HEARING LOSS AND AUDITORY FUNCTION IN SICKLE CELL DISEASES

镰状细胞病中的听力损失和听觉功能

• 批准号: 5213675
• 负责人: G P SIMS
• 金额: --
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/5213675
• 关键词: adolescence (12-20); adult human (21+); audiometry; auditory discrimination; auditory reflex; auditory stimulus; auditory threshold; behavior test; child (0-11); electrophysiology; epidemiology; evoked potentials; hearing tests; human subject; medical complication; pathologic process; sensorineural hearing loss; sickle cell anemia; sickle cell crisis

The primary concern of this investigation is the consequential relationship of sickle cell anemia to sensorineural hearing loss and abnormal auditory function. The ultimate goal of this project is to investigate and define this relationship by determining the prevalence and predominate site of hearing loss and/or auditory dysfunction in sickle cell patients during sickle cell crisis, with the disease under control (non-crisis) and in the general sickle cell population. A comprehensive audiological evaluation process, including non-invasive auditory evoked responses and otacoustic emissions electrophysiological techniques will be utilized. Various investigators have assessed peripheral auditory sensitivity, with a wide disparity of results. A variety of case studies have documented middle ear, cochlear, VIII nerve, and central auditory involvement of sickle cell patients. Different patterns and degrees of hearing loss have been reported in association with sickle cell disease, ranging from profound losses bilaterally with partial recovery over time, to mild to moderate unilateral losses predominately in the high frequencies. Limited electrophysiological assessment of sickle cell patients has appeared in the literature. The specific aims of this proposal are: to investigate the relationship between sickle cell status (control versus crisis); site of auditory deficit (cochlea versus eighth nerve); and neurological/developmental consequences related to abnormal auditory function; to effectively define a paradigm predictive of significant auditory system deficits in relation to the number, duration and severity of crisis episodes; and to develop an economical and efficient model for appropriate audiological assessment and treatment of sickle cell anemia patients.

这项调查的主要关注点是由此产生的后果 镰状细胞性贫血与感音神经性听力损失的关系 听觉功能异常。 该项目的最终目标是 通过确定患病率来调查和定义这种关系 以及听力损失和/或听觉功能障碍的主要部位 镰状细胞危机期间的镰状细胞患者，该疾病处于 控制（非危机）和一般镰状细胞群体。 一个 全面的听力学评估过程，包括非侵入性 听觉诱发反应和耳声发射电生理学 将使用技术。 不同研究者评估了 外周听觉敏感性，结果差异很大。 一个 各种案例研究记录了中耳、耳蜗、VIII 神经、 和镰状细胞病患者的中枢听觉受累。 不同的 相关报道报道了听力损失的模式和程度 患有镰状细胞病，从双侧严重损失到 随着时间的推移部分恢复，至轻度至中度单方面损失 主要集中在高频。 电生理学有限 对镰状细胞病患者的评估已出现在文献中。 这 该提案的具体目标是：调查两者之间的关系 镰状细胞状态之间（控制与危机）；听觉部位 缺陷（耳蜗与第八神经）；和神经/发育 与听觉功能异常相关的后果；有效地定义 预测相关听觉系统显着缺陷的范式 危机事件的数量、持续时间和严重程度；并发展 用于适当听力学评估的经济且有效的模型 以及镰状细胞性贫血患者的治疗。