz wondered why someone, who only had been to a handful of concerts and was not exposed to loud noise, would need hearing aids so young. The doctor didn’t seem interested in exploring that question.

Schultz was. Her persistence led to an 18-month journey that saw an allergist, an endocrinologist, and two additional ENTs. One of them found the underlying problem and treated it. 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.”

After tests showed Schultz could not hear low-pitched sounds, her first ENT determined that she had mild sensorineural loss in both of her ears. Sensorineural hearing loss, also known as inner ear damage, is common. It is the brain’s ability to hear and understand sounds. It typically affects the ability to hear high frequencies, such as women’s voices. It is most commonly caused by aging. However, it can also be caused by loud music or head trauma.

The other type of hearing loss — conductive — typically affects the middle ear, which transfers sounds to the inner ear. Conductive hearing loss may be caused by fluid in the ear, perforated eardrum, fluid in the middle ear, infection, benign tumors, or fluid in the ear. It can be treated depending on the cause. There are cases where conductive and sensorineural hearing losses can be combined.

Schultz was advised by her ENT that she could be fitted with hearing aids to improve but not restore her hearing.

“I didn’t have that kind of money,” Schultz said. Her insurance did not cover the devices, which cost her about $3,000 each. She decided to muddle through and hope her hearing didn’t get worse.

It had returned a year later. She felt like she had a bad virus and her hearing was muffled. Schultz was also new to a job at an open-plan office. Her coworkers speak softly in order not to disturb others.

Schultz consulted a second ENT who was affiliated with a different hospital in July 2019. He asked Schultz about her hearing tests results and whether her ear problems might be due to her declining hearing.

Schultz was diagnosed with postnasal drip by the second specialist. He said that Schultz’s eustachian tube, which connects the middle and nose, had become blocked. The doctor suspects that it could be an allergy.

He recommended that Schultz seek out an allergist to help her condition.

A month later, she went to see an allergist. He performed skin testing for common allergens like pollen, trees, dust mites, mold, and animals. All tests came back negative. The allergist concluded that Schultz had vasomotor rhinitis — a common condition of unknown cause that results in nasal inflammation. Stress, temperature changes and spicy foods, paint fumes and perfume, as well as certain medications, can all be environmental triggers.

“I was getting desperate.”

— Marlene Schultz

Another possibility was a bacterial infection. Schultz was recommended to continue using the nasal spray by an allergist.

Schultz devised her own solution to help clear her blocked eustachian tube and restore some of her hearing. To relieve the pressure, she placed a finger in each of her ears once an hour. But it worked only for a short time.

“I was getting desperate,” she recalled and made an appointment with an endocrinologist to see if he had any ideas. He suggested two prescriptions, but focused on her thyroid. He performed a needle biopsy of a small, peanut-sized tumor in October. It was confirmed to be benign.

Schultz had an MRI scan of the brain three weeks later. Doctors hoped it would shed some light on her ear swelling and possible hearing loss. The scan revealed nothing abnormal.

After over a year of research, Schultz’s hearing became worse. She was not further along than she was when she began.

“I wasn’t sure what to do or where to go,” she recalled.

Schultz contacted an ENT in Boston at the suggestion of a family member.

He advised her to see a hearing specialist at one of Philadelphia’s large teaching hospitals. Schultz looked at Penn Medicine’s website and reviewed the profiles of various otolaryngologists. He then scheduled an appointment with a specialist whose expertise seemed promising.

She met Douglas Bigelow in December 2019 four weeks later. He is a head-and-neck surgeon who leads the division of neurotology and otology.

Bigelow ordered a second round of hearing tests. These results were significantly different from the audiology original results. This time Schultz’s hearing loss was classified as conductive, not sensorineural. This meant that her problem could be fixed depending on the cause.

Bigelow said that her age, symptoms, and test results indicated that she had otosclerosis. This is the most common reason for middle-ear hearing loss in middle-aged and young adults.

Otosclerosis is a disease that affects approximately 3 million Americans, most of them white middle-aged women. Many cases can be inherited. Affected stapes is the smallest bone in your body behind the eardrum, and abnormal bone growth in the middle of the ear. The stapes become frozen and can no longer vibrate, which impairs the sound’s ability to reach 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 is usually a first sign. Many people experience difficulty hearing low-pitched sounds, or whispers at first. Some suffer from 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 also be treated with hearing aids. However, stapedectomy surgery might offer better results.

This procedure involves inserting a prosthetic device in the middle of the ear to replace the stapes. It restores hearing. Some hearing loss may persist after surgery. Sometimes, people who have the surgery 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 was “driving me nuts.”

“I was so relieved to know what I had and excited there was a way to fix it,” she said. She was diagnosed with otosclerosis in both her ears by CT scans.

Bigelow, who estimated he has performed about 1,000 stapedectomies in his 30-year career, operated on Schultz’s left ear in June 2020. One year later, the operation on Schultz’s right was done.

Schultz stated that the months leading up to the first operation were the most difficult. Schultz was unable to understand the conversations of others during the initial days of the pandemic. She often didn’t know when it was her turn to speak.

Schultz has since recovered about 90% of her hearing in both of her ears. The feeling of congestion and blockage have gone. Although the tinnitus is still present, it is now mild.

Her diagnosis also had other implications.

Her mother, who was told years before that her hearing loss was caused by age, was diagnosed with otosclerosis, but she opted 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!’”

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