Griz Silvana, Cabral Mariana, Azevedo Graziela, Ventura Liana
Fonoaudióloga, Doutora em Psicologia Cognitiva, Universidade Federal de Pernambuco, Endereço: Av. Flor de Santana, 189/301 Parnamirim, Recife/PE CEP 52060-290, Brazil.
Int J Pediatr Otorhinolaryngol. 2007 Sep;71(9):1457-63. doi: 10.1016/j.ijporl.2007.06.002. Epub 2007 Jul 20.
Moebiüs sequence is a pathology not very well understood regarding to the hearing status. The main goal of this study was to describe the audiologic findings in children and adolescent who carry Moebiüs sequence.
Participated in this study 17 children and adolescent, with age ranging from 3 to 13 years old. Prior to the testing, the family answered an interview. It was realized external auditory canal inspection, and the hearing testing (auditory instrumentation, pure tone audiometry, speech audiometry, immittanciometric measures, and otoacoustic emissions) on the participants.
The auditory instrumentation evaluation (n=6) was present in all participants. The pure tone audiometry presented normal hearing levels in 75.0% of the tested ears, one ear with conductive hearing loss, two ears with sensory neural hearing loss, and one ear with mixed hearing loss. The tympanometric measures showed Type A tympanograms in 63.0% of the ears, Type B in 11.1%, Type C in 18.5%, and Type As in 7.4%. T the acoustic reflexes measure showed contralateral acoustic reflexes present in 50.0% of the ears, and ipsilateral acoustic reflexes present in 34.6% ears. The OAE results showed presence in 73.0%, for the TOAE, and 76.9% for the DPOAE.
These results support the idea that there is no audiologic pattern for conductive hearing loss. The majority of the participants presented hearing in the normal range. Care should be taken in drawing conclusions regarding to auditory status of the individual with Moebiüs sequence, but what can be said is that not always those individuals present hearing loss.
关于莫比乌斯序列患者的听力状况，目前尚未完全了解。本研究的主要目的是描述患有莫比乌斯序列的儿童和青少年的听力学检查结果。
17名年龄在3至13岁之间的儿童和青少年参与了本研究。在测试前，其家人接受了访谈。对参与者进行了外耳道检查以及听力测试（听觉仪器检查、纯音听力测定、言语听力测定、声导抗测量和耳声发射测试）。
所有参与者均进行了听觉仪器评估（n = 6）。纯音听力测定结果显示，75.0%的受试耳听力水平正常，1耳为传导性听力损失，2耳为感音神经性听力损失，1耳为混合性听力损失。鼓室图测量结果显示，63.0%的耳为A型鼓室图，11.1%为B型，18.5%为C型，7.4%为As型。声反射测量结果显示，50.0%的耳存在对侧声反射，34.6%的耳存在同侧声反射。耳声发射测试结果显示，瞬态诱发耳声发射的出现率为73.0%，畸变产物耳声发射的出现率为76.9%。
这些结果支持了这样一种观点，即传导性听力损失不存在听力学模式。大多数参与者的听力在正常范围内。在对患有莫比乌斯序列的个体的听觉状况得出结论时应谨慎，但可以说的是，并非所有这些个体都存在听力损失。
