Background
Bedřich Smetana (1824–1884) of the Romantic Era is known today as the father of Czech music.Reference Höschl1 Famous works of this patriotic composer include Má vlast (My Fatherland), Prodaná nevěsta (The Bartered Bride), and String Quartet No. 1 in e minor: Z mého života (From My Life).Reference Smetana2 Gifted from a young age, Smetana flourished as a composer despite spending the last 10 years of his life deaf.Reference Clapham, In and Sadie3 His life’s work was tragically cut short as he then became progressively ill until his untimely death at 60 years of age in a mental asylum in Prague.
Clinical presentation
Smetana’s medical history and notable musical works throughout his life are summarized in Table 1.Reference Höschl1, Reference Smetana2, Reference Betka4–Reference Pesic and Pesic9
Medical history and notable musical achievements of Bedřich Smetana, born 2 March 1824 and died 12 May 1884
Smetana was born on 2 March 1824. In his medical history, he was afflicted with osteomyelitis of the right temporal bone and mandible due to a gunpowder explosion in his childhood.Reference Höschl1, Reference Smetana2, Reference Betka4
Smetana’s illness first manifested itself in the form of tonsillitis in 1874 and subsequent general exanthema, dizziness, and the catastrophic symptoms of tinnitus, auditory hallucinations, and progressive bilateral hearing impairment until complete deafness within a few months.Reference Höschl1, Reference Smetana2, Reference Betka4–Reference Pedro, Germiniani and Teive6 He was afterwards diagnosed with “labyrinth paralysis” in 1875 and treated with politzerisation, electrical stimulation, and mercury ointment, all of which failed. In 1879, he also developed symptoms of depression and what he himself suspected was insanity.
From 1881 onward, Smetana’s condition deteriorated sharply both physically and mentally, and his symptoms persisted until his death in 1884.Reference Smetana2 He was hypersensitive to coldness and had an ongoing cough with severe dyspnea, nocturnal chest pain, and crackles.Reference Wagnerová5 In addition to confusion, memory deficits and mental exhaustion, he also had repeated visual and auditory hallucinations, along with ataxia and expressive aphasia, which were exacerbated after a stroke-like seizure in 1882.Reference Höschl1, Reference Pedro, Germiniani and Teive6 In his final year, the composer had fully developed dementia, unable to recognize family or friends, and was psychotic with unpredictable agitation and rage attacks. He also had left facial paresis and swallowing difficulties. He was severely cachectic, and his speech and letters were incomprehensible. Smetana passed away on 12 May 1884.
Autopsy
Two autopsies have been performed on Smetana’s body, in 1884 immediately post-mortem and a century later in 1987 when the body was exhumed and re-examined.Reference Höschl1, Reference Smetana2, Reference Betka4, Reference Vacek7 The findings of both revealed:
• skull asymmetry, with hypoplasia of middle and lower third of right facial skeleton
• no significant inflammatory changes in right temporal bone or mastoid process
• (microscopic) signs of inflammation in right malleus and incus
• bilateral cochlear nerve atrophy without major pathological changes of inner ear
• chronic internal hydrocephalus, with ventricular dilation
• cerebral atrophy, with iron deposition in brain cortex
• granular ependymitis
• chronic leptomeningitis, with meningeal thickening and Treponema pallidum found in brain
• positive serology for syphilis in various regions of the body (Treponema pallidum haemagglutination test (TPHA) +, fluorescent treponemal antibody-absorption test (FTA-ABS IgG) ±, rapid plasma regain (RPR) test +++)
• chronic endarteritis, with suspected atherosclerotic or cerebral vascular sclerotic change
• thrombosis arteriae femoralis
• bilateral lobar pneumonia
• furunculosis
• block vertebrae at C2/3
• huge concentrations of mercury in tissues
Discussion
As medicine and technology were not as advanced or well-developed in Smetana’s time as they are today, doctors could only rely on history taking, physical examination, and post-mortem examinations to make a diagnosis. Smetana’s doctors therefore concluded with logical reasoning that he had labyrinth paralysis and treated his condition symptomatically.Reference Höschl1, Reference Betka4 From a modern point of view, however, and based on his autopsy results, Smetana died of neurosyphilis complicated with bilateral lobar pneumonia.
Today, hearing loss can be classified into two major groups: conductive hearing loss, in the outer and/or middle ear, and sensorineural hearing loss, in the inner ear, cochlear nerves, and/or brain. It can also be divided into lesions of the central or peripheral nervous system. Since Smetana’s death, two main potential etiologies for his deafness and other debilitating neurological symptoms have emerged based on his clinical presentation and the autopsies.Reference Höschl1, Reference Betka4, Reference Vacek7 One is that the composer suffered from complications of osteomyelitis of the right temporal bone. The other is that he was infected with syphilis, which progressed to neurosyphilis.
Osteomyelitis of the temporal bone is a bone infection that can be caused externally through trauma or medical procedures or internally as a complication of mastoiditis.Reference Prasad, Prasad, Kumar, Thada, Rao and Chalasani10 Mastoiditis is often a complication of acute or chronic otitis media with an internally disseminated infection that destructs the mastoid bone. Possible symptoms include local inflammation or tenderness, (chronic) ear discharge, and general complaints like headache, exhaustion, or fever. Both osteomyelitis and mastoiditis can result in further sequelae such as (conductive) hearing impairment or cranial nerve dysfunction. They are treated symptomatically and, when needed, with antibiotics or surgical intervention.
Neurosyphilis is an infection of the central nervous system (brain and/or spinal cord) by Treponema pallidum that can occur especially if a previous syphilis infection has not been thoroughly treated.11, 12 Complications include visual and/or auditory hallucinations, as well as meningeal or meningovascular syphilis, which may lead to meningitis or ischemic stroke. General paresis usually affects the frontotemporal brain parenchyma and causes changes in the patient’s mental status. Tabes dorsalis, demyelination of the dorsal columns and dorsal root ganglia, results in impaired proprioception and therefore ataxia. In later stages, damage to lower cranial nerves could also result in manifestations like facial paralysis and swallowing difficulties. If the infection spreads to the ear, it can also induce (sensorineural) hearing loss, tinnitus and dizziness. The mainstay of treatment for syphilis is penicillin.
In Smetana’s case, his clinical presentation and the two autopsies clearly indicate that the problem lay in the central nervous system.Reference Höschl1, Reference Smetana2, Reference Betka4, Reference Vacek7 Complete deafness, tinnitus, hallucinations, change in mental state and gait abnormalities all coincide with neurosyphilis. The positive serology for syphilis, along with proof of meningeal inflammation and cerebral changes in the autopsy, also validate the diagnosis. This is supported by records of his personal life, as he had at least one known mistress and lived an active social life.Reference Clapham, In and Sadie3
Furthermore, Smetana’s accounts and the pathological results argue against osteomyelitis or a peripheral lesion as the cause for his deafness. No significant pathological changes have been found in the middle or inner ear on either side, and the ossicles did not suffer severe damage despite his asymmetric skull.Reference Betka4 Smetana did not complain of other signs of chronic otitis media such as long-term ear discharge, infection or inflammation,Reference Höschl1 so it was unlikely that he developed chronic otitis media from tonsillitis. It was also unlikely the rapidly progressive bilateral hearing impairment that first appeared in 1874 was caused by the right-sided osteomyelitis that happened 40 years prior.
There was likewise a low probability that Smetana suffered from adult meningitis as a possible complication of childhood temporal bone fracture.Reference Patel, Lofgren and Varacallo13, Reference Diaz, Cervenka and Brodie14 Posttraumatic meningitis was not a prime consideration, as Smetana did not complain of chronic otorrhea or rhinorrhea or other potential signs or symptoms of a posttraumatic CSF leak.Reference Wetmore, Herrmann and Fisch15 The onset of his neurological symptoms first occurred about 40 years after his facial injury from the gunpowder explosion (Table 1), and no fractures were documented in either Smetana’s records or the autopsies.Reference Höschl1, Reference Smetana2, Reference Betka4, Reference Vacek7, Reference Vlček16 Rather, the osteomyelitis and subsequent hypoplasia of the right facial skeleton were a result of bone tissue damage caused by a complicated soft tissue infection from the injury.Reference Vlček16
In addition to neurosyphilis, Smetana might have suffered from mercury poisoning, based on the high concentrations of mercury discovered in his body.Reference Höschl1, Reference O’Shea17 Although syphilis had never been confirmed while he was alive, mercury was a common first-line treatment for syphilis in the 19th century. It is also known today that mercury is a neurotoxin that could lead to psychosis and slow down the auditory brainstem response, which may have aggravated Smetana’s neurological symptoms.
• Smetana was a renowned Romantic composer whose loss of hearing in his final decade has remained ambiguously explained for the past century.
• Smetana’s hearing loss was most likely caused by neurosyphilis.
• Smetana’s clinical picture overwhelmingly favors syphilis over osteomyelitis.
• Mercury poisoning may have accelerated Smetana’s hearing loss.
• As supported by his clinical presentation and autopsy results, the authors conclude that Smetana died of neurosyphilis complicated with bilateral lobar pneumonia.
Conclusion(s)
The true nature of Bedřich Smetana’s illness has been under debate since his death over a century ago. From Smetana’s clinical presentation to pathological analyses from autopsies, we have a clearer picture regarding the etiology of his neurological condition in the last decade of his life. Were Smetana alive today, he would have most likely been promptly diagnosed with syphilis and treated with modern medical resources.
Funding statement
This research received no specific grant from any funding agency, commercial or not-for-profit sectors.
Competing interests
The authors declare none.
Ethical standards
Ethical approval is not required for this study in accordance with local or national guidelines.
