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The father of Czech music, Bedřich Smetana was a brilliant, patriotic Romantic composer who spent his last decade completely deaf. He became progressively ill in his final years and passed away prematurely at 60 years old. Since then, there have been two main propositions for the etiology of his neurological symptoms, in particular his hearing loss: neurosyphilis or osteomyelitis of the temporal bone.
Methods
This article compares the clinical presentation and pathology of neurosyphilis and osteomyelitis.
Results
This article infers which one is arguably the most likely cause based on Smetana’s own medical history, signs and symptoms and autopsy findings.
Conclusion
Smetana’s clinical presentation and pathological results grant us a clearer picture of his neurological condition and allows us to diagnose his final neurological deterioration as complications of neurosyphilis and not osteomyelitis of the temporal bone.
Ménière’s disease is a chronic inner-ear disease attributed to endolymphatic hydrops. Magnetic resonance imaging with gadolinium allows visualisation of endolymphatic hydrops in vivo and may be an adjunct to diagnosis.
Methods
Thirty-eight patients suspected of having Ménière’s disease underwent T2 weighted three-dimensional fluid-attenuated inversion recovery and true inversion recovery sequence magnetic resonance imaging 4 hours post double-dose intra-venous gadolinium. Presence of endolymphatic hydrops was graded by two radiologists at 0 and 4 months. Correlation to clinical diagnosis was assessed using Fisher’s exact test.
Results
Hydrops was identified in 88 per cent, 17 per cent and 27 per cent of patients with Definite Ménière’s, Probable Ménière’s and Undifferentiated disease, respectively. A significant correlation existed between diagnosis and presence of hydrops. Sensitivity and specificity were 88 per cent and 67 per cent, respectively. Intra- and inter-observer agreement for presence and grading of hydrops was near-perfect and substantial to near-perfect, respectively.
Conclusion
Magnetic resonance imaging demonstrates radiographic hydrops with significant correlation to clinical diagnosis and good intra- and inter-observer agreement.
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