G/BBB 综合征患者的听觉发现和电生理学。
Auditory findings and electrophysiologics in individuals with G/BBB syndrome.
机构信息
Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru, São Paulo, Brasil.
出版信息
Braz J Otorhinolaryngol. 2011 Nov-Dec;77(6):768-74. doi: 10.1590/S1808-86942011000600014.
UNLABELLED
The G/BBB syndrome is a rare condition characterized by hypertelorism, cleft lip and palate, and hypospadias. No studies were found on the hearing of individuals with this syndrome.
AIM
To investigate the auditory function in patients with G/BBB syndrome, such as the occurrence of hearing loss, and central and peripheral auditory nerve conduction.
METHODS
Fourteen male patients aged 7-34 years with the G/BBB syndrome were assessed by otoscopy, audiometry, tympanometry and evoked auditory brainstem response (ABR).
MODEL
A retrospective clinical series study.
RESULTS
Audiometric thresholds were normal in 12 (66.7%) of the sample and altered in two (33.3%), one with conductive and one with sensorineural loss. ABR results were: all patients had normal absolute wave I latencies; absolute wave III and V latencies were increased in two and six patients, respectively; interpeak latencies I-III, IV and V interpeak latencies were increased in four, three and eight patients respectively.
CONCLUSION
Hearing loss can occur in the G/BBB syndrome. There is evidence of central auditory pathway involvement in the brainstem. Imaging studies are needed to clarify the clinical findings.
未加标签
G/BBB 综合征是一种罕见的疾病，其特征为眼距过宽、唇腭裂和尿道下裂。没有发现关于患有这种综合征的个体听力的研究。
目的
调查 G/BBB 综合征患者的听觉功能，如听力损失以及中枢和外周听觉神经传导的发生情况。
方法
对 14 名年龄在 7-34 岁的男性 G/BBB 综合征患者进行耳镜检查、听力测试、鼓室图和听觉脑干反应（ABR）检查。
模型
回顾性临床系列研究。
结果
样本中 12 名（66.7%）患者的听力阈值正常，2 名（33.3%）患者异常，1 名患者为传导性听力损失，1 名患者为感音神经性听力损失。ABR 结果：所有患者的绝对波 I 潜伏期均正常；有 2 名和 6 名患者的绝对波 III 和 V 潜伏期延长；4 名、3 名和 8 名患者的 I-III 峰间潜伏期、IV 和 V 峰间潜伏期分别延长。
结论
G/BBB 综合征可发生听力损失。存在脑干中枢听觉通路受累的证据。需要影像学研究来阐明临床发现。
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