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Why did Beethoven, possibly the greatest composer of all time, go deaf at the age of 28? Read on in this short Tweetorial to learn about a theory...

#medtwitter #FOAMed #MedEd #medicalhumanities
(theory co-described with Sunil Nair and Joseph Tremaglio)

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First, let's get Beethoven's review of symptoms, as documented in his letters and conversations 🤔:

+ Progressive hearing loss/tinnitus ➡️ total deafness
+ Chronic unilateral eye pain
+ Polyarthritis
+ Fatigue
+ Chronic abdominal pain and diarrhea

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Many theories try to explain Beethoven's deafness. Perhaps most popularly:

▪️Lead poisoning (supported by high lead levels found in the composer's hair)
▪️Paget's disease (auditory nerve compression from skull changes)
▪️Lupus
▪️Sarcoidosis
▪️Typhus

sciencedirect.com/science/articl…

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But none of these theories explains ALL of Beethoven's various chronic symptoms that we listed above, at least not when presenting in their most archetypal ways, and have been put in doubt by medical anthropologists.

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Luckily, Beethoven consented to (or, rather, requested) an autopsy, specifically to solve the mystery of why he lost his hearing, as reported in Anton Schindler's 1840 biography "Beethoven as I Knew Him".

amazon.com/Beethoven-Knew…

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A pathologist named Dr. Johann Wagner performed the procedure and reported that Beethoven's auditory arteries were "thickened and cartilaginous as though stretched over a raven’s quill”. 🖋️

Now, that sounds an awful lot like a vasculitic process...

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Q: If we assume that Beethoven went deaf from vasculitis affecting his inner ear, can we identify a syndrome that could account for his entire symptomatology and autopsy findings?

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We can!

Cogan's Syndrome:

⭐️Medium vessel vasculitis in the inner ear ➡️ arterial fibrotic hypertrophy ➡️ sensorineural deafness (consistent w/ autopsy)
⭐️Uveitis/keratitis
⭐️Protean systemic symptoms e.g. fatigue and arthritis
⭐️Associates w/ IBD

ncbi.nlm.nih.gov/pubmed/21272808

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Let's return to Beethoven's review of symptoms and ✅ with Cogan's Syndrome

+ Progressive hearing loss/tinnitus ➡️ total deafness ✅
+ Chronic unilateral eye pain (e.g. keratitis) ✅
+ Polyarthritis/fatigue ✅
+ Chronic abdominal pain and diarrhea (concerning for IBD) ✅

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Did Beethoven actually have Cogan's Syndrome? While his symptoms and autopsy suggest it, who knows!

Does his presentation remind you of any other syndromes?

If interested in learning more, check out our article "Diagnosing Beethoven" in @amjmed
amjmed.com/article/S0002-…