His persistent 18-month search led to a third ear, nose and throat specialist who discovered the curable reason
So in May 2018, Schultz saw 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 audiology tests which revealed 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 likely cause of her loss. permanent hearing. The only treatment, he advised, was hearing aids.
“I was pretty upset,” Schultz recalled. Why, she wondered, would someone who had only been to a handful of rock concerts and had little exposure to noise need hearing aids at such a young age? The doctor did not seem interested in exploring this question.
But Schultz was. Her perseverance fueled an 18-month quest involving an allergist, an endocrinologist and two additional ENT specialists, the second of whom discovered the underlying and treatable reason for her problem. It was a discovery that would dramatically improve Schultz’s quality of life and have implications for his 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 revealed Schultz was unable to hear low-pitched 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. This usually affects the ability to hear high frequencies, such as female voices. The most common cause is aging, although loud music or a knock to the head can also produce it.
The other type of hearing loss – conductive – usually affects the middle ear, which transfers sound 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 mixture 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. She decided to fend for herself and hope her hearing wouldn’t get worse.
But a year later, it was. Not only were the sounds more muffled, but her ears were perpetually clogged, as if she had a bad cold. To make matters worse, Schultz had recently started a new job in an open-plan office, where her colleagues spoke in soft tones to avoid disturbing others.
In July 2019, Schultz saw a second ENT affiliated with another health system. She told him about the results of her audiology tests and asked if the congestion in her ears could be linked to the deterioration of her hearing.
The second specialist diagnosed a postnasal drip and told Schultz that his Eustachian tubes, which connect the nose and middle ear, were blocked. The doctor suspects an allergy might be to blame.
He prescribed a steroid nasal spray to unclog his ears, which could improve his hearing, and recommended that Schultz see an allergist if his 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. Each test was negative. The allergist concluded that Schultz suffered from vasomotor rhinitis – a common condition of unknown cause that results in nasal inflammation. Environmental triggers can include stress, temperature changes, spicy foods, 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 recommended Schultz continue using the nasal spray.
In an attempt to clear his blocked Eustachian tubes and regain some of his weakened hearing, Schultz had devised his 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 recalls and made an appointment with an endocrinologist to see if he had any ideas. He recommended two over-the-counter medications, but focused on his enlarged thyroid. In late October, he performed a needle biopsy on a peanut-sized lump that turned out to be benign.
Three weeks later, Schultz underwent an MRI of her brain, which doctors hoped could shed some light on the cause of the fullness in her ears and possibly her hearing loss. It did not reveal anything abnormal.
After more than a year of searching, her hearing deteriorated and Schultz was no further ahead than when she started.
“I didn’t know what to do or where to go,” she recalls.
At a relative’s suggestion, Schultz contacted one of her cousins, an ENT in Boston.
He advised her to see a hearing specialist at one of the major teaching hospitals in Philadelphia. Schultz scoured the Penn Medicine website, scouring descriptions from various otolaryngologists, and made an appointment with a specialist whose expertise seemed promising.
Four weeks later, in December 2019, she met 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 and not sensorineural. This meant that depending on its cause, his problem could be fixable.
His age, symptoms and test results, Bigelow told him, 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 hereditary. 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 stirrup freezes in place and cannot vibrate, impairing the ability of sound to pass through 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 begins in one ear, tends to be the first symptom. Many people are initially unable to hear low-pitched sounds or a whisper. Some suffer from vertigo, balance problems or tinnitus.
A patient with a normal eardrum and an inability to hear low-pitched sounds “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 right diagnosis,” he said of the diagnosis. “She had other symptoms, including congestion and a feeling of fullness in her ears, which could 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 prosthesis into the middle ear to replace the stapes, restoring hearing. Some hearing loss may persist after surgery. And sometimes people who have the operation end up with poor 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 thrilled that there was a way to fix it,” she said. Later CT scans confirmed she had otosclerosis in both ears.
Bigelow, who estimated to have performed around 1,000 stapedectomies over his 30-year career, operated on Schultz’s left ear in June 2020. Surgery on his right was performed a year later.
The toughest months, Schultz said, were those leading up to the first surgery. Early in the pandemic, while working from home, Schultz spent hours in dreaded Zoom meetings, struggling to hear what others were saying. Often, she didn’t know when it was her turn to speak.
Schultz has since regained around 90% of his hearing in both ears. Congestion and stuffiness are gone. Tinnitus persists but is mild.
His diagnosis had other ramifications.
His mother, who had been told years earlier that her hearing loss was age-related, was found to have otosclerosis but decided not to have surgery. And as a result of Schultz’s experience, one of his co-workers was diagnosed with otosclerosis and had successful surgery.
“I hear most of the sounds now and it’s wonderful,” Schultz said. “I remember sitting in my kitchen and hearing a low moan and realizing it was coming from the fridge and I hadn’t been able to hear it in years. I thought, ‘That’s great!’ »
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