Download Lead and the deafness of Ludwig van Beethoven

The Laryngoscope
C 2013 The American Laryngological,
V
Rhinological and Otological Society, Inc.
Contemporary Review
Lead and the Deafness of Ludwig Van Beethoven
Michael H. Stevens, MM, MD; Teemarie Jacobsen, AuD; Alicia Kay Crofts, AuD
Objectives/Hypothesis: To reexamine the cause of Beethoven’s hearing loss because of significant recent articles.
Data Sources: Medical and musical literature online, in print, and personal communication.
Methods: Relevant literature review.
Results: Evidence of otosclerosis is lacking because close gross examination of Beethoven’s middle ears at autopsy did
not find any otosclerotic foci. His slowly progressive hearing loss over a period of years differs from reported cases of autoimmune hearing loss, which are rapidly progressive over a period of months. He also lacked bloody diarrhea that is invariably
present with autoimmune inflammatory bowel disease. The absence of mercury in Beethoven’s hair and bone samples leads
us to conclude that his deafness was not due to syphilis because in that era syphilis was treated with mercury. High levels of
lead deep in the bone suggest repeated exposure over a long period of time rather than limited exposure prior to the time of
death. The finding of shrunken cochlear nerves at his autopsy is consistent with axonal degeneration due to heavy metals
such as lead. Chronic low-level lead exposure causes a slowly progressive hearing loss with sensory and autonomic findings,
rather than the classic wrist drop due to motor neuropathy from sub-acute poisoning. Beethoven’s physicians thought that he
had alcohol dependence. He particularly liked wine that happened to be tainted with lead.
Conclusions: Beethoven’s chronic consumption of wine tainted with lead is a better explanation of his hearing loss than
other causes.
Key Words: Otosclerosis; autoimmune; syphilis; lead; alcohol.
Laryngoscope, 123:2854–2858, 2013
INTRODUCTION
There are many opinions regarding the etiology of
Beethoven’s hearing loss. This is primarily due to the
lack of confirmatory evidence from audiograms, temporal
bone histology, and hematological studies.
At age 27 Ludwig van Beethoven first noticed a
hearing problem. He developed tinnitus, with buzzing
and ringing, and became aware that he missed words
and phrases. At age 30 he first confessed this problem in
a letter to his childhood friend, who was also a physician, Franz Gerhardt Wegeler. It was sent on June 29,
1801. In it Beethoven said, “For the last three years my
hearing has grown weaker and weaker—I cannot hear
the high notes of instruments or voices—I can hear
sounds, but I cannot make out the words.”1 His hearing
became slowly and progressively worse, so that by 1814
conversation became impossible. He tried to use an ear
trumpet with limited success. He stopped playing the
piano in 1815. By 1816 Beethoven was completely deaf
in the right ear. In 1818 he resorted to written conversation books, and by 1822 he stopped conducting. At the
performance of his Ninth Symphony in 1824, where he
was the honorary conductor, he had to be turned toward
the cheering crowd to witness the applause when
the performance was complete because he could not
hear it.2
MATERIALS AND METHODS
An extensive review of the musical and medical literature
as a part of an unpublished research paper for a master’s
degree in history and literature, on file at the University of
Utah, was done. The information available from the literature
of only the most relevant of proposed diagnoses for Beethoven’s
hearing loss will be carefully analyzed, as commenting on
all proposed diagnosis is beyond the allotted space of this
journal.
RESULTS AND DISCUSSION
University of Utah School of Medicine, Salt Lake City, Utah,
U.S.A
Editor’s Note: This Manuscript was accepted for publication on
March 4, 2013.
Presented at the Triological Society Combined Sections Meeting in
Scottsdale, Arizona, January 24, 2013.
The authors have no funding, financial relationships, or conflicts
of interest to disclose.
Send correspondence to Michael H. Stevens, MM,MD, 50 North
Medical Drive Suite 3C120, Salt Lake City, UT 84132. E-mail:
[email protected]
DOI: 10.1002/lary.24120
Laryngoscope 123: November 2013
2854
What do the symptoms described by Beethoven tell
us about his hearing loss, as described above? First, it
was slowly progressive over a period of years. Second, it
involved the high frequencies, at least initially. And
third, he had reduced discrimination. Patricia Shearer,
in her excellent article supporting cochlear otosclerosis,
points out that the age of onset, and the slowly progressive nature of the loss fits with otosclerosis.3 However,
there are several strong arguments against this etiology.
One is that cochlear otosclerosis usually is associated
Stevens et al.: Lead and the Deafness of Ludwig Van Beethoven
with good discrimination. In addition, there is no family
history of deafness in Beethoven’s family. This is striking, since in a family of musicians like his, deafness
would probably have been mentioned had it been present. Most crucial of all, however, is that although no
temporal bone histologic examination has been done,
because Beethoven’s temporal bones were either lost or
stolen, a careful gross examination of the temporal bone
was completed at the time of his autopsy, including a
description of the Eustachian tube opening, the middle
ear space, and the mastoid. In the autopsy report there
is no mention of otosclerotic involvement of the oval window, which could have easily been seen by the naked
eye.4 Because of the careful description of the gross findings, it is extremely unlikely that this would have been
missed. Otosclerosis had been described by A. M. Valsalva in 1735 following an autopsy of a deaf patient,5
and by Beethoven’s time other reports were also available. It is very unlikely that otosclerosis present for over
30 years would not have involved the oval window.
Marked atrophy of the cochlear nerves seen at Beethoven’s autopsy is not a finding that is present, even
with extensive cochlear otosclerosis. Therefore, evidence
of otosclerosis as the cause of his deafness is lacking.
In a persuasive paper written by Collin S. Karmody,
autoimmune hearing loss has been championed as the
cause of Beethoven’s hearing loss.6 Karmody suggests
that autoimmune loss could also explain the diarrhea
and other medical problems that Beethoven had. Recent
papers have noted the association of autoimmune bowel
disease with hearing loss. Beethoven himself was the
first to think that his hearing loss had something to do
with his gastrointestinal problems. However, the criteria
described by Brian McCabe of autoimmune hearing loss
is that it is usually a bilateral hearing loss that progresses rapidly over a period of weeks or months not
years.7 Reported cases have also often presented with
sudden hearing loss and vertigo, which Beethoven
lacked. Although the clinical presentation of these
patients can be quite varied, our review of the literature
did not find any reported cases with such a slow progression over many years as Beethoven’s.
Beethoven also said that “when people shout I
cannot bear it.”1 What does this statement mean? Does
it represent recruitment that may be present with a
cochlear or sensory loss of hearing of autoimmune
disease origin? Is there another explanation? A similar
phenomenon is seen with seventh cranial nerve lesions,
as described by Geary McCandless at our institution,8
and also with central hyperexcitability due to headache,
depression, or anxiety.9 It is well known that Beethoven
had headaches as well as depression on multiple occasions.10 In addition, it is interesting to note that Beethoven said that his deafness “caused me the least trouble
in playing and composing, the most in association with
others.”11 McCabe also appropriately notes that if
Beethoven truly had recruitment, he could not have tolerated sound levels of ninety to one hundred decibels
while at the piano playing one of his concertos with a
full orchestra.11 This may support the concept of phonophobia due to a central or cerebral cause rather than
Laryngoscope 123: November 2013
Fig. 1. Lead fluorescence intensity in Beethoven’s hair (LVB) in comparison to standard counts (SN-1) of controls. [Color figure can be
viewed in the online issue, which is available at wileyonlinelibrary.com.]
recruitment from the peripheral ear. Beethoven’s reaction to people shouting might be interpreted as thus:
Since he was a musician, he was embarrassed that
someone would have to shout at him to be heard. He
suggests this latter possibility in one of his letters.1
In his provocative article of 1958, reprinted in 2004,
McCabe felt that Beethoven’s hearing loss was neural in
origin because of the finding of shrunken cochlear
nerves at autopsy that lacked pith, which the dictionary
defines as the center or core.11 This suggested to
McCabe the possibility of acquired syphilis as a cause of
his hearing loss because of the slowly progressive bilateral nature of that loss. Our reading of Beethoven’s medical history, however, indicates that none of his
physicians mentioned his having syphilis. One reason
for the proposal of syphilis is Beethoven’s use of a salve
that was thought to contain mercury, a drug commonly
used for the treatment of syphilis at that time. It has
been confirmed, however, that the salve contained ammonium and not mercury.12 In addition, researchers at
the U.S. Department of Energy’s Argonne National Laboratory in Argonne, Illinois, found no evidence of mercury in Beethoven’s hair and bone samples.13,14 This
gives additional support to the idea that his deafness
was not caused by syphilis.
What then was the cause of Beethoven’s deafness?
Recent analysis of his hair and bone has determined
that he had lead poisoning.13,14 Can lead poisoning
explain his hearing loss? A lock of his hair removed at
the time of his death, and stored in an airtight case, was
analyzed in 2000 by researchers in Illinois (Fig. 1). Beethoven’s parietal skull bone was later analyzed in 2005.
Both showed markedly elevated lead levels consistent
with lead poisoning. The lead found in the bone is very
significant in showing that Beethoven had been repeatedly exposed over a period of many years, according to
personal communication with Ken Kemner, physicist,
Stevens et al.: Lead and the Deafness of Ludwig Van Beethoven
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and leader of the X-ray characterization components of
this project.15 This is because the lead was found deep
in the bone, not just at the surface, indicative of chronic
exposure. This is in contrast to the suggestion of Reiter’s
that the lead represented exposure that was limited to
the time period at the end of Beethoven’s life due to
medical interventions.16 The speed of human hair
growth differs from person to person and from time to
time within one individual, so his ability to pinpoint
times of exposure raises considerable doubt.17 Reiter’s
analysis shows lead peaks between 200 and 369 days
before Beethoven’s death, but Reiter does not venture to
suggest the origin of these peaks. The scientific community feels that hair analysis has many issues that need
to be resolved before it can become a useful tool in
understanding environmental exposure, and that hair
analysis is useful for only about a year of exposure
time.18
Whole blood levels are the best way to follow lead
exposure in a living person, but lead levels in bone give
the most accurate measurement of total body burden
and are thus a better measurement of chronic exposure
levels.19 Even at low blood levels, chronic exposure to
lead can result in a slowly progressive high-frequency
loss.20,21 Increasing blood levels of lead correlate directly
with the amount of hearing loss.22–24 Findings at Beethoven’s autopsy included wrinkled cochlear nerves that
lacked pith. The recent retranslation of the original autopsy report in Latin state that “the acoustic nerves
were wrinkled and were without a medulla.”2 This damage to the central portion of the nerve is suggestive of
axonal degeneration that is seen in humans with lead
injury. Recent studies have demonstrated abnormal auditory brainstem responses in lead exposure indicative of
a neural site of damage.25,26
One of the primary reasons that lead poisoning has
been rejected as a cause of Beethoven’s hearing loss is
that over a period of 30 years it has been assumed that
he would have developed other neurological symptoms
such as wrist drop. Traditionally the neuromuscular disorder associated with lead poisoning has been primarily
a motor disorder. This classic lead toxicity picture, however, is one of subacute exposure over a mean period of
4 to 5 years, and is currently felt to be a form of leadinduced porphyria by the Mayo Clinic.27
A remarkable article from Latvia studied 151 workers with inorganic long-term lead exposure over a period
of 8 to 47 years with a mean of 21.7 years.28 In 46 workers who had neuropathy, mild sensory and autonomic
neuropathic features were found rather than the classic
motor neuropathy. The researchers of this study and
researchers at the Mayo Clinic think these findings are
due to a direct neurotoxic effect of lead. In this group of
46 patients they also found mood disturbances, abnormal liver and kidney function, and gastrointestinal disturbances similar to those experienced by Beethoven.
Their sensory symptoms included pain and paresthesias
in the feet and hands, of which Beethoven complained,
and which he thought were due to arthritis.
The first neuropathic symptoms in these Latvian
patients took between 7 and 45 years after initiation of
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2856
exposure. There has been no previous study that has
reported such extended exposure. This is similar to the
toxic neuropathy seen with other heavy metals. It
occurs at lower levels of exposure over a long period of
time.
Where could the lead have come from that resulted
in this devastating loss of hearing? Although lead can be
acquired from a number of external sources, including
dishes, lead-lined wine flasks, lead crystal, or mineral
water at the spas, in our opinion the most likely source
for Beethoven was wine. It is well known that at that
time lead was added illegally to inexpensive wine to
improve the flavor. Beethoven was particularly fond of
the adulterated or fortified Hungarian wine. It has been
suggested that after the death of Beethoven’s mother
when he was 17 years old, he began to use some wine to
help deal with his loss.29
As as result, there would have been 10 years for his
symptoms of tinnitus to begin. When he was 30 years of
age, Beethoven began to consume a lot of wine with
meals to stimulate his appetite and ease his abdominal
pain. This coincides with when he confessed his hearing
loss to others. Four of his physicians who treated him at
different times during the final 17 years of his life were
of the opinion that he had an alcohol dependence problem.10 Alexander Wheelock Thayer, a significant Beethoven musicologist, found in a meticulous study of
Beethoven’s daily housekeeping records that the cost of
his wine purchases was far from moderate.30 Friends
and tavern owners relate his drinking a bottle of wine
with each meal.31 Beethoven himself relates several
instances when he became intoxicated.30
Although Wegeler states that he never observed
Beethoven drunk,32 he had not personally lived with
him since his early days in Bonn, so this opinion lacks
proximate knowledge. His personal secretary Anton
Schindler, also denied Beethoven’s alcohol problem, but
he also deliberately destroyed some of the pages of Beethoven’s conversation books that were used extensively to
communicate when Beethoven could not hear.10 The purpose of this destruction may have been to erase the evidence of Beethoven’s alcohol problem. It is well known
that Schindler made deliberate falsifications to conceal
certain deficits in Beethoven’s character. Schindler also
stated that “Beethoven preferred wine of the heights
around Buda to every other. But as he was no judge of
wine, and therefore could not tell the adulterated from
the pure, and by drinking the former he often caused
great derangements to his weak stomach, but no warning had any effect on him.”33
Alcohol dependence is known to have a strong family history, and Beethoven’s family exhibits this. His own
father and grandmother died of complications directly
due to alcohol.
Beethoven’s problem was that of dependence, as he
had increased tolerance, used larger amounts over a longer period of time, and made no effort to reduce consumption. He did not, however, meet the criteria for
alcohol abuse commonly known as alcoholism.10
Francois Mai, a psychiatrist at the University of
Ottawa in Canada, thinks that Beethoven’s alcohol
Stevens et al.: Lead and the Deafness of Ludwig Van Beethoven
dependence problem was due to underlying depression
as a result of his hearing loss.
His reaction to the loss was so severe that Beethoven considered taking his own life in 1802. Symptoms of
depression are present in numerous letters written by
Beethoven.
In one that is particularly striking, he talks of suicide by his own hand.1 However, lead can also cause the
aggression, impulsivity, headaches, and depression that
Beethoven exhibited on numerous occasions.
Can any other of Beethoven’s other medical problems be traced to lead? Today lead is known in thirdworld countries to cause hepatotoxicity, kidney damage,
and abdominal colic.34,35 The onset of abdominal problems in his teenage years was perhaps due to the start
of his use of wine to deal with his mother’s death. His
symptoms of severe abdominal pain are a good description of “lead colic.” He also had diarrhea, which could
have been due to pancreatitis that was seen at the time
of his autopsy. As he was overly concerned about constipation, some of his diarrhea could have been iatrogenic.
In our review of the literature, there is no mention
of blood in the stools, which is invariably present with
autoimmune inflammatory bowel disease, and Beethoven
is not described as being anemic from blood loss. Findings observed with inflammatory bowel disease such as
adhesions, strictures, or perforations were not mentioned in his autopsy report. However, it is possible that
Beethoven may have had irritable bowel syndrome,
which is often associated with depression. This could
also explain his response to the spa treatments. They
may have helped his abdominal symptoms by soothing
his emotions.
A shrunken, macronodular liver found at Beethoven’s autopsy is a common but not pathopneumonic
finding of primary sclerosing cholangitis that is seen
with autoimmune inflammatory bowel disease.36 However, although alcohol commonly causes a large, fatty,
micronodular liver in early stages of cirrhosis, it can
also produce a shrunken, macronodular liver in endstage cirrhosis.37 One cannot tell the cause of the cirrhosis at autopsy by the appearance of the liver.38 Pruritis,
which often accompanies primary sclerosing cholangitis,
was notably absent. It is therefore much more likely
that his cirrhosis was due to alcohol than primary sclerosing cholangitis.39 Lead may have also played a role in
the liver toxicity. Although inflammatory bowel disease
has been reported with autoimmune hearing loss, there
is not conclusive evidence that Beethoven had either.
George Frederic Handel was also known to have had
headaches, irritability, rheumatic pains, bizarre behavior, and colic presumably from the lead in the wine that
he drank.40
CONCLUSION
We agree with the statement by Jeffrey Harris in
the 2010 William F. House lecture at the annual Neurotology Society meeting that “we will probably never
know the cause of Beethoven’s hearing loss.”41 If we possessed audiograms, temporal bone histology, blood tests,
Laryngoscope 123: November 2013
and microscopic examination of tissues, we could definitely pinpoint the cause of Beethoven’s deafness. Lacking these, in our opinion, the best explanation of
Beethoven’s deafness is that it was due to the lead in
the wine that he drank. His other medical problems can
also be related to lead, alcohol, or both. Ludwig van
Beethoven’s medical history may therefore be summarized as being caused by one entity: his consumption of
wine tainted with lead.
ACKNOWLEDGEMENT
Special thanks to Clough Shelton, M.D., Chairman of Otolaryngology, Head and Neck Surgery, University of Utah
School of Medicine, for reading this article.
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