成人依赖输血人群中的去铁胺耳毒性。

Desferrioxamine ototoxicity in an adult transfusion-dependent population.

作者信息

Chiodo A A, Alberti P W, Sher G D, Francombe W H, Tyler B

机构信息

Department of Otolaryngology, University of Toronto, Toronto Hospital, Ontario.

出版信息

J Otolaryngol. 1997 Apr;26(2):116-22.

PMID:9106087

Abstract

OBJECTIVE

The purpose of this study was to identify the incidence of hearing loss in a population of 75 adult (19-68 years old) transfusion-dependent patients with thalassemia major, sickle cell disease, Diamond-Blackfan anemia, and various other hematologic disorders treated with regular transfusion schedules. Ninety-three percent (70/75) of patients had a history of long-term subcutaneous or intravenous desferrioxamine therapy.

METHODS

The patients underwent routine otolaryngologic history and physical examination, along with standard pure-tone audiometry at 250, 500, 1000, 2000, 3000, 4000, 6000, and 8000 Hz, with hearing loss defined as 25 dB or greater at one or more frequencies.

RESULTS

Hearing loss was present in 57% (43/75) of patients. More importantly, hearing loss attributable to desferrioxamine ototoxicity was present in 29% (22/75) of patients. Sixteen patients treated previously with desferrioxamine were switched to the experimental oral chelating agent, L1. Eight of these 16 patients had hearing loss attributable to desferrioxamine, with 5 of these patients worsening with the experimental oral chelating agent L1. Seventy-nine percent (59/75) of patients were thalassemic. Fifty-four percent (33/59) of these thalassemic patients had hearing loss. However, 35% (21/59) of the thalassemic patients had hearing loss attributable to desferrioxamine ototoxicity. All thalassemic patients with desferrioxamine ototoxicity had high-frequency sensorineural hearing loss, with 33% (7/21) having a notch at 6 kHz. In addition, 5% (1/21) had notching at 3 khz. Few of the hearing losses were disabling.

CONCLUSIONS

Management of these patients requires proper dosing of desferrioxamine and transfusion therapy, along with regular monitoring of body iron burden and hemoglobin. In addition, regular otolaryngologic and audiometric follow-up with special care to include the frequencies of 3 and 6 kHz may help recognize and prevent permanent ototoxicity.

摘要

目的

本研究旨在确定75名成年（19 - 68岁）依赖输血的重型地中海贫血、镰状细胞病、先天性纯红细胞再生障碍性贫血以及其他各种接受定期输血治疗的血液系统疾病患者的听力损失发生率。93%（70/75）的患者有长期皮下或静脉注射去铁胺治疗史。

方法

患者接受常规耳鼻喉科病史及体格检查，并进行250、500、1000、2000、3000、4000、6000和8000赫兹的标准纯音听力测定，听力损失定义为一个或多个频率处听力下降25分贝或更多。

结果

57%（43/75）的患者存在听力损失。更重要的是，29%（22/75）的患者存在因去铁胺耳毒性导致的听力损失。16名先前接受过去铁胺治疗的患者改用实验性口服螯合剂L1。这16名患者中有8名存在因去铁胺导致的听力损失，其中5名患者在使用实验性口服螯合剂L1后听力恶化。79%（59/75）的患者为地中海贫血患者。这些地中海贫血患者中有54%（33/59）存在听力损失。然而，35%（21/59）的地中海贫血患者存在因去铁胺耳毒性导致的听力损失。所有因去铁胺耳毒性导致听力损失的地中海贫血患者均为高频感音神经性听力损失，其中33%（7/21）在6千赫兹处有切迹。此外，5%（1/21）在3千赫兹处有切迹。很少有听力损失导致残疾。