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成人终身未经治疗的孤立性生长激素缺乏症患者的听力状况。

Hearing status in adult individuals with lifetime, untreated isolated growth hormone deficiency.

机构信息

Division of Otorhinolaryngology, Federal University of Sergipe, Aracaju, Sergipe, Brazil.

出版信息

Otolaryngol Head Neck Surg. 2014 Mar;150(3):464-71. doi: 10.1177/0194599813517987. Epub 2014 Jan 7.

Abstract

OBJECTIVE

To evaluate the hearing status of growth hormone (GH)-naive adults with isolated GH deficiency (IGHD) belonging to an extended Brazilian kindred with a homozygous mutation in the GH-releasing hormone receptor gene.

STUDY DESIGN

Cross-sectional.

SETTING

Divisions of Endocrinology and Otorhinolaryngology of the Federal University of Sergipe.

SUBJECTS AND METHODS

Twenty-six individuals with IGHD (age, 47.6 ± 15.1 years; 13 women) and 25 controls (age, 46.3 ± 14.3 years; 15 women) were administered a questionnaire on hearing complaints and hearing health history. We performed pure-tone audiometry, logoaudiometry, electroacoustic immittance, and stapedial reflex. To assess outer hair cell function in the cochlea, we completed transient evoked otoacoustic emissions (TEOAEs). To assess the auditory nerve and auditory brainstem, we obtained auditory brainstem responses (ABRs).

RESULTS

Misophonia and dizziness complaints were more frequent in those with IGHD than in controls (P = .011). Patients with IGHD had higher thresholds at 250 Hz (P = .005), 500 Hz (P = .006), 3 KHz (P = .008), 4 KHz (P = .038), 6 KHz (P = .008), and 8 KHz (P = .048) and mild high-tones hearing loss (P = .029). Stapedial reflex (P < .001) and TEOAEs (P = .025) were more frequent in controls. There were no differences in ABR latencies. Hearing loss in patients with IGHD occurred earlier than in controls (P < .001).

CONCLUSION

Compared with controls of the same area, subjects with untreated, congenital lifetime IGHD report more misophonia and dizziness, have predominance of mild high-tones sensorineural hearing loss, and have an absence of stapedial reflex and TEOAEs.

摘要

目的

评估属于一个 GH 释放激素受体基因突变纯合子巴西大家族的、无生长激素(GH)治疗史的单纯 GH 缺乏症(IGHD)成年患者的听力状况。

研究设计

横断面研究。

地点

塞尔希培联邦大学内分泌科和耳鼻喉科。

受试者和方法

共纳入 26 名 IGHD 患者(年龄 47.6 ± 15.1 岁,13 名女性)和 25 名对照者(年龄 46.3 ± 14.3 岁,15 名女性),他们接受了听力投诉和听力健康史问卷调查。我们进行了纯音听阈测试、言语测听、声导抗测试和镫骨肌反射测试。为了评估耳蜗外毛细胞功能,我们完成了瞬态诱发耳声发射(TEOAEs)。为了评估听神经和听脑干,我们获得了听性脑干反应(ABRs)。

结果

IGHD 患者的幻听和头晕主诉较对照者更常见(P =.011)。IGHD 患者在 250 Hz(P =.005)、500 Hz(P =.006)、3 KHz(P =.008)、4 KHz(P =.038)、6 KHz(P =.008)和 8 KHz(P =.048)处阈值更高,且存在轻-中频感音神经性听力损失(P =.029)。对照组镫骨肌反射(P <.001)和 TEOAEs(P =.025)更常见。两组 ABR 潜伏期无差异。IGHD 患者的听力损失发生更早(P <.001)。

结论

与同一地区的对照者相比,未经治疗的、先天性终生 IGHD 患者报告的幻听和头晕更多,更易出现轻-中频感音神经性听力损失,且缺乏镫骨肌反射和 TEOAEs。

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