She had a loud, nonstop crunching noise in her head that doctors couldn’t quiet

She had a loud, nonstop crunching noise in her head that doctors couldn’t quiet

By Sandra G. Boodman , Reporter

August 18 ,2018                                     The Washingto Post

Maryjane Behforouz’s attempts to ignore the disturbing noise in her head always ended in failure, leaving her feeling increasingly desperate. No one seemed to know what was causing the nearly constant clicking — or sometimes crunching — sound that was so loud it would wake her in the middle of the night.

Behforouz, 48, who lives outside Indianapolis, had tried everything she could think of to make it stop.

She had seen three ear, nose and throat specialists, undergone painful steroid injections in her ear, tried acupuncture and changed her diet, all in a vain attempt to drown out the persistent auditory intrusion. When her doctors seemed unable to help, she scoured the Web, intensely focused on finding an expert who could.

Her husband, an ophthalmologist, was sympathetic, but at a loss to explain what sounded to Behforouz “like someone clicking their fingernails together, amplified by a megaphone.”

It was only after the cause had been identified and eliminated more than a year later by an expert Behforouz had found that she realized “how much energy it took every day just to deal with it.”

‘Driving me crazy’

Behforouz remembers very clearly when her problem started.

In July 2015, while driving near her home, 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 ENT. 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 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. The treatment is typically performed on patients with severe Meniere’s disease or sometimes sensorineural hearing loss. Behforouz agreed.

She had developed two additional problems: a high-pitched ringing sound known as tinnitus and the loud clicking noise. 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.

Behforouz consulted a third ENT at a teaching hospital. He concurred with the first two doctors and told her there wasn’t much more he could do.

On her own

At this point, 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. An estimated 72 percent of women with the mutation will develop breast cancer by age 80 (compared with 12 percent of average-risk women) and 44 percent will develop ovarian cancer (compared with 1 percent.)

Behforouz immersed herself in research about the gene and looked for experts who specialize in treating women who inherit such mutations. That led her to Boston’s Dana-Farber Cancer Institute,where Behforouz underwent a total hysterectomy and the removal of her ovaries in her 30s — a radical but, doctors say, effective way to prevent cancer. Behforouz also underwent a prophylactic double mastectomy and reconstruction at a New York hospital whose experts she had carefully vetted. Actress Angelina Jolie, who inherited the same gene, underwent similar surgeries several years ago.

Once again, Behforouz’s search led her to Boston, this time to another specialty hospital: Massachusetts Eye and Ear. “I kept feeling that there had to be a satisfactory explanation for the clicking and cause of my hearing loss,” she said. She pored over the hospital’s website, checking out the profiles of specialists. “I was looking to see what they’d published,” she said, paying particular attention to doctors who had focused on sudden sensorineural hearing loss.

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. “Her training and research indicated that she might know what was happening,” Behforouz said. She hoped a doctor at Mass Eye and Ear — a large referral center whose staff is accustomed to seeing unusual cases — might have encountered something resembling hers.

Behforouz planned to be in Boston in September 2016 on a college tour with her older daughter. She called Stankovic’s office and made an appointment.

What the patient is saying

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

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

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.”

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The sinking state

This is what happens when climate change forces an entire country to seek higher ground

By Joshua Keating  JULY 26, 2018

The central Pacific nation of Kiribati has a few claims to fame. Its flag-bearer at the past two summer Olympics won international attention and became a meme because of his memorable dancing. The country — known under British colonial rule as the Gilbert Islands (the name Kiribati, pronounced KI-ri-bahss, is a local transliteration of “Gilberts”) — has 33 islands spread over more than 1.3 million square miles, making it one of the world’s largest nations in terms of sea area, though one of the smallest in terms of land. But what it gets the most attention for these days is its impending doom: The nation may be one of the first in line to be wiped out by the effects of climate change.

In the century to come, we’re likely to see dramatic alterations to the physical shape of the world as we know it, thanks to rising sea levels and other environmental changes. But the immediate challenges faced by most countries pale in comparison to those of Kiribati, which has an average elevation of less than six feet. The atoll of Tarawa, where nearly half the country’s 110,000 residents live, could soon be substantially underwater. “By 2050, 18-80% of the land in Buariki, North Tarawa, and up to 50% of the land in Bikenibeu, South Tarawa could become inundated,” the government told the United Nations in 2015. Kiribati’s smaller outlying islands could be wiped out even sooner. “The results of sea level rise and increasing storm surge threaten the very existence and livelihoods of large segments of the population,” officials wrote.

Small island states like Kiribati and the Maldives have become symbols of the potential impacts of global warming. At the 2015 Paris climate summit, they pressured larger countries to accept the goal of limiting global warming to 1.5 degrees Celsius, rather than two degrees, over preindustrial levels. (It was mostly a symbolic victory: Barring unforeseen circumstances, particularly since the Trump administration pulled the United States out of the accord, both targets will be exceeded.) They are also working to develop first-line defenses against the effects of sea-level rise, including planting mangroves to prevent coastal erosion and improving rainwater-collection systems to protect water quality.

But if none of that works, they may have to consider more drastic options. And so, in 2014, Kiribati purchased about eight square miles on the Fijian island of Vanua Levu for a little less than $9 million, potentially for the purpose of moving its population there one day. “We would hope not to put everyone on one piece of land,” the country’s then-president, Anote Tong, said. “But if it became absolutely necessary, yes, we could do it.” Fiji would become the new home of the nation’s inhabitants, known as the I-Kiribati.

The relocation of people due to climate change isn’t unprecedented. Papua New Guinea has already begun moving the population of the Carteret Islands, a group of low-lying atolls, to the mainland. But this would be the first time an entire country had to relocate because the land on which it was built no longer existed. This raises a new and frightening question: If a country no longer exists in physical form, can it still exist as a political entity? Can a nation just up and move?

About half the population of Kiribati lives on Tarawa atoll. In the next 30 years, officials say, substantial portions of Tarawa could be submerged by rising seas. (Richard Vogel/AP)

I knew Tong by reputation from the impassioned speeches he delivered at U.N. General Assemblies and climate change conferences during his time as president, from 2003 to 2016. So when I visited Kiribati in 2016 to research a book about border changes and the future of the world map, I called him. When we met one afternoon in Tarawa, he had just come in from fishing and was relaxing in shorts and a sleeveless T-shirt in the maneaba, or meeting house, outside his family’s home in a crowded residential neighborhood. John Denver played softly from a Bluetooth speaker. But the former president was troubled. “One of the most difficult things I’ve had to expect is planning for the demise of my country,” Tong told me.

He wants the I-Kiribati to stay if it’s even remotely possible. But, he rued, relocation is probably unavoidable. “The science is pretty clear: zero emissions, we’ll still go underwater. Unless some drastic work is undertaken, there will be no option. That’s the reality. It’s not a hope. It’s not a desire. It’s the brutal reality.”

Yet no one’s quite sure what that reality will look like. When I visited Secretary of Foreign Affairs Akka Rimon, she cracked the joke I’d been afraid to make: “Climate change really put us back on the world map. The irony is that we’re being erased from the world map.” Rimon had tried to think through what relocation could entail, though she didn’t really know how Kiribati’s nationhood could be preserved. “We don’t have the answer. There doesn’t seem to be any entity that looks after that. Sovereignty exists within the borders of your nation, but what happens when that changes? Nobody has the answer,” she said.

Historically, countries are not physically destroyed; they simply become other countries, the land they occupy controlled by someone else. But at a minimum, to exist, a country needs a government, a population and a piece of real estate within a defined territory — the boot of Italy, the hanging triangle of India, the narrow strip of Chile. The shape of a nation has long been defined by two kinds of lines: the borders that separate it from other countries and the coasts that separate it from the sea. We may understand why political borders are subject to change, but in an era of rising seas and increasingly extreme weather and natural disasters, we have to get used to the fact that coastal boundaries can’t be taken for granted, either. Indeed, our land-water borders are changing quickly and significantly, and in ways that will probably never be reversed.

Environmental-law scholars have begun to discuss the notion of “ex-situ nationhood,” under which governments, with some financial support from the international community, would continue to represent their populations on an international level at bodies like the United Nations, without any connection to a physical territory. Under one model, the I-Kiribati would retain some rights as citizens, even as they dispersed around the globe. As Maxine Burkett of the University of Hawaii, who has written extensively on the political dilemmas facing small island states, told me in 2014: “A number of us understand the modern notion of citizenship, where people have ties to more than one country. But the notion of that happening without a physical territory is quite novel.”

In a 2013 essay, Jenny Grote Stoutenburg, a law professor at the University of Colorado at Boulder, recommended that, to maintain international recognition, island states facing destruction should reinforce their territory to keep at least some physical structure above water and keep a small group of inhabitants behind, even if the bulk of the population has relocated. The Kiribati of the future, in other words, may be little more than a skeleton crew, a reinforced platform with a flag perched in the open ocean after the rest of the population has moved to another piece of land or to several of them. This is a very different notion of national sovereignty than anything the world has seen before.

There are understandable motivations behind plans like these: The people of small island states want to continue to have political representation in the international community, and they have economic interests to protect — rights to fisheries and natural resources in their territory, for instance. But these plans also offer a version of cartographical stasis taken to the point of parody: the erection of a fig-leaf physical presence in the middle of the ocean just so that maps showing a country in a particular place will be technically correct.

Still, a nation ending entirely, with no successor, might be a wholly new event in human history. In grappling with the possibility, some scholars have dusted off models and concepts that predate the modern nation-state.

One is the Sovereign Order of Malta — a Catholic order that controls no physical territory but has existed in multiple locations, including Jerusalem, Cyprus, Malta and Rome, throughout its nearly 1,000-year history. In an odd geopolitical quirk, despite controlling no territory today, the order has diplomatic relations, including embassies, with dozens of countries and observer status at the United Nations.

The order’s sovereign status makes it a throwback to an earlier, more fluid era of international politics, when sovereignty was tied more closely to ruling families or dynasties than to territories with fixed locations. Today, for instance, the historical kingdom of Burgundy is associated with the central French region of that name. But in his book “Vanished Kingdoms,” British historian Norman Davies identifies 15 kingdoms of Burgundy dating back to 410 and occupying locations from the west bank of the Rhine to what is now Switzerland to the Netherlands. Describing the disintegration of Burgundy in the 13th century, Davies writes: “The typical Burgundian count was no longer the ruler of one straightforward fief dependent on one overlord. More often he was head of a complex clutch of lands, titles and claims, assembled over the generations by the combined efforts of his family’s knights, wives, children and lawyers.”

If the vanishing countries of the future are to survive in any form, they’re likely to look less like contemporary nation-states and more like the Knights of Malta or medieval Burgundy, political creations set up to represent a group of people, and their political interests, who will be increasingly dispersed geographically and culturally.

These are not scenarios you’re likely to hear much about in Kiribati. For all that countries like Kiribati have done to bring urgency to the fight against climate change, many locals seem no more troubled about the issue than people in the United States are. When I visited on my reporting trip, people for the most part agreed that the climate was changing, pointing to shifting rainy seasons and irregular fishing patterns. But they usually didn’t believe that the islands would come to an end.

I heard several odd pseudoscientific arguments from Kiribati people during my time there, including that hotter weather would evaporate all the water released by the melting polar ice caps and that the coral in the Kiribati atolls would help the islands float as the water rose. Many people pointed out, correctly, that the shape of the islands regularly shifted before sea-level rise — and that the impacts of climate change so far had been difficult to disentangle from other factors. Most people I met weren’t making plans to relocate anytime soon. In contrast to Tong, the new government has not made the evacuation of Kiribati a priority, even a theoretical one.

Instead, I heard a lot of frustration that the rest of the world seems to take notice of the I-Kiribati only to tell them they’re doomed. Several people I spoke with had already given interviews about climate change to foreign reporters. “In my case, you are the fifth person,” remarked Teewata Aromata, the director of Te Toa Matoa, an organization for people with disabilities. “People come and ask us the same questions. They see pictures of us and think we are drowning in the ocean.”

Yet the stubborn facts remain. Countries like the Maldives and Kiribati are probably disappearing — and not that long from now. I came to Kiribati expecting to find a place planning for its own destruction, but instead I found something more dispiriting: a place that, with a few exceptions, wasn’t even contemplating that destruction. “Who wants to believe that their home won’t be here?” said Tong. It was an understandable sentiment. “People here don’t even like to plan for next week. But we’ve got to be hardheaded about it.”

The mental block that prohibits thinking about what will happen when the islands are no longer inhabitable seems to be a major impediment to planning for that eventuality. In this regard, too, Kiribati is a microcosm of the world’s unwillingness to face the reality of the future. A nation disappearing off the map is something that’s never happened before and, so far, is something people seem unable to imagine.

He is accused of killing someone in a parking spot dispute. Authorities say he was standing his ground.

He is accused of killing someone in a parking spot dispute. Authorities say he was standing his ground.

By Cleve R. Wootson Jr.    July 21 at 4:17 PM                 The Washington PostPinellas County Sheriff Bob Gualtieri showed a video of a deadly July 19 shooting in Clearwater, Fla., and explained why Florida law protects the shooter. (Pinellas County Sheriff’s Office)

Britany Jacobs sat parked in the handicap spot, right in the middle of Michael Drejka’s pet peeve.

She had just finished up a nursing shift Thursday, and she and her boyfriend, Markeis McGlockton, had a car full of children, all under age 6. So she sent McGlockton and their 5-year-old into a Circle A in Clearwater, Fla., for snacks and drinks while she rested in the parked car — or at least tried to.

Also in the lot was Drejka, a regular at the Circle A who regularly took issue with able-bodied people parking in the reserved spot. He circled Jacob’s car, looking for a handicap decal and, finding none, proceeded to forcefully explain to her the finer points of Florida’s disabled parking regulations.

“He’s getting out like he’s a police officer or something, and he’s approaching me,” Jacobs told the Tampa Bay Times.

Jacobs said the conversation grew heated, drawing the attention of other store patrons, including McGlockton, who abandoned his snack run. He came out of the store, then quickly closed the distance between himself and the man confronting the mother of his children and shoved Drejka to the ground.

That action, and the seconds that followed it, have thrust the dispute over the handicap parking spot into the nationwide debate about “stand your ground” laws.

Now seated on the ground, Drejka reached into his pocket, pulled out a pistol and fired a single shot into McGlockton’s chest, an action shown clearly on surveillance video released by the Pinellas County Sheriff’s Office.

McGlockton clutched his chest, staggered into the convenience store and collapsed. Later, his girlfriend ran into the store and applied pressure to the bullet wound in an unsuccessful attempt to stop the bleeding.

McGlockton, 28, died a short time later, leaving his family to bury him and the rest of Pinellas County to grapple with the legality of his killer’s actions.

On Friday, Sheriff Bob Gualtieri announced that Drejka would not be arrested or charged with a crime, saying that his actions fell within the legal boundaries of Florida’s “stand your ground” law. Then, in an expansive 30-minute news conference, he tried to explain how the law connected to what was going through Drejka’s mind when he pulled the trigger.

Drejka “felt after being slammed to the ground, the next thing was he was going to be further attacked by McGlockton,” said Gualtieri, who has been sheriff since 2011 and also has a law degree. “He felt the next thing was that he was going to be slammed again. He was going to be struck again and he was in fear.”

Reporters asked whether the fact that Drejka initiated the incident made him more culpable — pointing to previous complaints the sheriff’s office has received about him.

A few months ago, according to the Tampa Bay Times, Rick Kelly parked his tanker truck in the same handicapped spot and said he was confronted by Drejka.

Drejka walked around his truck, looking for handicap decals, then demanded to know why Kelly had parked there, the trucker told the Tampa Bay Times. At one point Drejka threatened to shoot Kelly.

“It’s a repeat. It happened to me the first time. The second time it’s happening, someone’s life got taken,” Kelly told the Tampa Bay Times. “He provoked that.”

Still, the Gualtieri told reporters, the legal question is not about whether Drejka was right in being the self-appointed protector of the handicap spot.

“What’s relevant is not whether this guy’s a good guy, nice guy, or whether he’s a jerk, or whether he’s a thorn in people’s side and what he’s done, whether it’s three weeks ago, three months ago or three years ago,” Gualtieri said. “What’s relevant and the only thing we can look at here is was he in fear of further bodily harm.”

Floridians have always had the right to defend themselves, but the state’s “stand your ground” law  says people who believe someone is trying to kill or seriously harm them don’t have an obligation to retreat before using deadly force. The law was spotlighted following the 2012 slaying of 17-year-old Trayvon Martin when jurors discussed the statute in their deliberations before deciding to find George Zimmerman not guilty.

In the past, defense attorneys had to explain why their clients deserved immunity in a killing. Now prosecutors have to prove that people who claim they were standing their ground are wrong.

Last year, lawmakers shifted the burden of proof from defense attorneys to prosecutors. It has made the law no less controversial.

“Does this law create a situation potentially where people shoot first and ask questions later?” Gualtieri asked. “You can have that discussion. You can have that debate. I don’t make the law. We enforce the law. And I’m going to enforce it the way it’s written, the way the legislature intended for it to be applied, and others can have the debate about whether they like it or not.”

It is unclear whether Jacobs and her family will be involved in that debate. She told the Tampa Bay Times she is hiring an attorney to consider her legal options.

For now, there are bigger things to contend with.

There is a funeral to plan, and also the needs of the living.

After the shooting, as McGlockton bled to death on the floor of the convenience store, his 5-year-old son, Markeis Jr., watched as his mother pressed a shirt to the chest of his mortally wounded father, trying unsuccessfully to keep him alive.

“He’s not too good,” Jacobs said of the boy, according to the Tampa Bay Times. “It comes and goes, but he knows [his father] is dead.”