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Jul 25, 2018 · Maine basketball coach with rare hearing disorder crosses country for specialized surgery at UCLA

Out of the blue, Richard Barron, 49, woke up deaf in one ear.

The University of Maine women’s basketball coach was ultra-sensitive to noise–the sound of someone loading the dishwasher was excruciating.

Even stranger, Richard began hearing his bones creak and his eyelids move. Far worse was the noise-related brain fog that descended on him inside the raucous basketball gym. He’d call a timeout during a game—only to hear the ref blow a whistle or the band strike up a tune–and forget why he called the timeout.

Forced to take a medical leave from work, Richard retreated to bed, and his wife and three children took turns tip-toeing upstairs to share their day in whispers.

Over five months, Richard visited three prestigious medical centers and saw 15 doctors. No one could provide a diagnosis.

Finally, a CT scan of his skull revealed a tiny opening in his ear bone and an audiologist diagnosed him with superior semicircular canal dehiscence (SSCD).

Researching online, Richard found a webinar by a UCLA Health neurosurgeon, Dr. Isaac Yang, who offered to review his records electronically and confirmed the SSCD diagnosis.

Drawn to the minimally invasive approach that Yang pioneered with UCLA ear surgeon Dr. Quinton Gopen, Richard flew across the country for surgery at Ronald Reagan UCLA Medical Center.

In the operating room, Yang made a small incision in Richard’s skull behind his ear and gently pushed his brain out of the way, allowing Gopen to plug the miniscule hole in the ear bone.

When Richard awoke, he was ecstatic to realize that the bizarre noises in his head had vanished and his hearing had returned. That night, his wife and he celebrated over dinner in a Los Angeles restaurant.

Gopen and Yang say Richard’s frustrating journey toward diagnosis isn’t unusual. Superior canal dehiscence wasn’t identified until 1998 – a recent enough discovery only now being added to medical school textbooks. As a result, most physicians are unfamiliar with the rare syndrome, which affects an estimated 1% of the population.

Learn more about Semicircular Canal Dehiscence at UCLA Neurosurgery.

 

Jul 5, 2018 · UCLA doctor diagnoses teen's complex disorder, restoring her vision, ability to enjoy life

Rhianna Wilson spent her senior year of high school in and out of four San Diego hospitals, seeking relief for her vision loss, pain and debilitating headaches.

At age 18, she was diagnosed with Ehler-Danlos Syndrome (EDS), a rare genetic disorder marked by overly stretchy connective tissue. In Wilson’s case, the disorder revealed itself in double-jointedness that led to four dislocations of her shoulders.

An MRI also showed a small slippage of the brain, an abnormality called a Chiari malformation, Her doctors considered it too minor to be the source of her worsening vision loss, pain and headaches.

“I couldn’t see and couldn’t drive,” said Wilson, 20. “I lost the use of my legs. But because doctors couldn’t see my pain, to them it didn’t exist.”

Her physicians in San Diego where she was being treated encouraged Wilson’s parents to consider a seeing-eye dog and to enroll her in a school for the blind.

Refusing to give up, her mother scoured the internet for a possible link between EDS and Chiari. Her research led the family to pediatric neurosurgeon Dr. Aria Fallah at UCLA Mattel Children’s Hospital.

“Rhianna was absolutely debilitated by these problems,” said Fallah. “She had gone from doctor to doctor and hospital to hospital with essentially normal-looking MRIs. So they’d been quite dismissive of her symptoms.”

Fallah referred Wilson for an unconventional stand-up MRI: the scan revealed a diagnosis that previous doctors had missed. A large Chiari malformation—not visible on a traditional reclining scan– was compressing Rhianna’s brainstem and spinal cord, causing her excruciating pain and lost function.

Fallah removed Wilson’s top vertebra and the back bone of her skull, creating more space for her brain and relieving her symptoms.

When Wilson opened her eyes after surgery, she could see again.

In less than three weeks, she was living like a typical teenager: driving, working, hiking and enjoying the active social life she’d missed during her senior year.

“UCLA has experts in these rare conditions, which gives us a unique advantage in treating the most complex of disorders and really making a difference,” said Fallah.

Now 20, Wilson is reclaiming her life and making up for lost time. For more details, read The New York Times Magazine “Diagnosis” column on her case.

Jun 5, 2018 · Early detection of rare eye cancer is important

Ocular melanoma is a rare form of eye cancer. Each year, only 2,500 people –or five in 1 million– are diagnosed. So why, as the news media reports, are a baffling number of newly diagnosed ocular melanoma cases cropping up in the southern United States?

To shed light on the issue, we consulted Dr. Tara McCannel, director of the ophthalmic oncology center at UCLA’s Stein Eye Institute and Doheny Eye Institute, and one of the nation’s leading experts in ocular melanoma.

Q. What is ocular melanoma?

A. McCannel: Ocular melanoma is a tumor that arises in the pigmented cells under the retina of the eye. We don’t know exactly what causes it. When detected early, the cancer is treatable and a person’s prognosis for vision and survival remains good. When the tumor is large or detected at an advanced stage, patients face a higher risk of metastasis and losing their eye. If the cancer spreads to the liver or lungs, the five-year survival rate drops to 15 percent.

Q. What could explain the rise of new cases in Alabama and North Carolina?

A. McCannel:  Many of the newly diagnosed patients have lived at some point in the same geographic regions.  Also, most of these patients are far younger than 55–the average age of diagnosis for ocular melanoma. These factors suggest that the patients may be remotely related and have inherited a genetic mutation that predisposes them to the disease.

Here’s why: scientists have identified a gene mutation on chromosome 3 of the ocular melanoma cell.  Normally, this gene suppresses tumor growth. In rare cases, some patients may inherit this mutated gene and be predisposed to ocular melanoma and other cancers. Doctors must inform their patients that genetic testing is available and alert patients and their families to their cancer risk.

Q.  How is ocular melanoma treated?

A. McCannel: Radiation is the best way to treat eye cancer. At UCLA, we stitch a gold plaque to the outside of the eye, and remove the plaque seven days later. The plaque contains tiny radioactive seeds that kill the cancer cells. During this time, patients must keep family members at a distance and avoid crowds. Once the plaque is removed, the patient is considered radiation-free and may return to normal activities.

The downside to radiation is that it can injure the optic nerve and retina, potentially leading to future blindness. My lab has pioneered a method to shield the eye during radiation and protect patients’ vision.

Q.  How does your research influence how you treat your patients?

A. McCannel: At UCLA, we treat ocular melanoma in an entirely novel way, by including vision-saving strategies in our approach to destroying the cancer. In 2012, we discovered that placing silicone oil in the eye during treatment reduces the amount of radiation that reaches the retina. Our research found that patients treated this way enjoy better vision, healthier eye tissue and fewer side effects after radiation therapy compared to patients we treated without silicone oil.

Q. What should patients know about their options for treatment?

A. McCannel: Seek care from a leading academic medical center. Surgeons who treat melanoma at these centers must undergo special training to treat ocular melanoma and use the most advanced technology and clinical approaches.

Equally important, choose a physician whose practice is primarily devoted to ocular melanoma– both treating the primary tumor and addressing the potential vision loss. A university teaching hospital is best equipped to bring together a team of cancer experts and to participate in clinical trials that translate the latest findings into new therapies.

Dec 20, 2017 · A cup of hot tea a day could keep glaucoma away

Sipping a mug of piping hot tea on a cold day doesn’t just warm you up—it may also protect your vision.

That’s the good news from a new UCLA study, which found a daily cup of hot tea may reduce your risk by up to 74 percent of developing glaucoma, one of the leading causes of blindness worldwide.

Darjeeling not your cup of tea? Too bad. Drinking decaffeinated tea, regular or decaf coffee, iced tea and sodas appeared to make no difference to glaucoma risk. The British Journal of Ophthalmology published the findings.

Glaucoma develops when fluid build-up increases pressure inside the eye. Without treatment, the rise in pressure damages the optic nerve and eventually leads to permanent blindness. Already affecting 57.5 million people worldwide, the number of people with the disease is predicted to reach 65.5 million by 2020.

“Earlier research suggests that caffeine can influence pressure inside the eye. Our study, however, is the first to look at the impact of decaffeinated and caffeinated drinks on glaucoma risk,” said lead author Dr. Anne Coleman, the Fran and Ray Stark Professor of Ophthalmology at UCLA’s Stein Eye Institute and David Geffen School of Medicine.

Coleman and colleagues examined 2005-2006 survey data collected by the U.S. Centers for Disease Control and Prevention. Tests detected glaucoma in 5 percent of the 1,678 people who underwent eye exams as part of the survey.

Survey participants answered questions about how often they drank caffeinated and decaffeinated beverages and in what amounts over the past 12 months.

People who enjoyed a daily cuppa hot tea were less likely to have glaucoma than those who were tea-teetotalers, the data found, even after controlling for variables like smoking, diabetes, age, body mass index, ethnicity and gender.

Latte lovers were out of luck. No similar risk reduction was found in people who regularly consumed regular or decaf coffee, decaffeinated tea, iced tea or sodas.

Flickr/Luxbao

A few caveats before you throw a tea party:

  • The study observed a correlation only; researchers did not draw firm conclusions about cause and effect.
  • A limited number of participants had glaucoma, and the timing of their diagnosis was unknown.
  • The survey did not record details like cup size, tea type or brewing time, all of which may have influenced the findings.
  • Drinking hot caffeinated tea may be associated with other lifestyle factors that underlie the reduced risk.

“We know that tea contains antioxidants and chemicals that help fight inflammation while protecting the brain,” said Coleman, who is also a professor of epidemiology at the UCLA Fielding School of Public Health. “Previous studies suggest oxidation and neurodegeneration may pave the way for glaucoma to develop.”

Antioxidants and inflammation-fighting chemicals are also associated with a lower risk for serious health conditions like heart disease, cancer and diabetes.

“Our next step will be to determine whether drinking hot tea could play a role in preventing glaucoma and uncover the mechanisms that protect the eyes from increased pressure,” Coleman said.

For persons with a family history of glaucoma, however, there’s no harm in playing it safe. Treat yourself to a plate of crumpets, follow Captain Picard’s example and order “Tea, Earl Grey, hot.”

Dec 5, 2017 · Got bedroom eyes? You may qualify for corrective surgery

Lots of people have bedroom eyes – a lazy, heavy-lidded gaze that conjures up seduction. But if you’re 50 or older and your lids remain half-closed when you open your eyes, you may be a candidate for surgery to correct ptosis (pron: toe-sis), or droopy upper eyelids.

Insurance often covers some of the cost of the procedure if the sagging of your eyelids significantly obstructs your vision.

At the UCLA Stein Eye Institute, surgeons evaluate every week about 10 patients with drooping eyelids, making ptosis one of the most common conditions that eye plastic surgeons treat.

“Ptosis happens gradually as we age,” explained Dr. Robert Goldberg, the institute’s chief of orbital and ophthalmic plastic surgery and Dabby Professor of Ophthalmology at the David Geffen School of Medicine at UCLA. “The condition most often results from stretching of the tendon that lifts the eyelid and keeps it open.”

The condition often sneaks up on people, who unconsciously find themselves compensating by raising their eyebrows or dropping their chins to lengthen their fields of vision.  Ptosis patients often have tell-tale lines spreading horizontally across their foreheads as a result of all the eyebrow activity.

Corrective surgery is performed under local anesthetic in an outpatient setting, and requires about 30 minutes per eye, said Goldberg.

“We enter through the front or the back of the eyelid  and tighten the tendon, much like hemming a pair of pants,” he said.  “Excess skin also sometimes removed from the eyelid during the same procedure. Cosmetic work is not covered by insurance, he noted.

Doctors diagnose patients by examining and measuring the eye and testing eyelid muscle strength.  If the eyelid covers so much of the eye that vision is blocked, surgery is considered medically necessary by some insurers.

“In some cases, ptosis interferes with a person’s ability to read or drive,” said Goldberg.  “That’s when surgery can really enhance a patient’s quality of life.”

The average patient is 65 or older, and the condition is more common in contact-lens wearers.  The reason for the latter is unclear, however, the lens-wearers’ daily manipulation of the eyelid to remove contacts may contribute to the problem.

People who suffer from dry eyes are typically not candidates for the procedure, which can exacerbate the condition.

“Dry eyes can be a side effect of the surgery,” explained Goldberg. “When the lid remains open, it exposes more of the eye to air and allows the tear film to evaporate. Mild cases can be resolved by the regular use of artificial tears.”

Patients can expect some bruising and swelling for seven to 10 days after the procedure, and are usually able to drive again after one or two days.  Contact lenses can usually be tolerated a week after surgery.

For more information about ptosis surgery, please call the orbital and ophthalmic plastic surgery division at the UCLA Stein Eye Institute at (310) 206-8250.

Nov 17, 2017 · Considering an all-nighter? Pity your poor brain cells and skip it.

Pulling an all-nighter can do a number on your brain cells.

People get too little sleep for lots of reasons. But whether you’re binge-watching Game of Thrones or nursing a colicky baby, the results are the same: you’re really tired the next day.

Now a new UCLA study shows that your brain cells get sleepy, too. And that can lead to some serious spacing out… with potentially dangerous implications.

“We discovered that starving the body of sleep also robs neurons of the ability to communicate with each other,” said senior author Dr. Itzhak Fried, a professor of neurosurgery at the David Geffen School of Medicine at UCLA and Tel Aviv University. “This leads to mental lapses in how we perceive and react to the world around us.”

Fried and his colleagues studied 12 people with epilepsy who had electrodes implanted in their brains in order to pinpoint the origin of their seizures before surgery. Because lack of sleep can provoke seizures, some patients stayed awake all night to speed the onset of an epileptic episode.

The team asked participants to quickly categorize a variety of images while electrodes recorded each patient’s neurons firing. Researchers paid particular attention to cells in the temporal lobe, which controls visual perception and memory.

Performing the task grew more challenging as the patients grew sleepier. As the patients slowed down, their brain cells did, too.

Sleep deprivation caused the bursts of electrical activity that brain cells use to communicate to become slower and weaker, reported National Public Radio. This sabotaged the brain’s ability to encode information and translate visual input into conscious thought.

Fried offered the examples of a pedestrian stepping in front of a sleep-deprived driver’s car.

“The very act of seeing the pedestrian slows down in the driver’s overtired brain,” Fried said. “It takes longer for his brain to register what he perceives.”

Equally alarming, noted NBC News, the scientists discovered that slower brain waves – the kind that accompany sleep – washed across key parts of the brain even when the volunteers remained awake.

“This phenomenon suggests that select regions of the patients’ brains were dozing, causing mental lapses, while the rest of the brain was awake and running as usual,” said Fried.

So if we force ourselves to stay awake, Quartz observed, individual cells might try to sneak in a catnap.

It might be time to rethink how we view sleep deprivation, which can have a similar effect on the brain to alcohol intoxication, reported Newsweek.

“Severe fatigue exerts a similar influence on the brain to drinking too much,” Fried said. “Yet no legal or medical standards exist for identifying overtired drivers on the road the same way we target drunk drivers.”

Nov 2, 2016 · Hot news flash! Menopause speeds up aging.

Woman with fanAs if the hot flashes, night sweats and dry skin caused by menopause weren’t indignity enough, a new UCLA study has found that the change of life also accelerates the body’s biological clock, increasing women’s risk for aging-related diseases and earlier death.

Published July 25 in the Proceedings of the National Academy of Sciences, the results could rekindle a national debate about the merits of hormone therapy, particularly for women who enter menopause early following surgical removal of the ovaries.

“For decades, scientists have disagreed over whether menopause causes aging or aging causes menopause,” said principal investigator Steve Horvath, a professor of human genetics and biostatistics at the David Geffen School of Medicine at UCLA and UCLA Fielding School of Public Health. “It’s like the chicken or the egg: which came first?  Our study is the first to demonstrate that menopause makes you age faster.”

Using an epigenetic clock Horvath invented in 2013, he and first author Morgan Levine tracked methylation, a chemical biomarker linked to aging, to analyze DNA samples from more than 3,100 women enrolled in four large studies. In 2010, data from one of these studies was used to confirm the controversial finding linking hormone therapy to a higher risk of cardiovascular disease in menopausal women.

In the UCLA study, researchers measured the biological age of cells from blood, saliva and inside the cheek to explore the relationship between each woman’s chronological age and her body’s biological age.

“We discovered that menopause speeds up cellular aging by an average of 6 percent,” said Horvath. “That doesn’t sound like much but it adds up over a woman’s lifespan.”

Take, for example, a woman who enters early menopause at age 42.  Eight years later, her body would be a full year older biologically than another 50-year-old woman who entered menopause naturally at age 50.

On average, a woman who had both ovaries removed at 35 is one and a half biological years older than a pre-menopausal woman of the same age.

“The younger a woman is when she enters menopause, the faster her blood ages,” said Levine, a postdoctoral fellow in Horvath’s lab. “That’s significant because a person’s blood may mirror what’s happening in other parts of the body, which could have implications for death and disease risk.”

The UCLA finding echoed earlier studies showing that women who live past 100 tend to enter menopause later in life.

“Young women possess a strong health advantage over men the same age,” said Levine. “That benefit disappears at menopause, when declining sex hormones stop protecting women from cardiovascular diseases and many age-related illnesses.”

“Our results suggest that female sex hormones like estrogen and progesterone may influence aging and longevity,” said Horvath. Nude middle-aged woman

The coauthors propose that scientists in the future may use the epigenetic clock as a diagnostic tool to evaluate the effects of therapies, like hormone therapy for menopause. Researchers will try to answer the question of which menopausal hormone therapy offers the strongest anti-aging effect while limiting health risks, said Horvath.

“No longer will researchers need to follow patients for years to track their health and occurrence of diseases.  Instead we can use the epigenetic clock to monitor their cells’ aging rate and to evaluate which therapies slow the biological aging process,” said Horvath. “This could greatly reduce the length and costs of clinical trials and speed benefits to women.

The UCLA scientists next plan to study the effects of menopause and hormone therapy in fat, skin, vaginal and breast tissue, and to carry out long-term studies of biological aging in the same women before and after menopause.

Aug 2, 2016 · 5 tips for soothing dry eyes

5 Tips for Soothing Dry Eyes
Having trouble reading your smartphone or wearing your contacts for as long as usual?  Air conditioning, blustery fans and summer heat all can dry out your eyes. Dr. John Bartlett, medical director of University Ophthalmology Associates at the Stein Eye Institute at UCLA, offers five tips for keeping your eyes more comfortable.
  1.  Avoid red-eye rebound. Rely on over-the-counter products, like artificial tears or ointment, every four to six hours to lubricate your eyes and ease dryness. But shun drops that claim to relieve redness.  They cause a rebound effect that leads to increased redness and irritation over time.
  2. Consider the environment. Don’t point fans or air-conditioning vents toward your face while driving or sleeping. Angle the gusts toward your body instead to preserve natural tears.
  3. Monitor your computer use. Staring at the screen reduces blinking and revs up tear evaporation. Think 20/20/20: take a 20-second break every 20 minutes and focus on something 20 feet away.
  4. Try oil and heat. Consuming Omega-3 fatty acids, found in fish or flax-seed oil, helps some patients improve their tear film and reduce evaporation.  Hot compresses can also help lubricate the eyes.
  5. See a professional. If these simple solutions don’t bring relief, see your eye doctor. He or she can evaluate whether you’d benefit from prescription medications or other procedures.