Friedman E M, Herer G R, Luban N L, Williams I
Ann Otol Rhinol Laryngol. 1980 Jul-Aug;89(4 Pt 1):342-7. doi: 10.1177/000348948008900409.
The present investigation examined the effects of sickle cell anemia on threshold hearing. The study included 43 homozygous sickle cell anemia patients, ages 7-18 years, and 23 age-matched controls with documented normal hemoglobin. Both the study and control groups received otologic and audiologic examinations. Bilaterally normal hearing was found in 88% of the sickle cell subjects. Unilateral or bilateral mild high frequency sensorineural hearing loss was demonstrated in 12% (5 of 43). The control subjects revealed no hearing loss. The sickle cell group, therefore, exhibited a higher than usual incidence of hearing loss. Further, three of the five subjects with hearing loss evidenced central nervous system involvement, resulting in a probability of < 0.025 that this relation might occur by chance. Periodic hearing evaluations of children with sickle cell anemia should be performed to identify those with auditory complications and those who possibly may have CNS involvement.
本研究调查了镰状细胞贫血对听力阈值的影响。该研究纳入了43名年龄在7至18岁的纯合子镰状细胞贫血患者，以及23名年龄匹配、血红蛋白记录正常的对照者。研究组和对照组均接受了耳科和听力学检查。88%的镰状细胞受试者双耳听力正常。12%（43名中的5名）表现为单侧或双侧轻度高频感音神经性听力损失。对照受试者未发现听力损失。因此，镰状细胞组的听力损失发生率高于正常水平。此外，5名听力损失受试者中有3名有中枢神经系统受累的证据，这种关系偶然发生的概率<0.025。应对镰状细胞贫血患儿进行定期听力评估，以识别出有听觉并发症以及可能有中枢神经系统受累的患儿。
