Joyce Maier recently write:

>I sent a short list of names of those who speculated about the cause of
>Beethoven's deafness, but I forgot Larkin's digressions on the possible
>diagnosis SLE, that could have caused the deafness and the bowel problems
>and liver cirrhosis as well.

Having followed this topic with some interest I find myself enticed out
of my lengthy silence to write as an Internist that the hypothesis of
SLE (systemic lupus erythematosus) sounds to me too far fetched.  SLE is
extraordinarily rare in men.  Lupus hepatitis can cause liver cirrhosis but
it is a different disease entity to proper SLE, where death from hepatic
failure is most unusual.  Renal failure is the commonest cause of premature
death in SLE.  While textbooks commonly mention abdominal pain as a feature
of SLE, it is not a feature seen as widely as you would think.  Bowel
problems in general usually are not that prominent a feature of the
disorder as much as joint pains, fevers, dramatic hair loss (hair falling
out in large clumps - not just male pattern balding!), skin rashes,
shortness of breath and pleuritic chest pains.  I am not aware of Beethoven
having any of these symptoms.  As for deafness, I have never seen or heard
of a case of SLE causing this in my life.  All in all the SLE theory sounds
about as attractive as an alien abduction hypothesis: POSSIBLE perhaps but
scarcely credulous.

>Also interesting is the publication by Kubba and Young in The Lancet
>(1996).  Their conclusion is that a diagnosis is not possible, because we
>don't have enough data.  Their choice for the bowel problems and liver
>cirrhosis is Crohn's disease.

This one is more plausible.  Crohn's usually causes bloody diarrhea however
and I am unaware of this being a feature of Beethoven's medical history.
Liver cirrhosis from associated sclerosing cholangitis is known to occur in
Crohn's, but is again uncommon for it lead to end stage hepatic cirrhosis.
This hypothesis posits a rare complications of a relatively uncommon
disease.

>Other recent attempts to throw some light on Beethoven's mysterious
>chronic illnesses (the bowel problems and the deafness, both already
>present when he was still in his twenties) are Sharma's choice for
>Whipple's disease (1994) and Palferman's choice for sarcoidosis (1990).

These theories also sound wacky.  They are not impossible hypotheses but it
is just that sarcoidosis causing deafness and abdominal disease would be
extraordinarily rare whether in our age or centuries ago.  The commonest
manifestation of sarcoidosis is respiratory, when pulmonary sarcoid causes
shortness of breath.  To have sarcoid that is so widely disseminated that
you have both sarcoidosis in your head as well as in your abdomen, while
somehow conveniently missing out the biggest organ that lies in between
(namely the lungs) seems most bizarre.

As for the Whipple's - well that's another alien abduction equivalent.  One
of the physicians at my hospital says Whipple's is a diagnostic possibility
you mention when you are really stuck for an answer and you want to sound
clever when you really have no idea.  Also Whipple's causes neurological
disease such as myoclonus, dementia and gaits disturbances.  It is as rare
as hens' teeth.

As for the syphilis hypothesis, I don't think it can be entirely thrown
out.  In fact you would have to remain suspicious of this condition.
Having said that, to say he had deafness from syphilis means that he
had tertiary neurosyphilis.  Yet for years after the onset of deafness
quaternary neurosyphilis and its characteristic dementia and paralysis
did not develop. No good statistics are available as to what percentage
of patients with tertiary neurosyphilis develop quaternary GPI (general
paralysis of the insane) but it should be extremely high.  The fact that no
mercury was found in Beethoven's hair also fails to exclude the diagnosis.

As far as the lead poisoning theory goes, I think it is very plausible
indeed.  Lead poisoning can be very insidious and equally difficult to
diagnose or exclude on purely clinical grounds.  The only sure way to
diagnose it with the combination of a suspicious history plus the
appropriate serological testing.  Absence of peripheral neuropathy,
gum discoloration and the reputed lack of documentation of other purely
clinical signs of lead toxicity count for NOTHING when trying to exclude
this diagnosis.  Sure, it might not explain the hearing loss, but when you
have to resort to the above sort of outlandish nonsense to try to explain
everything with a 'one hit hypothesis' it is time to say enough with
Occum's razor and resort to multiple diagnoses to explain things.  Of all
the theories at least lead poisoning can be corroborated using at least
some sort of quantitative evidence.  If you throw that type of evidence out
then you back to your assortment of alien abduction type of speculative
diagnoses, where anything vaguely 'plausible' goes.

In fact lawyers like to come up with this sort of 'but could it be just
POSSIBLE doctor?' type of questioning when cross examining medical experts
in court.  At which point the doctor is forced to concede 'yes it could
be POSSIBLE'.  In one celebrated case in Australia a lawyer asked the
pathologist how he could be so sure the patient was dead if he hadn't
checked the pulse, blood pressure etc, whereupon the pathologist answered:

   Well let me put it this way, by the time I was through with the
   patient his brain was sitting on my desk in pot of formalin, but I
   guess it is just POSSIBLE that he might still be out there somewhere
   practicing as a lawyer.

Lastly I have accessed the Lancet article on Beethoven on line and
was reassured to find that the authors often came to roughly similar
conclusions to mine.

Reference:
Ludwig van Beethoven: a medical biography
Kubba, Adam K.; Young, Madeleine
Volume 347(8995)     20 January 1996      pp 167-170

Satoshi Akima
Sydney, Australia