gms | German Medical Science

Dedicated to Prof. Dr. Dr. Ulrich Eysholdt on his 75^th birthday.

Bedrich Smetana is regarded as the Czech national composer. This year marks his 200^th birthday. Of his works, “Die Moldau” is probably the best known and most frequently performed. Like Beethoven, Smetana also suffered from severe hearing problems and, like Beethoven, he composed the most important pieces with almost complete deafness [14], [33]. His illness has been analysed and controversially discussed in numerous articles, especially with regard to the assumed causal structure in the context of a syphilis infection. Smetana’s life and work are described below. Syphilis and its relationship to hearing loss are then described.

Bedrich Smetana was born on March 2^nd 1824. After studying music, he earned his living by giving piano lessons and founded his own music school in 1848. In the same year, he married Katharina Kolar and lived with her for several years in Gothenburg (Sweden), where he worked as an orchestra conductor. Of his four children, only his daughter Sophie (1853–1902) survived. His wife died in 1859 and he returned to Prague. There, in 1860, he married Betty Fernandy, sixteen years younger than him. Smetana achieved public recognition in 1866 with his operas such as “The Bartered Bride”, and he was eventually appointed conductor at Prague opera house. In this position, he was subjected to a great deal of hostility. He was accused of rehearsing too little with the orchestra and composing too little, which led to his resignation in 1873. In 1874, he finally fell ill with an “ulcer”. He first wrote about hearing problems in his diary in July 1874: “At times I have misaligned ears and at the same time my head is spinning as if I’m having dizzy spells. The sickness started after a little duck hunt, during which the weather suddenly changed.” [2]

He impressively described the development of acoustic hallucinations, phantom noises, tinnitus and the progression of hearing loss in his diary: “It was in August 1874 when, on one of my usual evening walks through the forest, I suddenly thought I heard such strangely beautiful flute sounds that I stopped in amazement and looked all around for the marvellous flute player. No matter how hard I looked, there was nothing to be seen. I forgot about the incident, but when it happened again the next day, I gave up my walks. But the sensory illusion was repeated later in a closed room and I sought advice from a doctor. A short time later, a terrible ringing in my ears joined in and when I started to play, the piano seemed to be terribly out of tune, especially in the middle registers. I travelled to Prague and was forced to take extreme care of myself and refrain from playing the piano at all. But it was already too late: on October 20^th I lost my hearing completely.” [2]

Completely deaf, he completed the six-part cycle “Má vlast”, which also includes “Vltava”, in the following weeks. He sought advice from Zoufal in Prague, Politzer in Vienna and von Tröltsch in Würzburg, among others. The doctors may have already recognised syphilis as the cause of his deafness, as they prescribed him unspecified “smear cures”, as had been commonly used since early modern times: Paracelsus (1493–1541) had already used mercury in a mixture with lard, turpentine and sulphur, which was rubbed into the patient (“smear cure”) several times a day with full baths in between for a period of 20 days [19]. All attempts to treat Smetana with ointment, noise avoidance, hearing exercises or “electrifying” were completely unsuccessful. In May 1875 he visited his friend Otakar Hostinsky. He describes how he played his latest pieces for him: “He played as excellently as ever before, only at pianissimo he sometimes didn't realise that this or that key didn't make a sound, and at fortissimo he sometimes exaggerated, which he would never have done before.” [2]

Similarly, Adolf Cech describes an experience in which Smetana accompanied his own piano playing: “...Stone-deaf as he was, he never sang in the same key in which he played, but always off key: either higher or lower and usually in loud dissonance.” [2]

It was only when this was pointed out to him that he realised the situation: “For God’s sake, I’'ve come so far that I can no longer tell the difference between the keys!”

But Cech reassured him. He wrote that “his inner ear hears quite correctly, as his ability to compose proves” and explained “...his outer ear, which has not received any impression from the outside for so long, is simply weaned from hearing, and therefore he cannot judge the extent to which he has to strain his vocal cords when singing if he lacks the control of the outer ear over the height or depth of the notes he is singing.” [...]. [2]

In the following years, he increasingly accepted his deafness and further compositions followed. At the end of 1876, he completed the string quartet in E minor “From my life” (Z mého života). In the finale of the quartet, he describes the tinnitus that had occurred shortly before his deafness: “For many weeks before the onset of complete deafness, I was always haunted in the evening between 6 and 7 o’clock by the strong whistle of the chord a sharp-e-c in the highest piccolo position...I have endeavoured to describe this terrible catastrophe in my fate with the bright whistling e in the finale.” [21] (Figure 1 [Fig. 1])

Smetana wrote about the string quartet in a letter in 1878: “My intention was to describe the course of my life in words. The first movement: an inclination towards art in my youth, a romantic mood, an inexpressible longing for something that I could not express in words and could not even imagine in a specific form; but at the same time, in this beginning, the warning of the fate that awaited me and the long-lasting tone in the finale already made itself heard. It contains that fateful whistling in the highest notes, which arose in my ears in 1874 and signalled my incipient deafness. I allowed myself this little game because my destiny lies hidden in it.” [2] (Figure 2 [Fig. 2])

Further compositions followed the years after. For Smetana personally, however, they were characterised by the progression of the underlying illness with aphasic symptoms, acoustic hallucinations, tinnitus, gait disorders and other neurological and psychiatric disorders. After years of increasing mental derangement, Bedrich Smetana finally died in Prague on 12 May 1884.

Syphilis is an almost exclusively sexually transmitted infectious disease caused by the spirochete Treponema pallidum, which according to German law does not have to be reported by name; connatal cases also occur. It initially manifests itself regionally with a typical primary lesion (papule, ulcer) at the site of entry (>90% genitoanal mucosa) and indolent regional lymphadenopathy (primary syphilis). After about 9 to 12 weeks and healing of the primary lesion, the secondary stage follows with bacteraemia, general symptoms (flu-like, fever, headache, others) and a very broad spectrum of skin and mucous membrane symptoms.

Primary and secondary syphilis up to one year after infection are referred to as early syphilis, all other forms are summarised as late syphilis.

If left untreated, secondary syphilis is chronically recurrent for up to a year and can then turn into a latency phase that can last for decades. In the case of an inflammatory reaction against the pathogens with granulomatous symptoms in the late phase of the disease, this is referred to as tertiary syphilis.

Syphilis can affect the central nervous system, the eyes and the vestibulocochlear system at any stage of infection, in isolation, in combination and with a very broad spectrum of clinical signs and manifestations. Untreated cases can lead to persistent dysfunction and death [6].

As early as the 16^th century, syphilis was referred to as a venereal disease because the goddess of love, Venus, was considered to be responsible for its occurrence. The term syphilis became established in the 18^th century. Until then, there were several hundred different names, including disrespectful terms such as “morbus gallicus” and others. Until the middle of the 19^th century, syphilis was trivialised as the price of amorous pleasures and dismissed as a “gallant” disease [19].

After years of research, Fritz Schaudinn and Erich Hoffmann finally succeeded in 1905 at the Charité hospital in Berlin in obtaining the first microscopic evidence of syphilis spirochetes [24].

According to the “pre-Columbian” hypothesis, the disease already existed in Europe before the end of the 15^th century; according to the “post-Columbian” hypothesis, it was brought from the New World by Columbus in 1493: this thesis is supported by European bone findings from before 1493, in which no evidence of syphilitic changes could be found, in contrast to such from North America. The spread in Europe can be traced back to the war participation of men who had previously travelled with Columbus. The spread to Asia was again “by ship” [1].

In Germany, 7,332 cases of syphilis were reported to the Robert Koch Institute in 2018. As with other sexually transmitted diseases, homosexual men (“MSM”) accounted for the highest proportion [18], while the proportion of heterosexually acquired infections was 15% in 2018. Prostitution contacts were assumed to be a possible source of infection in 131 cases, corresponding to 0.018% of all infections. Females were affected in 6.1% of cases. Among men, the incidence was highest in the 30 to 39 age group, and among women in the 20 to 29 age group.

The highest incidences were in the city states of Berlin and Hamburg, above the national average were Bremen, North Rhine-Westphalia and Hesse, while the lowest incidences were in Mecklenburg-Western Pomerania, Brandenburg and Thuringia. High incidences were also recorded in the cities of Cologne, Frankfurt/M. and Munich. Data is also available on the presumed country of infection: in 93% of cases this was Germany, 54 infections were probably acquired in Spain and 30 in Thailand.

In a long-term comparison, the number of infections in newborns and children in Germany is less than 10 per year: this reported figure may be too low, as preventive examinations are not always utilised due to a lack of health insurance, for example, and a number of unreported cases can therefore be assumed. According to WHO estimates, there were 661,000 congenital syphilis infections worldwide in 2016, which led to around 355,000 birth defects including 200,000 stillbirths.

Co-infection with HIV was present in 33% of cases in 2018, more frequently in MSM in 46% than in cases with a heterosexual route of infection (6.7%). Although there is no data on the actual incidence of neurosyphilis, it is likely to be higher in cases of co-infection with HIV [15], [18], [6].

The diagnosis follows evidence based guidelines. It is based on clinical signs, histopathological findings of the skin and mucous membrane, imaging procedures, organ-related laboratory diagnostics including cerebrospinal fluid diagnostics, direct pathogen detection in early pathogen-rich lesions and the detection of anti-treponema antibodies in serum.

In principle, the following applies: In the event of a clinical suspicion, a pathogen-specific screening test is carried out first. If this is positive, a specific confirmation test is carried out. If this is positive, a determination of so-called activity parameters is carried out to differentiate an earlier infection (“seronarcosis”) from syphilis requiring treatment. There is further consensus in the guideline on the diagnosis of very early primary syphilis, in the case of persistent suspicion of syphilis with inconspicuous laboratory results, with regard to the diagnostics to be offered to exclude other infectious diseases and on the procedure in the case of reinfection or reactivation [6].

With bacteraemia, neurosyphilis can occur at any other time of the disease. A distinction is generally made between 5 syndromes as a result of neuroinvasion. Early manifestations involving the meninges, cerebrospinal fluid and vascular structures are recognised:

• Asymptomatic neurosyphilis
• Syphilitic meningitis
• Meningovascular neurosyphilis

Late forms are progressive paralysis and tabes dorsalis, which often occur decades after the primary infection and are characterised by the involvement of central and spinal structures.

Otogenic and ocular syphilis cannot be clearly assigned to the syndromal complexes mentioned, but are subject to the same treatment criteria as neurosyphilis [15].

The syndromes are characterised by different constellations of findings. In syphilitic meningitis, the lesions of the VII and VIII cranial nerves should also be emphasised. In the meningovascular form, visual disturbances occur most frequently (50%), hearing loss in 20% of cases, as well as various motor deficits and organic brain psychosyndromes [15].

Progressive paralysis is a chronic progressive encephalitis with cognitive deficits, psychotic episodes, epileptic seizures, loss of speech, dementia, etc. Tabes dorsalis is a chronic progressive dorsal radiculoganglionitis with secondary spinal cord damage and the resulting functional deficits including pain symptoms [15].

The diagnosis of neurosyphilis has a highly complex structure and should be considered if there are unclear neurological, ocular and/or otogenic symptoms in the presence of proven syphilis.

The staged course of syphilis also applies in cases of comorbid HIV infection, but there are more atypical and severe courses, including more frequent neurosyphilis with neurocognitive deficits [17], [26].

Treatment is generally antibiotic with penicillin, in the case of a penicillin allergy with doxycycline or ceftriaxone. Dosage, method of application and duration of therapy are defined for the different stages of the disease and also for neurosyphilis. This is supplemented by treatment of the clinical symptoms, which is usually guideline-based. In principle, therapy also follows these key points in the case of comorbid HIV disease [6].

The pathophysiology of otosyphilis is probably based on different mechanisms. The VIII^th cranial nerve can be attacked outside and inside the cerebrospinal fluid space, the cochleovestibular apparatus can be affected as well as the petrous bone. In the early stages, the pathogens can infiltrate the perilymph of the inner ear, resulting in inflammation of the bony labyrinth later on. In cases of restriction to the cochlea and labyrinth, symptoms similar to those of Menière’s disease with an unremarkable CSF would be expected. Another hypothesised mechanism is the spread of the pathogens via the CSF and the subarachnoid space and via the cochlear aqueduct into the perilymph of the inner ear – in such cases a conspicuous CSF finding would be expected. Osteitis and periosteitis were also found in the petrous bone, as well as involvement of the ossicles. On the basis of endarteritis, the vascular supply could be impaired, resulting in bone necrosis. These different mechanisms would also explain divergent constellations of findings in inter-individual comparisons [16], [23], [27].

The following data on the epidemiology of otosyphilis are significant: Jackson et al. [15] report 166 cases with syphilitic ear manifestations on the basis of 41,187 diagnosed syphilis cases. They differentiate between 12 confirmed cases, 27 probable and 127 possible cases. In this group, the occurrence of ear symptoms was independent of HIV status. Similar to neurological and eye symptoms, ear symptoms occur more frequently in cases with an unclear duration of illness and in late stages.

The diverse symptomatology of otosyphilis is evidenced by the following data:

Garcia-Berrocal et al. [12] apodictically describe sudden or rapidly progressive bilateral sensorineural hearing loss with or without mild vestibular symptoms as a typical ear manifestation of late syphilis; the hearing loss is often asymmetrical. In early syphilis, hearing loss may be less common; fluctuating hearing loss also occurs, especially in tertiary syphilis.

Yimtae et al. [32] differentiated the clinical manifestations of confirmed syphilitic ear diseases on the basis of 85 cases. According to this study, hearing loss was present in 90.6%, tinnitus in 72.9% and dizziness in 52.9%. Gradual hearing loss was reported by 75.3% of patients, bilaterally symmetrical in 42.4% and bilaterally asymmetrical in 32.9%. Sudden hearing loss was present in 23.5%, symmetrically in 10.6%, asymmetrically in 9.4%. Unilateral hearing loss was present in 3.5% of patients. Acute vertigo symptoms were present in one patient. Initial hearing was normal in 9.4%, 32.9% had a hearing loss of 21 to 40 dB, 27.1% had a hearing loss of 41 to 55 dB, 14.1% had a hearing loss of 56 to 74 dB and 10.5% had a hearing loss of >75 dB. The hearing threshold curves in this group were very different, with 42.3% most frequently showing a decreasing curve in the tone audiogram.

Theeuwen et al. [29], in their report comprising 12 cases, also see a great heterogeneity in the symptoms complained of and the clinical findings.

According to Ogungbemi et al. [20], long-lasting disease progression can also lead to sound conduction disorders based on a compromise of the ossicular chain.

The precise diagnosis of otosyphilis is made more difficult by the fact that the symptoms of the individual case appear to mimic other ear diseases. In clinical practice, this means that other possible causes must be considered and possibly ruled out; usually, an interdisciplinary approach is required, including early diagnostic imaging, which, however, is not specifically conclusive [1], [4], [8], [28], [31].

The treatment of otosyphilis follows the same principles as syphilis and neurosyphilis in general. In comorbid HIV-positive patients, the immunosuppressive potency of steroids must be taken into account when using them symptomatically [5], [7].

The prognosis of treated otosyphilis depends on the individual stage of the disease and thus also on the extent of the possibly irreversible tissue changes. An older study [7] describes very strongly divergent treatment results for ear syphilis, according to which the overall prognosis is “poor”. Song et al. [26] report casuistically on an HIV-positive homosexual man with asymmetrical, fluctuating hearing loss and bilateral vestibular loss. After penicillin therapy, the hearing was slightly improved at the follow-up examination after 6 months, but the vestibular symptoms were not. He et al. [13] describe a reversible hearing loss.

Feldmann [10] concluded the following from his own research of diary entries in the Smetana Museum in Prague: Smetana initially suffered from syphilitic angina, after about 16 weeks from a generalised exanthema and two weeks later developed ear symptoms, namely hearing loss, ringing in the ears and dizziness. After ten years, he finally died of taboparalysis. According to Feldmann, the cause of the deafness was syphilis, and the autopsy findings also support this assessment.

Borik and Borikova [3] painted a different picture and demanded “that the erroneous and unsubstantiated claim of a Luetian disease of our greatest and most deserving composer be erased from the scientific literature”. The authors describe that Smetana suffered an explosion trauma in early childhood and complained of ear problems at the age of 18. He loved very noisy ensemble playing with several pianos and thus exposed himself to severe acoustic stress. He also used firearms when hunting and was therefore regularly exposed to high sound levels. He had also described acoustic hallucinations as early as the age of 38. At the age of 46, his poor hearing had already been noticed by friends and he had spoken very loudly, “as is usual with hearing impaired people”. He was often ill and was advised by a doctor as early as 1872 that he should “bury himself in the forest for a few weeks so that ... his torn nerves could recover”. His physical condition was also so good in 1881 that he was still conducting.

Both according to Feldmann’s assessment [10], [11] as well as according to the current state of knowledge on noise-induced hearing loss, these considered mechanisms can be ruled out as the cause of Smetana’s ear disease. Smetana’s “auditory illusions” (hearing a double voice singing in G major with organ accompaniment) were analysed by Borik and Borikova [3] with regard to their cause, namely as part of a luetic psychiatric symptomatology. They regarded the (luetic) skin symptoms as part or consequence of a longer history of repeated ulcers of the skin, the syphilitic angina was regarded as a recurrence of previously suffered pharyngeal diseases, the progressive paralysis was regarded as unproven without further discussion. Feldmann [9] responded to the work of Borik and Borikova with a further objective and precise summary of the facts. After all, today it is recognised that Smetana’s illness was syphilis.

Syphilis is – today again – a relevant infectious disease with a potentially considerable burden of disease for the person affected, including death. The casuistic view of the illness of the Czech national composer Bedrich Smetana impressively demonstrates this from a historical perspective.

The epidemiological data on congenital syphilis is questionable. In Germany, preventive diagnostics should also be made available to pregnant women with undefined legal status and/or no health insurance.

For comorbid HIV-positive patients, no other aspects apply to therapy than for persons not infected with HIV; only in the symptomatic use of steroids in the case of otosyphilis their potentially immunosuppressive effect has to be taken into account.

Beyond primary prevention, the late forms of syphilis can be avoided through early diagnosis and treatment.

According to current epidemiological data, otosyphilis is a rarity, possibly because it is “underdiagnosed”. For clinical audiological use, it makes sense to seek specific clarification in cases of acute bilateral hearing loss and (asymmetrical) fluctuating hearing.

Bedrich Smetana's fate with complex symptoms leading to death, including otosyphilis, is avoidable today.

The authors declare that they have no competing interests.