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1.
Vestn Otorinolaringol ; 88(6): 5-14, 2023.
Article in Russian | MEDLINE | ID: mdl-38153887

ABSTRACT

Osteogenesis imperfecta (OI) is a form of congenital osteoporosis. Depending on the type of OI, patients experience various types of hearing loss. Depending on the type and degree of hearing loss, various methods of hearing rehabilitation are used in this category of patients. OBJECTIVE: To evaluate the features and results of surgical rehabilitation of hearing loss in patients with osteogenesis imperfecta. MATERIAL AND METHODS: During the period from 2009 to 2022, 2221 primary stapedoplasty was performed in the department, of which 23 (1.04%) in 21 patients were performed in patients with OI. There were 14 women and 7 men. According to TPA, bilateral hearing loss was detected in 19 patients and unilateral in 2. Conductive hearing loss was observed in 9 cases and mixed - in 14. The average thresholds for bone conduction (BC) were 22.7±8.04 dB, and the bone-air interval (ABG) - 36.1±5.3 dB. According to CT of the temporal bones, all patients showed a bilateral and symmetrical decrease in the density of the auditory ossicles, and in 7 patients there were extensive areas of non-uniform decrease in the density of the bone labyrinth up to +500 - +1000 HU.21 patients underwent 23 operations: in 21 cases stapedoplasty with laser assistance and in 2 cases ossiculoplasty. RESULTS: BC thresholds 6 months after surgery averaged 24.6±8.2 dB, and ABG - 12.1±2.9 dB. Closing of ABG ≤10 dB at spoken frequencies was detected in 30.5%, ABG ≤20 dB - in 95%. After 12 months or more after the operation, no change in the audiological parameters was noted. CONCLUSIONS: Stapes surgery for conductive and mixed hearing loss in OI patients is functionally effective. The best results are achieved after therapy with bisphosphonates with preparations of sodium fluoride, calcium and vitamin D, performing the operation when the density of demineralization zones reaches 1000 HU and using laser assistance. Taking into account the demineralization of the bone structures of the temporal bone, it is recommended to use autocartilaginous stirrup prostheses to restore sound conduction or to cover the attachment area of other prostheses with autologous tissues to prevent necrosis of the long stalk of the incus and stabilize long-term functional results.


Subject(s)
Deafness , Hearing Loss , Osteogenesis Imperfecta , Stapes Surgery , Male , Humans , Female , Osteogenesis Imperfecta/complications , Osteogenesis Imperfecta/diagnosis , Osteogenesis Imperfecta/surgery , Audiometry, Pure-Tone , Hearing Loss/diagnosis , Hearing Loss/etiology , Hearing Loss/surgery , Hearing , Hearing Loss, Conductive/surgery , Deafness/surgery , Bone Conduction , Stapes Surgery/methods , Retrospective Studies , Treatment Outcome
2.
Vestn Otorinolaringol ; 87(5): 70-74, 2022.
Article in Russian | MEDLINE | ID: mdl-36404694

ABSTRACT

Modern literature data are presented on the choice of a drug for hormonal therapy in acute neurosensory hearing loss of various origins, the doses used for systemic therapy, the features and methods of intratympanic administration of glucocorticosteroids, and the evaluation of the effectiveness of treatment with this group of drugs.


Subject(s)
Hearing Loss, Sensorineural , Hearing Loss, Sudden , Humans , Hearing Loss, Sudden/diagnosis , Hearing Loss, Sudden/drug therapy , Dexamethasone , Treatment Outcome , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/drug therapy , Administration, Oral
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