Berlucchi Marco, Soresina Annarosa, Redaelli De Zinis Luca O, Valetti Luisa, Valotti Roberta, Lougaris Vassilios, Meini Antonella, Salsi Daria, Nicolai Piero, Plebani Alessandro
Department of Pediatric Otorhinolaringology, Spedali Civili, Brescia, Italy.
J Pediatr. 2008 Aug;153(2):293-6. doi: 10.1016/j.jpeds.2008.03.008.
To evaluate the hearing function in patients affected by primary antibody deficiency disorders. Forty-seven patients, 25 of whom were affected by X-linked agammaglobulinemia and 22 of whom were affected by common variable immunodeficiency were evaluated with audiologic tests that included pure tone audiometry, acoustic immittance assessment and auditory brainstem-evoked response. Eighteen patients (38%), 7 with X-linked agammaglobulinemia and 11 with common variable immunodeficiency, showed sensorineural hearing loss, bilateral in 12 and unilateral in 6. Our data underline the high frequency of hearing loss in patients with antibody deficiency and suggest that a systematic audiologic evaluation should be part of the clinical care of these patients.
评估原发性抗体缺陷疾病患者的听力功能。对47例患者进行了听力测试评估，其中25例患有X连锁无丙种球蛋白血症，22例患有常见变异型免疫缺陷，听力测试包括纯音听力测定、声导抗评估和听觉脑干诱发电位。18例患者（38%）出现感音神经性听力损失，其中7例患有X连锁无丙种球蛋白血症，11例患有常见变异型免疫缺陷，12例为双侧听力损失，6例为单侧听力损失。我们的数据强调了抗体缺陷患者听力损失的高发生率，并表明系统的听力评估应成为这些患者临床护理的一部分。
