Abstract：
OBJECTIVE: To evaluate otorhinolaryngologic findings and the relationship between aminoglycoside (AG) exposure and hearing loss in paediatric patients with cystic fibrosis (cwCF). We also aimed to investigate the genetic predisposition to AG ototoxicity by screening for m.1555A>G mutations.
METHODS: CwCF who underwent otorhinolaryngologic and audiologic examinations were retrospectively included. Clinical characteristics, ear-nose-throat related symptoms, and a history of ototoxic drug exposure were recorded. m.1555A>G mutations were retrospectively screened among patients with audiologic evaluations.
RESULTS: Two hundred thirty-four cwCF were included in this study with a median age of 10.7 (range, 6.8-14.2) years. Nasal obstruction (14.1%) was the most common symptom. Fifty-two (22.2%) patients had chronic rhinosinusitis (CRS) with nasal polyps (CRSwNP). There was a positive correlation between CRSwNP and the symptom of nasal obstruction (r:.234, p < .001), snoring (r:.179, p = .006), and sleeping with mouth open (r:.138, p = .034). One hundred forty-nine (63.6%) patients had audiologic evaluations; 14 (9.4%) had hearing impairment. No statistical significance existed between ototoxicity and IV AG exposure (p = .90). Six (42.8%) of 14 patients did not receive ototoxic drugs. One hundred nineteen (50.8%) patients were screened for m.1555A>G mutations, and none were detected.
CONCLUSIONS: Almost a quarter of the study population had CRSwNP. Neither the relationship between AGs exposure and hearing loss nor the genetic predisposition to AG ototoxicity could be shown in cwCF.
METHODS: CwCF who underwent otorhinolaryngologic and audiologic examinations were retrospectively included. Clinical characteristics, ear-nose-throat related symptoms, and a history of ototoxic drug exposure were recorded. m.1555A>G mutations were retrospectively screened among patients with audiologic evaluations.
RESULTS: Two hundred thirty-four cwCF were included in this study with a median age of 10.7 (range, 6.8-14.2) years. Nasal obstruction (14.1%) was the most common symptom. Fifty-two (22.2%) patients had chronic rhinosinusitis (CRS) with nasal polyps (CRSwNP). There was a positive correlation between CRSwNP and the symptom of nasal obstruction (r:.234, p < .001), snoring (r:.179, p = .006), and sleeping with mouth open (r:.138, p = .034). One hundred forty-nine (63.6%) patients had audiologic evaluations; 14 (9.4%) had hearing impairment. No statistical significance existed between ototoxicity and IV AG exposure (p = .90). Six (42.8%) of 14 patients did not receive ototoxic drugs. One hundred nineteen (50.8%) patients were screened for m.1555A>G mutations, and none were detected.
CONCLUSIONS: Almost a quarter of the study population had CRSwNP. Neither the relationship between AGs exposure and hearing loss nor the genetic predisposition to AG ototoxicity could be shown in cwCF.
摘要：
目的：评估囊性纤维化（cwCF）儿科患者耳鼻咽喉科表现以及氨基糖苷（AG）暴露与听力损失之间的关系。我们还旨在通过筛选m.155A>G突变来研究AG耳毒性的遗传易感性。
方法：回顾性纳入接受耳鼻喉和听力学检查的CwCF。临床特征,耳鼻喉相关症状,并记录耳毒性药物暴露史.在听力学评估的患者中回顾性筛查m.155A>G突变。
结果：这项研究包括了2334个cwCF，中位年龄为10.7岁（范围，6.8-14.2）年。鼻塞（14.1%）是最常见的症状。52例（22.2％）患者患有慢性鼻-鼻窦炎（CRS）伴鼻息肉（CRSwNP）。CRSwNP与鼻塞症状呈正相关(r:.234,p<.001),打鼾(r:.179，p=.006)，张开嘴睡觉（r：.138，p=.034）。一百四十九名（63.6％）患者进行了听力学评估；14（9.4％）有听力障碍。耳毒性和IVAG暴露之间无统计学意义（p=.90）。14例患者中有6例（42.8％）未接受耳毒性药物。一百十九名（50.8%）患者进行了m.155A>G突变筛查，没有人被发现。
结论：几乎四分之一的研究人群患有CRSwNP。在cwCF中既不能显示AG暴露与听力损失之间的关系，也不能显示AG耳毒性的遗传易感性。
方法：回顾性纳入接受耳鼻喉和听力学检查的CwCF。临床特征,耳鼻喉相关症状,并记录耳毒性药物暴露史.在听力学评估的患者中回顾性筛查m.155A>G突变。
结果：这项研究包括了2334个cwCF，中位年龄为10.7岁（范围，6.8-14.2）年。鼻塞（14.1%）是最常见的症状。52例（22.2％）患者患有慢性鼻-鼻窦炎（CRS）伴鼻息肉（CRSwNP）。CRSwNP与鼻塞症状呈正相关(r:.234,p<.001),打鼾(r:.179，p=.006)，张开嘴睡觉（r：.138，p=.034）。一百四十九名（63.6％）患者进行了听力学评估；14（9.4％）有听力障碍。耳毒性和IVAG暴露之间无统计学意义（p=.90）。14例患者中有6例（42.8％）未接受耳毒性药物。一百十九名（50.8%）患者进行了m.155A>G突变筛查，没有人被发现。
结论：几乎四分之一的研究人群患有CRSwNP。在cwCF中既不能显示AG暴露与听力损失之间的关系，也不能显示AG耳毒性的遗传易感性。
