By Kavita Varma-White

At the age of 5, my son Jayan had a routine set of dental X-rays that showed a disturbing fact.

The X-rays revealed that he possessed not one, not two, but three sets of front teeth. There were his baby teeth, his permanent teeth and in between, an extra set. A bonus pair, if you will.

I reacted the way any parent would upon discovering their child has an extra body part:
I freaked. "He has what?!" I yelled at my husband, who had taken him to the appointment. (Having a general fear of dentistry, I avoid going whenever possible.)

Humans are normally born with 20 baby teeth and have 32 permanent teeth. As it turns out, Jayan is the proud owner of supernumerary teeth, which are teeth additional to the regular number of chompers and can be found in almost any region of the horseshoe-shaped dental arch. They are most common in the central incisors, or front teeth.

Supernumerary teeth are often hereditary, although pediatric dentist Patrick Arnold of Fort Lauderdale, Fla., says it's hard to tell what causes them. "A portion of the developing tissue from early on may get pushed off and a tooth bud might split. Or, there also might be hyperactivity of the dental lamina, which is the precursor to a tooth."

It's also unclear how common supernumerary teeth are in children. The American Academy of Pediatric Dentistry cites a 1999 report in the Journal of Canadian Dental Association, where in a survey of 2,000 British schoolchildren, researchers found that supernumerary teeth were present in 0.8 percent of kids who still had baby teeth. Of the children who already had their permanent teeth, 2.1 percent had supernumeraries.

Arnold, the dentist who diagnosed our son, has not seen a lot of supernumerary teeth in his eight-year practice. He estimates about 1 percent of his patients are affected.

After discovering Jayan's sharklike extra row of teeth, the dentist explained the likely course of treatment.

First, Jayan's baby front teeth would have to be extracted prematurely. Second, we'd have to wait for the supernumerary teeth to come in. Hopefully, they would appear before the permanent teeth. (An unappealing side effect: When the supernumerary comes in, it is commonly not a fully formed tooth, but rather a malformed, peglike tooth.) Once his ivories presented themselves, the supernumerary teeth would have to be pulled, making room for the permanent teeth.

If the permanent teeth edged ahead of the supernumeraries, Jayan would face a miserable operation of oral maxillofacial surgery to remove the extra teeth.

Fortunately, fate stepped in. A few months after the diagnosis, Jayan had a playground collision that loosened both of his front teeth. That afternoon, with my husband inconveniently out of town, I watched Arnold stick a huge needle in my son's mouth to numb it, and then pull out both teeth.

It is truly amazing how losing the front teeth completely transforms a child's face. The babyness leaves and they are instantly … older. I cried at the sight of my toothless boy, who I felt had to unfairly grow up too fast.

Meanwhile, Jayan, 5, enjoyed celebrity status in his kindergarten class, as no one had lost both front teeth yet. Most kids don't lose their first tooth until age 6; they commonly don't lose both front teeth before age 7.

It also helped that the Tooth Fairy, who is a sucker for dentist-extracted teeth, left $10 under the pillow, rather than her usual fare of $1 per tooth.

Shortly afterward, we moved to a new city and took Jayan to a new dentist. Fresh X-rays showed in the past year the supernumerary teeth had made significant movement. While one is coming down in the correct position, the other one seems to be pointing backwards, possibly requiring surgery. Only time will tell which direction it takes.

As we play the waiting game, we'll celebrate yet another holiday season where the theme song in our house is the familiar tune, "All I Want for Christmas Is My Two Front Teeth."

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By Brian Alexander

For as long as she could remember a 60-year-old British woman, known only as KH, has been unable to recognize voices, not even the voice of her own daughter. Unless she sees the face of the person speaking, she often has no idea who is talking to her. If her daughter calls on the phone, or an unseen colleague from work says something to her, it's as if she's hearing the voice for the first time.

Except when Sean Connery speaks.

KH didn't know what caused her problem until a few years ago when she read a magazine article about a neurological defect which makes it extremely difficult for people to recognize faces — a condition called prosopagnosia, or face blindness. She wondered if there could be some connection to her experiences. Hoping the doctors might solve her own mystery, she contacted prosopagnosia researcher Dr. Brad Duchaine at University College London.

"She thought she had a vocal [version of] prosopagnosia," Duchaine said in an interview. "But we had never done anything involving voices. So we ran her through some face tests, some voice tests, and we could see she was on the level."

An MRI showed no obvious structural defects or injuries. So Duchaine and colleague Lucia Garrido of UCL's Institute of Cognitive Neuroscience created a series of more complex tasks to more thoroughly test KH's ability to recognize faces, voices, emotions, music and overall perception of speech.

Duchaine and Garrido exposed KH to the voices of famous people with distinctive voices. Actress Joanna Lumley (known best in the U.S. for her role in the British comedy series "Absolutely Fabulous"), former British prime minister Margaret Thatcher, and David Beckham were easily tagged by a group of people participating in the research, although none of them rang a bell for KH.

Only one of the voices perked up her ears with recognition: the unmistakable Scottish burr of actor Sean Connery, the original James Bond.

"His accent is distinctive," Duchaine explained. "And she is a British woman in her sixties…let's say it's probable he got her attention."

Eventually Duchaine and his colleagues realized KH was the first documented case of someone born without the ability to detect familiar voices. Doctors do sometimes see this condition called phonagnosia typically in the aftermath of a stroke when brain damage affects auditory perception. Developmental phonagnosia, however, is an inborn defect whose cause is still unknown.

To learn more about the condition, Duchaine is conducting further research with KH. The scientists will use specialized scans to compare her brain while listening to voices with those of normal subjects to see which areas of brain are stimulated -- or not, in her case. But based on his work with prosopagnosia, in which few, if any, differences can be seen, he doubts his team will find much.

As for KH, her neurological defect has not prevented her from leading a highly successful professional life, although she has had to adopt several coping mechanisms.

For example, she must make rigid appointments to receive telephone calls so that if the phone rings at, say, 7 p.m., she knows who will be on the other end. And there have been embarrassing social encounters, like the time a former boss spoke to her while standing behind a couch where KH was sitting. Because she didn't turn to acknowledge him, he thought he was being snubbed.

Duchaine has issued a call for any others who think they might be phonagnosic and invites them to contact him through a Web site, www.faceblind.org. So far, he says, five or six people have called. He expects to finds that developmental phonagnosia may not be all that uncommon. In comparison, developmental face blindness appeared to be very rare ten years ago, but it's now known that many people cope with it.

"We hope that studies with KH's condition will help us better understand a range of issues related to voice recognition," says Duchaine. "Developmental prosopagnosia has helped us understand the cognitive, neural, and genetic basis of face recognition, and voice agnosia could do the same for voice recognition."

The research could help not only people like KH, but others who have problems understanding voices due to other kinds of developmental issues or brain damage.

More on neuroscience  |  Sean Connery

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by Diane Mapes

A six-fingered spy seems a natural fit for a James Bond film, right in keeping with other odd-bodied henchmen such as the metal-mouthed giant Jaws or Tee Hee, the assassin with a hook for a hand. 

But the supernumerary appearing in the latest 007 adventure, "Quantum of Solace," is no quirky Bond villain. She's the new Bond beauty, Gemma Arterton, a 22-year-old British actress who was born with six fingers on each hand, a condition known as polydactyly.

Polydactyly, which runs in families, occurs in approximately 1 in 1,000 children and can involve either multiple toes or multiple fingers —everything from small skin buds next to the pinkie to two fingernails on one finger to fully functioning extra digits to fingers or thumbs split into a Y shape.

According to Dr. Terry Light, a hand surgeon and chairman of the department of orthopedic surgery and rehabilitation at Loyola University Medical Center in Illinois, polydactyly and syndactyly, the webbing or fusing of fingers and toes, are the two most common congenital hand anomalies seen in the U.S.

"The limb bud starts off as a glob – like a paddle – that normally separates into five distinct rays or digits," says Dr. Light. "But if that process of separation goes a step too far, then it separates into more packets, or one of the digits, such as the thumb, becomes separated yet again."

In Arterton's case, her extra digits weren't fully formed fingers but rather soft bits of floppy tissue without any bone. They were removed when she was a child using a technique known as "tying." Small scars still remain.

"The pediatrician will tie a little string around [the extra digit], almost the way you would an umbilical cord," says Dr. Light. "It will wilt away and fall off in the first couple of days of life."

In other cases, though, the surgery can be much more complicated.

In many forms of polydactyly, the condition is not simply an extra digit but an abnormal formation of several fingers. "When you have polydactyly of the thumb, the stuff that was going to make one thumb has been split into two packages and neither one is quite normal. If you remove one, the one that remains may be substantially inadequate." As a result, part of both digits are used in reconstruction.

While polydactyly is now accepted as a relatively common birth defect — Arterton told Esquire she's even a bit proud of her "little oddity" — people born with extra digits in the past didn't always fare so well.

Folk beliefs from Eastern Europe and Africa sometimes associated six-fingered children (or anyone with any kind of "unnatural" feature) with witchcraft, or pegged them as the "exchanged child" of a witch, swapped out in the middle of the night while the mother was asleep.  Even Anne Boleyn, wife of Henry VIII, was thought of as a witch thanks to the sixth finger rumored to be on her right hand (some believe she merely had an extra fingernail on one of her fingers).

While the witchcraft accusations (and the occasional fear-fueled murder of a polydactyl child) have faded away over the centuries, a six-fingered stigma does seem to remain in popular fiction. In the Thomas Harris crime novels, serial killer Hannibal Lecter bore a sixth finger on his left hand; Count Rugen, the villain who murdered Inigo Montoya's father in "The Princess Bride," had six fingers on his right hand.

Still it's not all bad news for polydactyls.

Six fingers are also said to a sign of good fortune or future prosperity. Sailors believe six-toed cats bring good luck. (The condition is also found in mice and chickens.) The association with good fortune, coupled with a lack of modern health care, may be one reason people outside the U.S. sometimes keep their extra fingers and toes.

"There are some cultures, such as in Latin America, where it's regarded as a sign of good luck," says Dr. Light. "Occasionally, I'll run into an adult who's retained an extra digit because it was thought to be a lucky omen. But usually in the U.S., most families would not see that as a positive."

Relief pitcher and polydactyl Antonio "Six Fingers" Alfonseca, who hails from the Dominican Republic, says he considers his extra fingers and toes a blessing.

"I think God gave me more fingers and toes because He wanted to show that I'm special and that I will be special someday," Alfonseca, also known as El Pulpo (The Octopus), told The Miami Herald in 2003.

Although Alfonseca's pitching is not affected by his sixth finger — it  doesn't actually touch the ball — he may be on to something.

The last team he pitched for, the Philadelphia Phillies, just won the World Series.

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By Mark Leyner and Dr. Billy Goldberg

Imagine an accidental bop on the head changed your accent from the grating stridence of Fran Drescher to the dulcet, euphonious tones of, say, Kate Winslet. Or if you're a man, what if a whack to the forehead transformed your speech from something out of Homer Simpson's pie-hole to the adorably urbane voice of Stewie Griffin from "Family Guy?"

That kind of bizarre voice change happened for real to a woman from Washington's Olympic Peninsula. Recent newspaper reports and a cable TV show featured CindyLou Romberg, who split her head from front to back after falling out of a moving car in 1981. Despite the serious brain injury, after her awful headaches and lingering back pain abated, she resumed a normal life as a caregiver and motorcycle enthusiast.

Until her back started bothering her again about a year ago.

After visiting a local chiropractor, Romberg soon began speaking gibberish.When she began speaking normally again, she had a German accent, tinged with what some friends thought was vaguely French or Russian. This strange accent was coming from an American woman who had never studied a foreign language, nor been to any foreign country, except Canada.

Romberg was diagnosed with Foreign Accent Syndrome, a neurologically-based speech disorder most probably caused by her traumatic brain injury. Although this particular diagnosis is rare — only 50 or 60 cases have been verified worldwide — other problems following traumatic brain injury, such as aphasia (the loss or impairment to use or comprehend words) or stroke, are not uncommon. The delay of so many years before the appearance of symptoms makes Romberg's case especially unusual.

We recognize that traumatic brain injuries can cause myriad personality changes in patients — everything  from paranoia, nihilistic delusions, lethargy, mania, impulsiveness, a tendency towards various kinds of disinhibited behavior like sexual promiscuity and frenetic gambling. The wife of a patient treated at the Johns Hopkins Brain Injury Clinic reported that after her husband eventually recovered from the brain injuries he suffered from a serious motorcycle accident, he became unusually cheerful, talking nonstop, and spending excessive amounts of time in karaoke bars. He was also uncharacteristically preoccupied with Internet pornography.

But there's something uniquely and irresistibly fanciful about the story of CindyLou Romberg. It has almost a fairly tale quality to it. Go to sleep as a normal American, wake up as an exotic European.

What really happened to her? According to a recent article in the Journal of Neurolinguistics, "foreign accent syndrome" is something of a misnomer. Following a stroke or severe brain injury, these patients don't actually manifest a speech pattern that corresponds to any particular language. What's going on is that they are displaying changes in the rhythms, stresses and intonations of their speech that listeners mistakenly ascribe to a new and different accent. Most cases of "foreign accent syndrome" are associated with injuries to the left hemisphere of the brain, which is associated with language.

Even the voices we hear in our heads after an accident can develop speech impairments. A medical journal recently reported a woman who, as the result of a brain injury from a bicycle accident, developed aphasia, rendering her able to only speak in very short sentences and single words. She also began having audio hallucinations of voices that shared her very speech problems. The voices she hallucinated expressed themselves in short sentences or single words.

Neuroscience is an endlessly fascinating field, and there is so much about the brain that we are only beginning to understand.  The neurological source of Peter Sellers' accent as Inspector Clouseau in the "Pink Panther" movies and, most spectacularly, Al Pacino's bizarre dialect as Tony Montana in "Scarface" ("Say hello to my little friend!") remain profound mysteries.

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