Madell J R, Sculerati N
New York (NY) League for the Hard of Hearing, University School of Medicine, NY.
Arch Otolaryngol Head Neck Surg. 1991 Mar;117(3):332-5. doi: 10.1001/archotol.1991.01870150100015.
Younger siblings of children with sensorineural hearing loss of possible hereditary cause underwent interval audiologic examination. Seven siblings (in unrelated families) were found to have progressive sensorineural hearing loss despite early audiograms documenting normal hearing levels for age. Continued testing of these children allowed for early identification and intervention. We advocate regular otolaryngologic and audiologic follow-up even after normal audiologic assessments are made for younger siblings of children with documented sensorineural hearing loss, unless a definite nongenetic origin of the hearing loss in the older child is known. Recessive sensorineural hearing loss with onset in infancy or childhood may present with no antecedent family history and with normal behavioral audiograms early in life.
可能由遗传因素导致感音神经性听力损失的儿童的弟弟妹妹们接受了定期听力检查。发现7名(来自不同家庭的)弟弟妹妹患有进行性感音神经性听力损失,尽管早期听力图显示其听力水平在同龄人中属正常。对这些儿童持续进行检测有助于早期识别和干预。我们主张,即使对已确诊有感音神经性听力损失的儿童的弟弟妹妹进行听力评估结果正常后,也应定期进行耳鼻喉科和听力方面的随访,除非已知年龄较大儿童的听力损失有明确的非遗传病因。婴儿期或儿童期起病的隐性感音神经性听力损失可能没有家族病史,且早期行为听力图正常。