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Lesions of the ear labyrinth in syphilis
Last reviewed: 04.07.2025
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Syphilitic lesions of the ear labyrinth are characterized by a complex pathogenesis, some aspects of which remain unexplored to this day. Many authors interpret these lesions as one of the manifestations of neurosyphilis (neurolabyrinthitis), caused by syphilitic changes in the fluid environments of the inner ear (by analogy with changes in syphilis in the cerebrospinal fluid). Other authors associate dysfunctions of the inner ear with syphilitic meningoencephalitis and vasculitis, arising in the labyrinth-dependent structures of the brain and in the vessels that feed the sheaths of the auditory and vestibular nerves.
Cochleovestibular disorders may occur at any stage of acquired or congenital syphilis, but most often they occur in secondary syphilis and worsen with its progressive course in later periods of the disease. Most often, cochleovestibular disorders in the secondary period occur with spirochetemia and generalization of the process, which is based on decreased immunity, increased permeability of the BBB and penetration of pale treponemas and their exotoxins into the cerebrospinal fluid and brain matter.
[ Symptoms of damage to the ear labyrinth in syphilis
According to various authors, hearing impairment at different stages of syphilis 25-30 years ago was observed in 13-14% of patients. According to N.N. Reshteyn (1986), about 20 years ago, hearing impairment in patients with contagious forms of syphilis accounted for 43.4%. Clinical manifestations of syphilitic cochleovestibular disorders can be classified into four forms - hyperacute apoplectiform, acute, subacute and latent.
The hyperacute form is characterized by sudden dizziness, severe imbalance, severe spontaneous nystagmus, loud noise in one or both ears, rapidly progressing hearing loss and deafness. At this stage, sharply positive serological reactions appear both in the blood and in the CSF. In this form of syphilitic labyrinthitis, complete and irreversible shutdown of the auditory and vestibular functions occurs.
The acute form of syphilitic cochleovestibular disorders is manifested by slowly increasing but clearly differentiated signs of cochleopathy (tinnitus, progressive hearing loss, gradually increasing dizziness and balance disorders). Vestibular disorders are aggravated by provocative tests and optokinetic stimuli, auditory disorders - in noise conditions, speech intelligibility is sharply impaired. If treatment is started late, deafness may occur.
The subacute form is characterized by a latent course of syphilitic inflammation of the labyrinth, in which auditory and vestibular disorders remain unnoticed for a long time. After months, a slight ringing in the ears appears, mainly at night, then after several weeks or months, a decrease in the acuity of tonal hearing and a deterioration in speech intelligibility join in. Vestibular symptoms are absent or weakly expressed, mainly by instability of balance in the dark.
Latent syphilitic neurolabyrinthitis occurs without labyrinthine symptoms. The auditory function gradually, over months and years, steadily fades, up to complete deafness.
A characteristic feature of syphilitic hearing loss is a sharp impairment of tissue (bone) sound conduction, up to its complete loss. At the same time, the air type of sound conduction may be maintained at a satisfactory level. The specified change in the function of tissue sound conduction in syphilis has not yet found an exhaustive explanation. The existing opinion that the phenomenon of loss of tissue sound conduction is determined by the peculiar neurotropism of the syphilitic neurotoxin and structural specific changes in the bone capsule of the labyrinth, acquiring the properties of an amorphous substance that poorly conducts sound, is not very convincing.
In congenital syphilis, inner ear damage occurs in 15-20% of cases and occurs in the form of obvious or latent forms of neurolabyrinthitis, syphilitic meningitis, and meningoradiculitis of the vestibular-cochlear nerve. Hearing impairment in congenital syphilis in children is detected after one year of age, but other obvious congenital defects of luetic genesis make one suspect a disease of the inner ear. Knowledge of the manifestations of some specific syndromes facilitates the diagnosis of congenital syphilis in children.
Denis-Marfan syndrome is a lesion of the central nervous system in children with congenital syphilis: spastic paralysis of the limbs, mental retardation, often fever, seizures, cataracts, nystagmus. When nystagmus is combined with fever, suspicions of the presence of banal labyrinthitis may arise, especially if these signs are observed against the background of chronic purulent otitis media, which is common in patients with this pathological condition.
Ennebert's symptom is a sign of early congenital syphilis: paroxysmal spontaneous nystagmus with dizziness that occurs with an increase in pressure in the external auditory canal (pseudo-fistula symptom).
Hutchinson's syndrome (triad) - parenchymatous keratitis, syphilitic labyrinthitis, chisel-shaped or barrel-shaped anterior upper incisors.
Usually, late congenital syphilitic labyrinthitis manifests itself during puberty. In adults, complete deafness suddenly appears. The process is caused by infiltrative inflammation of the structures of the membranous labyrinth and degeneration of the auditory nerve. During this period, pathological changes in the subcortical and cortical auditory centers develop.
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Diagnosis of syphilitic lesions of the ear labyrinth
The diagnosis of syphilitic labyrinthitis is based on "causeless" hearing loss in combination with the symptoms and syndromes described above and confirmed by specific serological tests. Difficulties in diagnosis arise in seronegative forms of syphilis. In this case, a trial antisyphilitic treatment is used.
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Treatment of syphilitic lesions of the ear labyrinth
Treatment of syphilitic lesions of the ear labyrinth is included in the planned therapy of general syphilitic infection. In case of vestibular crises, appropriate symptomatic treatment is prescribed; for the prevention and treatment of sensorineural hearing loss, complex antineuritic treatment is prescribed according to generally accepted schemes. Intensive use of antibiotics and mass death of treponemas with the release of their endotoxin can be accompanied by the so-called Herxheimer reaction, which is manifested by an exacerbation of inflammatory reactions in the places of microorganisms, including in the inner ear, which can provoke an acute attack of labyrinthine pathology and cause a complete shutdown of the auditory and vestibular apparatus. Usually, this reaction occurs from 3 hours to 3 days from the beginning of specific treatment using massive doses of antibiotics, therefore, neuroprotectors, B vitamins, antihypoxants and antihistamines should be prescribed simultaneously with antibiotic therapy.