heart9H heart7Above is Frank’s Sign, but it has nothing to do with this week’s Hearing International.   This week’s discussion is not about any of the famous Franks that you know, such as “Ol’ Blue Eyes” Frank Sinatra, ”heavy metal Frank”,  Frank Zappa,heart4  ”designer Frank”,  Frank Lloyd Wright, or even ”Football Frank”, Frank Gifford.  heart6heart5

Our story this week is about an interesting portion of the auditory anatomy with which all audiologists are all familiar, the Auricle or Pinna.

Early Chinese references suggest that the ear is related to all parts of the human body and internal organs and that all meridians converge at the ear, specifically at the Auricle.  According to Chinese medicine references there are some 91 acupoints on the auricle, each relating to a part or organ of the body.  And, in their opinion, when changes are noted in the auricle they can signal the happenings in other parts of the body.  Similarly, when appropriate stimulation is presented to these 91 special areas of the auricle, changes may occur in those areas of the body sympathetic  to the stimulation.  The ear, as a diagnostic and treatment area, was first mentioned in the earliest Chinese medical book, Yellow Emperor’s Classics of Internal Medicine, published more than 2,000 years ago.  To the Chinese, the ear has a reflexive property where physical attributes may appear on the auricle when body disorders exist.

Fast Forward to 1973

 Since its first description by Sanders T. Frank, M.D.,  an American Pulmonologist, in the New England Journal of Medicine in 1973, the presence of the diagonal earlobe crease heart2(ELC) either unilaterally or bheart1ilaterally has been recognized as a possible marker of coronary artery disease (CAD).  Subsequent studies seem to confirm the ELC (or Frank’s Sign) as a predictor of CAD independent of age, cholesterol, blood pressure, or smoking status.  On the other hand, several studies found no correlation between ELC and CAD and suggest that it is simply a marker of advancing age. Such attributes include variations in shape, color, size, and sensation; appearance of papules, creases, and edema; and increased tenderness or decreased electrical conductivity.  Over 50 papers have been published regarding this physical diagnosis sign and, for almost four decades, controversy has raged over its utility. Is the ELC a clinically useful predictor of CAD?  Some studies have additionally linked Frank’s Sign to acquired hypertension, heart disease, and diabetes.

Subsequently, several studies have demonstrated similar associations, either in patients with CHD or in forensic autopsy cases. Other auricular signs associated with body functions also exist. For example, Evrungu (2004) in Dermatology reported that the presence of ELC has a high CHD predictive value.  While it seems too  simple to consider these earlobe creases, they probably should not be dismissed too lightly, as they fall into the surprisingly large category of “weird signs” that are possibly indicative of heart disease.  As shown in the pictures above, earlobe crease (ELC) is a line running diagonally from the bottom of the ear opening to the ear’s lower tip. The studies that discuss an ear hair correlation with heart disease are probably false, but the ELC or Franks Sign’s status as portent of doom has been suggested by a number of studies:

  • A Swedish study of 520 autopsies found ELC had a “positive predictive value” for coronary artery disease of 68 percent — 80 percent in those under 40.
  • A Turkish study found ELC was a higher risk factor for heart disease than diabetes, family history of cardiovascular trouble, or smoking.
  • Of 340 patients admitted to the Montreal Heart Institute, 91 percent of those with ELC had heart disease versus only 61 percent of those without.
  • An Irish study of 247 patients found ELC had a predictive value of 71 percent for heart disease, showing what statisticians call low sensitivity but high specificity.

While these and other studies seem to confirm ELC or “Franks Sign” as indicative of heart disease and other maladies, there a other studies that do not confirm this finding.  Since there are confliheart12cting studies regarding ELC or Frank’s Sign, most physicians place little stock in the Sign, but are careful NOT to ignore it.  While it could be bunk, there may be something to is presence.

And Just when we did not Believe…..

Publius, Aelius Hadrianus better known as Hadrian, Emperor of Rome (113-138, BC) traveler, warrior and lover of all things Greek, fell ill at age 60.  He developed progressive edema and episodic epistaxis, fell into a depression soothed by rich food and drink, and succumbed to death within 2 years. The exact cause of Hadrian’s death – whether by heart failure, glomerulonephritis, or even hereditary hemorrhagic telangiectasia – has been a topic of debate among paleopathologists.  It was not until 1980 that a crucial clue was found…… he was sculpted in stone busts with a deep diagonal crease memorialized in both earlobes….Hmmmm…..Could it have been heart disease?

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Last but NOT Least…..

Another famous person that has ELC is former US President George W. Bush.  The Time Magazine cover of Dec. 1, 2003 confirms an ear lobe crease on the right and  suggests the presence of hair in the ear canal (also a controversial sign to most physicians), but to some the combination of ear lobe crease and hair in the ear canal has been reported as being EVEN MORE predictive of coronary artery disease than either alone.   Suspect we can just wait that one out and see what happens!

 

polio8As part of the vaccination quandary fueled by inaccurate information, Polio has made a worldwide comeback affecting millions of individuals around the world.  Officially called poliomyelitis, or infantile paralysis, polio is an acute, viral, infectious disease spread from person to person, primarily via the fecal-oral route.

The Disease

The disease is an inflammation of the spinal cord’s gray matter, but a severe infection can extend into the brainstem and even higher structures, resulting in polioencephalitis, producing apnea that requires mechanical assistance to breathe. Although approximately 90% of polio infections cause no symptoms at all, affected individuals can exhibit a range of symptoms if the virus enters the blood stream. In about 1% of cases, the virus enters the central nervous system, preferentially infecting and destroying motor neurons, leading to muscle weakness and acute flaccid paralysis.

Various types of paralysis may occur, depending on the specific nerves involved. Spinal polio is the most common form, characterized by asymmetric paralysis that most often involves the legs.  Bulbar polio leads to weakness polio10of muscles innervated by cranial nerves. Bulbospinal polio is a combination of bulbar and spinal paralysis.

Poliomyelitis was first recognized as a distinct condition by Jakob Heine, a German Orthopedist, in 1840.  His findings were the first medical account of polio and, because of his work in explaining the disease it was acknowledged as a clinical entity. In 1890, Karl Oskar Medin distinguished and diagnosed  polio as an acute infection. The disorder was originally called Heine-Medin Disease for the two scientists.

A Swedish physician, Otto Ivar Wickman made a finding concerning the infectious and contagious nature of polio and published his hypothesis on the disease in Germany.  The Austrian physician Karl Landsteiner, along with Erwin Popper, discovered the poliovirus in 1909.

Polio Epidemics

 polio7Although major polio epidemics were unknown before the late 19th century, polio became one of the most dreaded childhood diseases of the 20th century. Polio epidemics have crippled thousands of people, mostly young children. However, the disease has caused paralysis and death for much of human history. It had existed quietly for thousands of years as an endemic pathogen until the 1880s when the major epidemics began to occur in Europe. Soon after that, widespread epidemics also appeared in the United States.

Polio was probably  feared most in the first half of the 20th century, when mass urbanization led to sporadic, localized epidemics in the US.  The first official epidemic was announced in New York in 1916, and in 1952 over 58,000 cases were reported, causing the deaths of 3,145 people and polio15leaving another 21,269 with paralysis that ranged from mild to disabling.

Thanks to the invention of vaccines, by 1988, the USA, Canada, the UK, and much of mainland Europe was polio free. But the story was very different in other regions. There were still 125 countries - including all of Africa and Asia - where polio was endemic and continued to be a threat. At its annual meeting, the World Health Assembly - the decision-making body of the WHO – voted to launch a global polio eradication initiative (GPEI) with a target date of 2000. That date  has now passed and polio is still found in some parts of the world, despite continuing efforts to end it.

Treatment

polio16Polio probably started B.C., as there are Egyptian hieroglyphs that show withered legs.  We can only guess at how they may have treated polio back then.  In the early 1900s people were being put in casts and on bed rest, which immobilized their limbs and caused them to wither.  In the 1920s Sister Kenny started treating polio victims, using warm, wet woolen blankets as hot packs and then slowly moving an affected limb so it would learn how to work after being paralyzed.  There was much controversy over her treatment, butpolio5 it was very successful in helping children walk again.

Generally, there was no real treatment for the disease until 1928 when the iron lung was invented to assist patients in their breathing. Before that, polio patients with breathing problem would usually die because their breathing muscles did not function. While the intimidating metal contraption was certainly a life saver, for those left with permanent paralysis, it was also a life sentence. The patient was permanently encased in the air-tight metal tank in order to force their weakened lung muscles to function.

In the 1950s, Dr. Jonas Salk, an American virologist, invented a vaccine that was successful.  Salk’s approach, first attempted unsuccessfully by Maurice Brodie, also an American, in the 1930s, was to kill several strains of the virus and then inject the benign viruses into a healthy person’s bloodstream. The person’s immune system would then create antibodies designed to protect the person in case of future exposure to poliomyelitis. Salk conducted the first human trials on former polio14polio patients and on himself and his family, and by 1953 was ready to announce his findings. This occurred on the CBS national radio network on March 25 and two days later in an article in the Journal of the American Medical Association. Dr. Salk became an immediate celebrity.  In 1954, clinical trials using the Salk vaccine and a placebo began on nearly two million American schoolchildren. In April 1955, it was announced that the vaccine was effective and safe, and a nationwide inoculation campaign began. New polio cases dropped to under 6,000 in 1957, the first year after the vaccine was widely available.

In 1962, an oral vaccine developed by the Polish-American researcher Albert Sabin became available, greatly facilitating distribution of the polio vaccine. What followed was a mass vaccination program that marked the beginning of the end for polio in the developed world.  polio12But just when polio was on the verge of extinction, it has come back in many parts of the world. Some are places where there was no vaccination available.  In the West, lack of immunization is a factor in its resurrence.  One organization that has made a major impact on the eradication of poliomyelitis is Rotary International. Check out the video on Rotary’s efforts to eradicate Poliomyelitis.

The Hearing Loss Connection

While some sources indicate that polio has not much to do with hearing loss, others report that over the last three to four decades the incidence of acquired sensorineural polio17hearing loss (SNHL) in children living in developed countries has fallen as a result of improved neonatal care and the widespread implementation of immunization programs.  There are indications that even non-paralytic Polio sometimes leads to hearing loss or learning abnormalitiesBachor and Karmody (2001) found that in the temporal bones of a 26-month-old white female with a paralytic syndrome clinically and pathologically identical to poliomyelitis there was a complete absence of the cochlear neurons and substantially reduced peripheral and central axons with loss of some inner hair cells but preservation of outer hair cells. They also found that Scarpa’s ganglion and the geniculate ganglion were partially atrophied and that the saccule and utricle were mildly dilated and Reissner’s membrane at  the apical turn was bulging.

In two previous audiological studies a 10-20 dB bilateral sensorineural hearing loss was found in poliomyelitis patients and a neuronal lesion was suggested.  While the jury is still out as to how prevalent hearing impairment is among polio victims, Audiologists need to be vigilant and look for hearing impairment in patients that have contracted poliomyelitis, especially in children.