Lesion of the ear maze with syphilis
Last reviewed: 23.04.2024
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Syphilitic lesions of the ear maze are characterized by a complex pathogenesis, some aspects of which remain unexplored to the present day. Many authors treat these lesions as one of the manifestations of neurosyphilis (neirolabirintit), caused by syphilitic changes in the liquid media of the inner ear (similar to changes in syphilis in the cerebrospinal fluid). Other authors associate violations of the functions of the inner ear with syphilitic meningoencephalitis and vasculitis arising in labyrinth-dependent structures of the brain and in vessels feeding the vagina of the auditory and vestibular nerves.
Cochleovestibular disorders can occur at any stage of acquired or congenital syphilis, but most often they occur in secondary syphilis and worsen with progressive progress in later periods of the disease. Most often, cochleovestibular disorders in the secondary period occur with spirochaemia and generalization of the process, which is based on a decrease in immunity, increased permeability of the BBB and penetration of pale treponem and their exotoxins in the cerebrospinal fluid and brain substance.
[Symptoms of defeat of the ear maze with syphilis
According to different authors, hearing damage at different stages of syphilis 25-30 years ago was observed in 13-14% of patients. According to NN Reštejn (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 in four forms - super-acute apoplectiform, acute, subacute and latent.
The superficial form is characterized by sudden dizziness, a sharp imbalance, a coarse spontaneous nystagmus, severe noise in one or both ears, rapidly progressive deafness and deafness. At this stage, sharply positive serological reactions occur both in the blood and in the CSF. With this form of syphilitic labyrinthitis, complete and irreversible shutdown of auditory and vestibular functions occurs.
The acute form of syphilitic cochleovestibular disorders manifests itself slowly but distinctly differentiating signs of cochleopathy (tinnitus, progressive deafness, gradually increasing dizziness and disturbance of balance). Vestibular disturbances are amplified by provocative tests and optokinetic stimulations, auditory disorders - under noise conditions, the intelligibility of speech sharply worsens. With late-onset treatment, deafness may occur.
Subacute form is characterized by the latent course of syphilitic inflammation of the labyrinth, in which auditory and vestibular disorders remain undetected for a long time. A few months later, a slight ringing appears in the ears, mostly at night, then a few weeks or months later, a decrease in the acuity of the tonal hearing and deterioration in the intelligibility of speech are added. Vestibular symptoms are absent or weakly expressed mainly by instability of equilibrium in the dark.
Latent syphilitic neural labyrinthitis occurs without labyrinthine symptoms. The auditory function gradually, within months and years, is steadily dying out, down to total deafness.
A specific feature of syphilitic hearing loss is a sharp disruption of the tissue (bone) conductivity of sound, until its complete loss. At the same time, the air type of sound transmission can be maintained at a satisfactory level. This change in the function of tissue sound conduction in syphilis has not yet been fully explained. The current opinion that the phenomenon of tissue sound loss is determined by the peculiar neurotropy of the syphilitic neurotoxin and the structural specific changes in the bone capsule of the labyrinth, which acquires the properties of an amorphous substance that does not conduct a sound, is not convincing enough.
In congenital syphilis, the damage to the inner ear occurs in 15-20% of cases and proceeds in the form of explicit or hidden forms of neurolabirintite, syphilitic meningitis and meningoradiculitis of the pre-cochlear nerve. Hearing impairment in congenital syphilis in children is found after the age of one year, but other obvious birth defects of a lyuetic genesis cause a suspected internal ear disease. Diagnosis of congenital syphilis in children is facilitated by the knowledge of the manifestations of certain specific syndromes.
Denis-Marfan syndrome - CNS involvement in children with congenital syphilis: spastic paralysis of the limbs, mental retardation, often - fever, convulsive seizures, cataracts, nystagmus. When combining nystagmus with fever, suspicions of banal labyrinthitis may occur, especially if these symptoms are observed against a background of chronic suppurative otitis media, often in patients with this pathological condition.
Anneber's symptom is a sign of early congenital syphilis: paroxysmal spontaneous nystagmus with dizziness, arising with increased pressure in the external auditory canal (pseudofistral symptom).
The Hutchinson syndrome (triad) is parenchymal keratitis, syphilitic labyrinthitis, chisel-shaped or barrel-shaped anterior superior incisors.
Usually late congenital syphilitic labyrinthitis occurs during puberty. In adults suddenly there is complete deafness. The process is due to the infiltrative inflammation of the membranous labyrinth structures and the degeneration of the auditory nerve. In the same period, pathological changes develop in the subcortical and cortical auditory centers.
Where does it hurt?
Diagnosis of syphilitic lesions of the ear maze
The diagnosis of syphilitic labyrinthitis is based on "causeless" hearing loss in combination with the symptoms and syndromes described above and the confirmation of its specific serological tests. Difficulties in diagnosis arise with seronegative forms of syphilis. In this case, a trial antisyphilitic treatment is used.
What do need to examine?
How to examine?
Treatment of syphilitic lesions of the ear maze
Treatment of syphilitic lesions of the ear maze is included in the planned therapy of general syphilitic infection. When vestibular crises prescribed appropriate symptomatic treatment, for the prevention and treatment of sensorineural hearing loss, a complex antineuritic is prescribed according to generally accepted patterns. Intensive use of antibiotics and mass death of treponemus with the release of their endotoxin may be accompanied by the so-called Herxheimer reaction, manifested by exacerbation of inflammatory reactions in places of microorganisms, including in the inner ear, which can provoke an acute attack of labyrinthopathy and cause complete exclusion of the auditory and vestibular apparatus. Usually this reaction occurs from 3 hours to 3 days after the start of a specific treatment with massive doses of antibiotic, therefore, simultaneously with the use of antibiotic therapy, neuroprotectors, B group vitamins, antihypoxants and antihistamines should be prescribed.