纯合子镰状细胞病的听觉功能评估。

The evaluation of auditory function in homozygous sickle cell disease.

作者信息

Forman-Franco B, Karayalcin G, Mandel D D, Abramson A L

机构信息

Department of Otolaryngology and Communicative Disorders, Long Island Jewish-Hillside Medical Center, New Hyde Park, NY 11042, USA.

出版信息

Otolaryngol Head Neck Surg. 1982 Nov-Dec;90(6):850-6. doi: 10.1177/019459988209000630.

DOI:10.1177/019459988209000630

PMID:10994441

Abstract

The most significant clinical manifestations of sickle cell disease (SCD) are those associated with CNS involvement. This disease is systemic in character, with the tissues of the CNS being the most sensitive to even temporary anoxia. For this reason, the incidence of peripheral and central auditory dysfunction in patients with SCD may be greater than in the "normal" population. We examined the peripheral auditory function of 54 patients with homozygous sickle cell disease and the central auditory function of 28 of these patients. Results were compared with a control population. Peripheral hearing loss was found in 11% (6) of the SCD population. Central auditory testing indicated that 46% (13) of the sickle cell population had mild central auditory dysfunction not involving Heschl's gyrus. This study suggests a higher than usual incidence of peripheral and central auditory impairment in sickle cell disease.

摘要

镰状细胞病（SCD）最显著的临床表现是那些与中枢神经系统受累相关的表现。这种疾病具有全身性，中枢神经系统的组织对哪怕是短暂的缺氧最为敏感。因此，SCD患者外周和中枢听觉功能障碍的发生率可能高于“正常”人群。我们检查了54例纯合子镰状细胞病患者的外周听觉功能以及其中28例患者的中枢听觉功能。将结果与对照组人群进行了比较。在SCD人群中，11%（6例）发现有外周听力损失。中枢听觉测试表明，46%（13例）的镰状细胞病患者有轻度中枢听觉功能障碍，且未累及颞横回。这项研究表明，镰状细胞病中外周和中枢听觉损害的发生率高于正常情况。