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: 6109891
• 负责人: G P SIMS
• 金额: $ 13.5万
• 依托单位: MEHARRY MEDICAL COLLEGE
• 依托单位国家: 美国
• 财政年份: 1997
• 资助国家: 美国
• 起止时间: 1997-04-01 至 2001-03-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6109891
• 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结果 镰状细胞贫血与感觉神经性听力损失的关系和 异常听觉功能。 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 并占听力损失和/或听觉功能障碍的位置 镰状细胞危机期间的镰状细胞患者 对照（非危机）和一般镰状细胞种群。 一个 全面的听力学评估过程，包括无创的 听觉诱发的反应和耳鼻喉电器电生理学 技术将被利用。 各种调查人员已经评估了 外围听觉敏感性，结果差异很大。 一个 各种案例研究记录了中耳，人工耳蜗，VIII神经， 和镰状细胞患者的中央听觉参与。 不同的 已经报道了模式和听力损失程度 与镰状细胞疾病相关，从双侧造成深远的损失 随着时间的流逝，部分恢复，轻度至中度损失 主要在高频中。 有限的电生理学 镰状细胞患者的评估已出现在文献中。 这 该提案的具体目的是：调查关系 在镰状细胞状态（控制与危机）之间；听觉站点 赤字（耳蜗与第八神经）；和神经/发育 与异常听觉功能有关的后果；有效定义 一个范式可预测相关的大量听觉系统缺陷 危机情节的数量，持续时间和严重性；并发展 适当的听力学评估的经济和高效模型 和镰状细胞贫血患者的治疗。