耳硬化症的遗传倾向及其防治的探讨

目的 探讨具有遗传倾向的耳硬化症的防治。方法 回顾分析６个家庭１４例患者的诊断、治疗情况及追踪结果，根据活动型耳硬化症的病理特征，提出防治的可行性方案。结果 ６个家庭１４例患者均经听力学和／或手术及病理学确诊。１１例作了镫骨手术，其中１０例选听力损失较重侧行手术，１例双耳先后作手术，听力均有不同程度的改善，平均提高２３ｓｄＢ，３例末行镫骨手术；１例末接受治疗。２例已建议作手术，现在口服硫酸软骨素的

The hereditary liability of otosclerosis and the strategies for treatment and prevention]

OBJECTIVE: Otosclerosis is a disease of hereditary liability. It might be related to an autosomal dominant inheritance. The genetic penetrance is determined by multifactorial influences. This paper was designed to study the reasonable strategies for prevention and treatment of this disease. METHODS: A retrospective review derived from data of the management and follow-up of 14 cases in 6 families. According to the special pathological features of active phase of the otospongiosis, the suitable strategies for prevention and treatment of this disease were suggested. RESULTS: Diagnosis of 14 cases were confirmed by audiological, operative and/or pathological examinations. Eleven of fourteen cases underwent stapedectomy, in which 10 cases had the surgery on the ear with severer hearing loss, and one case on both sides. In all of the surgical cases, hearing levels improved significantly. Three cases have not yet received the stapedectomy, in which two cases scheduled for the surgery are currently treated by chondroitin sulfate and one case refused the surgical treatment due to financial shortage. CONCLUSION: Examination of the ear function at regular intervals (6-12 months) is recommended for the adults among the family members with high-incidence of otosclerosis. Audiological and radiological examination play an important role in early diagnosis and treatment. Attention should be paid to the medicine for management and prevention of this disease. Stapedectomy is an optimal choice for clinical otosclerosis not only due to its capacity to improve the hearing level but also to prevent the advance of hearing loss. Hearing aid is the suitable choice if stapedectomy is contraindicated.