When Ann Schueller was pregnant with her fourth child, she noticed a change in her hearing. “Why do you have that up so loud?” her husband would ask, walking into a room where she was watching a Brewers game on TV. A 40-year-old Shorewood resident, Schueller hadn’t experienced this problem during previous pregnancies. She checked with her obstetrician, who suggested she get her ears evaluated after delivery if her hearing was still bad.
It was. At night, Schueller wouldn’t hear the baby crying, only waking when her husband returned to bed from tending to him. Her right ear was much worse than her left, and if she rolled onto her good ear at night, she couldn’t hear a thing. Still, she didn’t see a doctor. “I was so busy with the baby and the other kids, I just put it out of my mind and thought, ‘It’ll get better,’ ” Schueller recalls.
Finally, a friend convinced her it was time to start taking care of herself. An ear, nose and throat doctor diagnosed Schueller with otosclerosis, a condition that affects the bones of the middle ear and impairs hearing.
Anyone can develop otosclerosis, but the condition appears most commonly in Caucasian women. It’s estimated that 10 percent of Caucasian adults – both male and female – have some evidence of otosclerosis, although most will have no symptoms during their lifetime. The disease is progressive, meaning hearing and other symptoms worsen over time. In about 80 percent of cases, both ears are affected, although not necessarily to the same degree. Onset of the disease typically occurs in the late teens, 20s or 30s, but it may take decades before the symptoms are noticeable.
However, pregnancy can accelerate the progression of the disease, sometimes rapidly, for reasons that aren’t well understood. “In many women, it will be a pregnancy that results in the first complaints of hearing loss,” explains Dr.
Michael Nordstrom, an otolaryngologist with Greater Milwaukee Otolaryngology. While nothing can be done to reduce the impact of a pregnancy on hearing loss, there are good treatment options for otosclerosis that a woman can take advantage of after delivery.
Hearing Bones
Otosclerosis affects the bones of the middle ear, interrupting the delicate chain of motion that allows us to hear. In a healthy ear, sound travels down the ear canal to the eardrum, causing it to vibrate. These vibrations are transmitted to the inner ear by a series of three tiny bones. The last of these bones, called the stapes, sets the inner ear fluid waves in motion, which stimulates the hearing nerve to carry the signal to your brain so you can hear.
In an ear with otosclerosis, abnormal bone growth causes the stapes to harden and become fixed, so it doesn’t vibrate the way it should. Because the stapes can’t transmit sound, the patient experiences a loss of hearing. “The sound is not getting through the ear to the hearing nerve, but the hearing nerve is fine,” explains Dr. David Friedland, an otolaryngologist at Froedtert and Children’s hospitals, and an associate professor at the Medical College of Wisconsin. This type of hearing loss is called a conductive hearing loss, notes Nordstrom, because it impairs the conduction of sound from the eardrum to the inner ear. The majority of otosclerosis patients have conductive hearing loss only. However, in some cases, there may also be damage to the hearing nerve, called sensorineural hearing loss.
It’s unclear what causes otosclerosis to develop. Genetics plays a role in some cases; people with one or two parents who have otosclerosis have increased risks of developing the disorder. Other theories suggest there may be a viral component to the disease.
Gradual hearing loss is the most common symptom of otosclerosis. At first, patients might notice they can’t hear low-pitched sounds or whispers. Over time, a greater range of sound may be affected. Dizziness, balance problems and tinnitus – a sensation of ringing or buzzing in the ears – may also occur.
Despite the connection between pregnancy and the worsening of otosclerosis symptoms, pregnant women shouldn’t necessarily become alarmed if their hearing seems off. “There are many other reasons that women develop clogged ears and hearing problems during pregnancy,” says Friedland. Nasal congestion is common during pregnancy, he explains. But if the hearing problem doesn’t go away in the months after delivery, it should
be evaluated.
A Delicate Surgery
Patients who are diagnosed with otosclerosis have a few choices when it comes to treatment, beginning with doing nothing at all. “If a patient has a very mild hearing loss, living with it and doing nothing might be fine for her,” says Nordstrom. In this case, the doctor would continue to monitor the patient with hearing tests every year or two to track the disease, and more often if she noticed an acceleration of hearing loss.
If the hearing loss is more advanced, hearing aids are an option, one that can be very effective for otosclerosis, notes Friedland. A more controversial, over-the-counter treatment is the use of fluoride supplements to slow the progression of the disorder. Some physicians suggest this treatment as an alternative to patients; Friedland doesn’t recommend it. “I don’t find it overly effective, and I don’t find that most people are agreeable to the dosage and the side effects,” which can include upset stomach, he explains.
Perhaps the most effective treatment for otosclerosis is a delicate surgical procedure called a stapedectomy (or stapedotomy), which can restore or greatly improve hearing for many patients. One of the advantages surgery offers over a hearing aid is a superior sound quality, notes Nordstrom. “Almost all [patients] will do much better with the surgery in terms of the quality of sound. What they hear is much more natural, less mechanical.” Other advantages have to do with lifestyle and quality of life. “When [the surgery] works – and most often it works – it’s ‘on’ 24/7, there’s no upkeep, and insurance typically covers the cost of surgery,” says Friedland. The procedure has about a 90 percent success rate, and has been performed since 1956.
Still, not everyone is a good candidate for a stapedectomy. The treatment is only suitable for otosclerosis patients with conductive hearing loss; nerve hearing loss can’t be treated surgically. Likewise, patients with pronounced dizziness may be better candidates for a hearing aid because of the risk of complications.
Physicians generally prefer to perform the operation on a patient who has fairly significant hearing loss. “The worse your conductive hearing loss, the better we can probably anticipate the outcome being,” says Nordstrom. Patients with mild hearing problems may opt for the surgery down the road if their hearing continues to deteriorate.
Depending on the doctor, the surgery may be performed under general or local anesthetic, and it’s usually an outpatient procedure. Working through the ear canal, without any outer incisions, the physician removes part or all of the stapes and replaces it with a prosthesis. The device allows the bones of the middle ear to resume their movement, stimulating sound waves in the inner ear and thereby restoring hearing. “Basically, the procedure is bypassing the area that gets stuck,” explains Friedland. “It doesn’t cure the disease. It doesn’t stop the disease. It bypasses the effects of the disease.”
The results are generally long-lasting, although, as with any surgery, there are potential complications, including about a 1 percent chance that the patient could lose her hearing altogether. “As horrible as that sounds, some patients’ hearing is so bad, there’s not an appreciable difference [between severe and total hearing loss],” says Nordstrom. Facial nerve weakness or paralysis is another risk, albeit an uncommon one. Over the course of a lifetime, there is a chance that the prosthesis could pop out of place or otherwise fail. Revision surgery is possible, but it carries more risks than a first-time procedure, including an increased risk of substantial hearing loss.
Some patients experience dizziness for the first few days after surgery or a metallic taste in their mouth. In most cases, these side effects go away as the body heals. Because the ear is packed after surgery, patients won’t notice the hearing improvement right away. Friedland estimates a full recovery to take about three weeks.
Ann Schueller considered all the options for treating her otosclerosis. “I thought about hearing aids. But I really needed [my hearing] at night, and I suspected I would be taking the hearing aids out when I went to sleep for comfort.” Ultimately, she decided to have the surgery, and these days, she can detect a big difference. “I always thought my baby was a great sleeper,” she says, laughing. “Now, I can hear him at night.”
Caroline Goyette is Milwaukee Magazine’s monthly health columnist.
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