Kryukov A I, Garov E V, Zelikovich E I, Zagorskaya E E, Kaloshina A S, Garova E E, Panasova A S, Kovtun O V, Khublaryan A G
Sverzhevsky Research Clinical Institute of Otorhinolaryngology, Moscow, Russia.
Pirogov Russian National Research Medical University, Moscow, Russia.
Vestn Otorinolaringol. 2023;88(6):5-14. doi: 10.17116/otorino2023880615.
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.
To evaluate the features and results of surgical rehabilitation of hearing loss in patients with osteogenesis imperfecta.
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.
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.
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.
成骨不全症（OI）是一种先天性骨质疏松症。根据OI的类型，患者会出现各种类型的听力损失。根据听力损失的类型和程度，这类患者会采用各种听力康复方法。
评估成骨不全症患者听力损失的手术康复特点及效果。
2009年至2022年期间，该科室共进行了2221例初次镫骨手术，其中21例患者中的23例（1.04%）为成骨不全症患者。女性14例，男性7例。根据听阈图，19例患者为双侧听力损失，2例为单侧听力损失。9例为传导性听力损失，14例为混合性听力损失。骨导平均阈值为22.7±8.04dB，气骨导间距（ABG）为36.1±5.3dB。根据颞骨CT，所有患者均显示双侧听小骨密度对称性降低，7例患者骨迷路密度存在广泛不均匀降低区域，高达+500 - +1000HU。21例患者接受了23次手术：21例为激光辅助镫骨手术，2例为听骨成形术。
术后6个月骨导阈值平均为24.6±8.2dB，ABG为12.1±2.9dB。在言语频率下ABG≤10dB的患者占30.5%，ABG≤20dB的患者占95%。术后12个月或更长时间后，听力学参数无变化。
成骨不全症患者传导性和混合性听力损失的镫骨手术在功能上是有效的。在使用双膦酸盐与氟化钠、钙和维生素D制剂进行治疗后，当脱矿区域密度达到1000HU并使用激光辅助进行手术时，可取得最佳效果。考虑到颞骨骨结构的脱矿情况，建议使用自体软骨镫骨假体来恢复声音传导，或用自体组织覆盖其他假体的附着区域，以防止砧骨长脚坏死并稳定长期功能结果。
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