Foreign Accent Syndrome

“Foreign Accent Syndrome” (FAS) is a rare disorder in which patients start to speak with a foreign or regional tone. This striking condition is often associated with brain damage, such as stroke. Presumably, the lesion affects the neural pathways by which the brain controls the tongue and vocal cords, thus producing a strange sounding speech.

Yet there may be more to FAS than meets the eye (or ear). According to a new paper in the Journal of Neurology, Neurosurgery and Psychiatry, many or even most cases of FAS are ‘functional’, meaning that the cause of the symptoms lies in psychological processes rather than a brain lesion.

To reach this conclusion, authors Laura McWhirter and colleagues recruited 49 self-described FAS suffers from two online communities to participate in a study. All were English-speaking. The most common reported foreign accents were Italian (12 cases), Eastern European (11), French (8) and German (7), but more obscure accents were also reported including Dutch, Nigerian, and Croatian.

Participants submitted a recording of their voice for assessment by speech experts, as well as answering questions about their symptoms, other health conditions, and personal situation. McWhirter et al. classified 35 of the 49 patients (71%) as having ‘probably functional’ FAS, while only 10/49 (20%) were said to probably have a neurological basis, with the rest unclear.

These classifications are somewhat subjective in that there are no hard-and-fast criteria for functional FAS. None of the ‘functional’ cases reported hard evidence of neurological damage from a brain scan, but only 50% of the ‘neurological’ cases did report such evidence. The presence of other ‘functional’ symptoms such as irritable bowel syndrome (IBS) was higher in the ‘functional’ group.

In terms of the characteristics of the foreign accents, patients with a presumed functional origin often presented with speech patterns that showed inconsistency or variability. For instance, pronouncing ‘cookie jar’ as ‘tutty dar’ but being able to correctly produce ‘j’, /k/, /g/ and ‘sh’ sounds as part of other words.

But if FAS is often a psychological disorder, what is the psychology behind it? McWhirtner et al. don’t get into this, but it is interesting to note that FAS is often a media-friendly condition. In recent years there have been many news stories dedicated to individual FAS cases. To take just three:

American beauty queen with Foreign Accent Syndrome sounds IRISH, AUSTRALIAN and BRITISH

Scouse mum regains speech after stroke – but is shocked when her accent turns Russian

Traumatic car accident victim has Irish accent after suffering severe brain injury

Woman suffers acute reverse-slope hearing loss (RSHL) and becomes unable to hear male voices

By Fiza Pirani,

A woman in Xiamen, China, unexpectedly developed an ear condition that left her unable to hear male voices, the Daily Mail recently reported citing local AsiaWire reports.

The woman, who has only been identified by her last name (Chen), said she realized something was wrong when she woke up and couldn’t hear her boyfriend’s voice. Before going to bed, she said she heard ringing in her ears and vomited.

When she made it to Qianpu Hospital, doctors diagnosed Chen with reverse-slope hearing loss (RSHL), a rare condition in which lower frequencies become difficult to hear. It’s named for the shape it produces in visualizations — “the graph starts in the lower-left-hand corner and slopes upward steeply,” according to Georgia audiology clinic, Audiology HEARS, P.C.

Chen “was able to hear me when I spoke to her,” treating provider Dr. Lin Xiaoqing, a woman, told the Daily Mail. “But when a young male patient walked in, she couldn’t hear him at all.”

Xiaoqing told local media she believes fatigue and stress played a role in Chen’s condition and expects her patient will make a full recovery.

When humans hear sounds, the tiny hairs inside the ear vibrate. But genetic conditions, injuries or types of drug use may make the hairs “brittle and prone to breakage,” affecting one’s ability to hear higher-pitched sounds, Dr. Michelle Kraskin, an audiologist at New York-Presbyterian Hospital who was not involved in Chen’s case, told Live Science. But hearing loss of low-pitched sounds like Chen experienced is less common because the ear’s cochlea, responsible for the lower frequencies, is usually highly protected.

In fact, RSHL only affects an estimated 3,000 people in the United States and Canada. For every 12,000 people with some type of hearing loss, Audiology HEARS states on its website, only one individual has RSHL.

It’s most often caused by genetics, and many people with the condition might not even know they have it. Those with Wolfram syndrome, Mondini dysplasia and inheritance through a dominant gene are at increased risk, according to the clinic.

Other causes of RSHL may include diseases like sudden hearing loss, viral infections or Ménière’s disease, all of which affect the hair cells. Autoimmune disorders that affect the inner ear, also rare, are another potential source. These conditions could also lead to dizziness, nausea and vomiting.

Any procedures or conditions that cause a change in pressure of inner ear fluid (or the endolymph) may also cause RSHL. These conditions include spinal or general anesthesia, intracranial hypertension or a perilymphatic fistula.

Some symptoms of RSHL may include difficulty comprehending speech over phones, which largely deliver low and middle frequencies; an inability to hear low-frequency sounds like a running refrigerator or thunder and, as Chen displayed, a difficulty hearing male voices compared to higher-frequency speech of women and children.

It’s best to catch the condition within 48 hours for the best chance of recovery, Kraskin said. Once diagnosed, treatment may involve high doses of steroids.

Though RSHL may go away without any treatment at all, the condition can potentially worsen and become problematic in terms of safety.

“If you can’t hear a car coming, you can’t avoid it. If someone some distance from you is trying to warn you away from something, you might not hear it, because volume is a product of the lower frequencies,” according to Audiology HEARS.

Because general industry standards cater to high-frequency hearing loss, which is much more common, treating worsening RSHL can be quite difficult. Audiologists are encouraged to listen to the patient’s concerns and customize hearing aids and should take the time to determine “channel by channel, frequency by frequency” what patients finds “comfortable, audible, and helpful.”

Approximately 25 percent of people in the United States between ages 55 and 64 have some degree of hearing loss, according to the Mayo Clinic. It becomes increasingly common as you age. In fact, hearing loss affects 1 in 2 people older than age 65. Anyone who experiences sudden hearing loss, particularly in one ear, should seek medical attention immediately.

Migraine headaches in the NFL

Jets linebacker Lorenzo Mauldin (55) wears special contact lenses and a helmet shade to prevent migraines.


FWhen he woke last Sunday morning, Jeremy Kerley sensed trouble already coming on. Fitful sleep is often his trigger, he said. The migraine eventually hit him like an anvil late in last week’s game against the Giants.

His eyes grew blurry and he felt what he described as a “sharp, shooting, throbbing pain.” He wanted to sit down. He wanted to lie down. He knew he needed to leave the field.

Kerley, the Jets’ punt returner, departed to the locker room and did not return. As the Jets came from behind to beat the Giants in overtime, he was receiving intravenous fluids and oxygen to help relieve the anguish from a struggle that has afflicted him since high school.

For Kerley, migraines are the silent menace that constantly lurks. They ambush him almost once a month, even though he rarely talks about it. He knew his grandfather got them; only recently, he discovered that his dad did, too. He just never knows when they will affect him.

Though Kerley is one of approximately 38 million Americans who suffer from them, migraines are not something that is openly discussed in N.F.L. locker rooms. They are far more common in women, and often minimized as simply a headache, a stigma that Kerley acknowledged could make it difficult to pull himself out of a game.

But those who do struggle with migraines — which the Migraine Research Foundation considers a neurological disease, like epilepsy — understand the plight. When Kerley felt a severe headache coming on last season after a game at Minnesota, his teammate Percy Harvin patted him on the back.

“I know how you feel,” Harvin said quietly. He has struggled with migraines throughout his career.

Kerley did the same thing earlier this season, after linebacker Lorenzo Mauldin revealed that he had had migraines since adolescence. Kerley gave him recommendations about nutritional supplements that he found helpful, like fish oil and magnesium. Mauldin also now takes prescription medication to both relieve and prevent severe headaches.

He said that light could often trigger his migraine episodes, so Mauldin wears special contact lenses and a protective shade on his helmet.

“It hurts because it’s pulsating and you can’t really stop it,” Mauldin said. “With a bruise or something, you can put alcohol or peroxide over it and it’ll be fine. Or if you’ve hurt a muscle, you can ice it. But you can’t put ice over a migraine.”

In September, a migraine forced Ohio State quarterback Cardale Jones to the emergency room, something that is not uncommon, said Dr. Melissa Leber, the director of emergency department sports medicine at the Icahn School of Medicine at Mount Sinai. She typically treats patients intravenously. But that often cannot relieve the crippling symptoms right away.

“Some people can’t even get out of bed,” Leber said. “Others can function just while not feeling well. It really runs the gamut for how debilitating it can be.”

Migraines are thought to be related to the brain’s trigeminal nerve, which can grow hypersensitive and cause pain signals to fire throughout the brain, typically concentrated around the eyes or temples. Though migraines are strongly hereditary, showing up in people who have had no sports history, they are often clinically similar to post-traumatic headaches, like the headaches that arise after a concussion, according to Dr. Tad Seifert, a neurologist at Norton Healthcare in Louisville, Ky.

During the summer, Seifert led a study of 74 high school football players in the Louisville area and found that 33.8 percent of them suffered from migraines, a rate twice that of the normal population. The rate rose to 37.5 percent in players who reported having sustained a concussion once in their lives, and 40.7 percent in those who reported multiple concussions.

“The elephant in the room is whether there is some influence of contact sports and the development of frequent or chronic headache later in life,” Seifert said. “And if so, how much?”

Seifert, who also chairs an N.C.A.A. task force on headaches, said that he expected to publish a similar report involving 834 Division I athletes in the spring. Though he would not go into detail about the results, he said that it looked to be “very similar to what we’ve found in this sample of high school players.” Mauldin, it should be noted, sustained a concussion earlier this season.

There is no cure for migraines, and sufferers often go the rest of their lives “controlling” the issue, Seifert said, comparing it to those dealing with high blood pressure or diabetes. What concerns him, though, are the studies that have shown that people with migraines are more susceptible to concussions, and when they do sustain one, it takes them longer to recover.

“We know that the migraine brain is just wired differently,” Seifert said. “And we know that it’s a brain that is hypersensitive to external injury. And those pain receptors that are in overdrive — it takes that much longer to calm down and return to baseline.”

In the time it takes for the receptors to settle, though, the pain can bring a linebacker to his knees.

“When they pop up out of nowhere, you start to feel a sensation like in between the middle of your forehead,” Mauldin said. “But it’s in the back of your head as well. It’s like somebody’s punching you in the side of the head.”

Kerley said he had yet to receive a migraine disease diagnosis, but he thinks it could be related to difficulties he regularly has with sleeping, being someone who has sleep apnea. When he feels a headache coming on, he has a nasal spray that he said often cured his symptoms within a half-hour. But last Sunday, it was too late.

“If you don’t catch it while it’s early, it could get pretty bad,” Kerley said. “Mine got there.”