Medical Mystery: What caused that maddening clicking in her head?

Maryjane Behforouz remembers very clearly when her problem started.

In July 2015, while driving near her home outside Indianapolis, she felt an itch deep in her left ear, as though something was tickling her eardrum. She suspected the problem was residual water in her ear from swimming, or the remnant of a recent cold. Behforouz covered the opening of her ear with a fingertip then forcefully pressed several times in quick succession to create pressure that would expel the water and eliminate the itch. Almost immediately, she realized that her hearing in that ear seemed diminished.

Ten days later, Behforouz consulted an ear, nose and throat specialist. He diagnosed mild hearing loss and prescribed a nasal spray and antibiotics. It was possible, he told her, that a cold had caused a bacterial infection that was muffling her hearing.

When the drugs didn't help, he sent Behforouz, 48, to a colleague with a more specialized practice. This ENT asked whether she had a family history of hearing loss — she didn't — and told Behforouz that she had sensorineural hearing loss, the result of damage to nerve cells in the inner ear.

Causes in adults include aging or exposure to loud noise; such hearing loss is permanent, but can be alleviated by wearing a hearing aid. In Behforouz's case, the cause was deemed to be idiopathic — medical jargon meaning unknown. Possible explanations, the doctor told her, were an unspecified autoimmune problem or Meniere's disease, a disorder of the inner ear, although Behforouz lacked the vertigo typical of Meniere's.

The ENT recommended a series of intratympanic steroid injections, which involves injecting the drug into the middle ear; the goal in her case was to reduce inflammation and diminish her hearing loss. Behforouz agreed.

She had developed two additional problems: a high-pitched ringing sound known as tinnitus and a nearly constant clicking sound that was so loud it would wake her in the middle of the night. Behforouz found the tinnitus was manageable — she could drown it out while she slept by using the white noise of a fan.

But the clicking, she recalled, was "literally driving me crazy." It interfered with her ability to have a simple conversation or to listen to music, and was constantly distracting.

After a consultation with a third ENT was unhelpful, Behforouz said, she realized she was more or less on her own. She turned to the Internet, which had proved to be an invaluable source of help nearly a decade earlier when she confronted a frightening finding.

In 2007, Behforouz learned that she had inherited the BRCA1 gene, which greatly increases the lifetime risk of both breast and ovarian cancer. Seeking experts who specialize in treating women who inherit such mutations led her to Boston's Dana-Farber Cancer Institute for prophylactic surgeries.

Now, Behforouz's search for answers to her hearing problem led her to Boston, this time to another specialty hospital: Massachusetts Eye and Ear. She pored over the hospital's website, checking out the profiles of specialists.

She zeroed in on one: Konstantina Stankovic, an associate professor of otolaryngology at Harvard Medical School who earned both a medical degree from Harvard and a doctorate in auditory neuroscience from MIT. She made an appointment.

As she had with each doctor she had seen, Behforouz recounted "the whole rigmarole."

"It was her story that really told me what it probably was," Stankovic said of her initial meeting with Behforouz. "You really have to listen to a patient's story to even think of it."

To Stankovic, chief of the division of otology and neuro-otology, the problem did not sound idiopathic; she suspected that Behforouz had inadvertently fractured a tiny, delicate bone in her middle ear called the malleus.

When she examined the records of Behforouz's previous hearing tests, Stankovic realized that the results had been misinterpreted. Behforouz didn't have sensorineural hearing loss — damage to the nerve. Instead, she had conductive hearing loss, a problem with the way sound is transmitted. The difference is important because some forms of conductive hearing loss can be fixed through surgery.

Malleus fractures, which prevent sound from being properly delivered to the middle ear, are rare, Stankovic said, and probably underdiagnosed. She said researchers at Mass Eye and Ear have studied 13 patients with the fracture and "every one had the same story": sudden hearing loss after what doctors call "digital manipulation."

Moisture "provides the perfect pressure seal," Stankovic said, and force can result in fractures to the bones in the ear, which are the smallest in the body. Behforouz had an additional risk factor: osteoporosis, the bone-thinning disease.

Behforouz said she "nearly fell off the chair" when, five minutes into her first appointment, Stankovic told her what she suspected had happened.

"She really listened to my story, and her history was much more thorough," Behforouz recalled. The hearing tests were more extensive and careful, she said, describing them as "a very different experience."

Stankovic told Behforouz that the problem could be corrected surgically, although her hearing loss, which was mild, might not improve and could even get worse.

It was a chance Behforouz was willing to take.

A month later, she returned to Boston. In an hour-long operation, Stankovic repaired the fracture using bone cement. But to Stankovic's surprise, the broken bone wasn't the malleus but another tiny one called the incus.

Behforouz said she was elated by the results. The clicking immediately ceased, although her hearing loss and tinnitus were largely unchanged. Behforouz was also relieved that the problem wasn't caused by a progressive disorder that she feared could impair hearing in her right ear.

"I think this is very diagnosable," said Stankovic, who explained that the clicking was caused by two fragments of bone vibrating out of sync in response to sound. "You just have to be thinking about it. The important thing is you really have to listen to a patient."