Lackner A, Acham A, Alborno T, Moser M, Engele H, Raggam R B, Halwachs-Baumann G, Kapitan M, Walch C
Department of Neurotology, ENT University Hospital, Graz Medical University, Austria.
J Laryngol Otol. 2009 Apr;123(4):391-6. doi: 10.1017/S0022215108003162. Epub 2008 Jun 30.
Congenital cytomegalovirus infection is the leading identified nongenetic cause of congenital sensorineural hearing loss. Most of the infections are asymptomatic but may be detected from umbilical cord vein and/or newborn serum positivity for human cytomegalovirus immunoglobulin M, and from urine positivity (on polymerase chain reaction) for human cytomegalovirus deoxyribonucleic acid in the newborn period. Children infected by cytomegalovirus may later develop sensorineural hearing loss. In symptomatically infected infants, ganciclovir therapy administered in the neonatal period prevents hearing deterioration. However, preventative therapy of asymptomatic congenital cytomegalovirus disease with ganciclovir is controversial, as side effects such as severe neutropenia may occur during treatment.
The study population consisted of 23 asymptomatic children with congenital cytomegalovirus infection. Twelve children were treated just after diagnosis of cytomegalovirus infection in the newborn period, with ganciclovir 10 mg/kg bodyweight for 21 days. The other 11 children were observed without therapy. Over a four to 10 year follow-up period, we evaluated all the children's hearing status using pure tone audiometry.
All 23 children had normal sensorineural hearing at one year follow up. Five of the 23 children (21.7 per cent) were lost to follow up over the four to 11 year follow-up period. Of the remaining 18 children, sensorineural hearing loss occurred in two (11.1 per cent). Neither child had been treated with ganciclovir in the newborn period. An eight-year-old boy showed bilateral high frequency loss and a 10-year-old girl showed severe unilateral sensorineural hearing loss. In the ganciclovir-treated group (nine children), none showed sensorineural hearing loss. During ganciclovir therapy, moderate neutropenia occurred as a side effect in two out of 12 (16.6 per cent) treated children. Speech and general development were normal in all children.
Asymptomatic congenital cytomegalovirus infection is likely to be a leading cause of sensorineural hearing loss in young children. Intravenous ganciclovir therapy seems to offer a medical option to prevent subsequent sensorineural hearing loss. Further studies including a greater number of children are needed. Cytomegalovirus screening models are mandatory if medical therapy is to be implemented in time.
先天性巨细胞病毒感染是已确定的先天性感音神经性听力损失的主要非遗传病因。大多数感染是无症状的,但可通过新生儿期脐静脉和/或新生儿血清中人巨细胞病毒免疫球蛋白M呈阳性,以及尿液(采用聚合酶链反应)中人巨细胞病毒脱氧核糖核酸呈阳性检测出来。感染巨细胞病毒的儿童日后可能会出现感音神经性听力损失。对于有症状感染的婴儿,新生儿期给予更昔洛韦治疗可防止听力恶化。然而,用更昔洛韦对无症状先天性巨细胞病毒病进行预防性治疗存在争议,因为治疗期间可能会出现严重中性粒细胞减少等副作用。
研究人群包括23名无症状先天性巨细胞病毒感染儿童。12名儿童在新生儿期诊断出巨细胞病毒感染后立即接受治疗,给予更昔洛韦10mg/kg体重,持续21天。另外11名儿童未接受治疗,进行观察。在4至10年的随访期内,我们使用纯音听力测定法评估了所有儿童的听力状况。
在1年随访时,所有23名儿童的感音神经性听力均正常。在4至11年的随访期内,23名儿童中有5名(21.7%)失访。其余18名儿童中,有2名(11.1%)出现感音神经性听力损失。这两名儿童在新生儿期均未接受更昔洛韦治疗。一名8岁男孩出现双侧高频听力损失,一名10岁女孩出现严重单侧感音神经性听力损失。在更昔洛韦治疗组(9名儿童)中,无人出现感音神经性听力损失。在更昔洛韦治疗期间,12名接受治疗的儿童中有2名(16.6%)出现中度中性粒细胞减少这一副作用。所有儿童的言语和总体发育均正常。
无症状先天性巨细胞病毒感染可能是幼儿感音神经性听力损失的主要原因。静脉注射更昔洛韦治疗似乎为预防后续感音神经性听力损失提供了一种医学选择。需要开展包括更多儿童的进一步研究。如果要及时实施药物治疗,巨细胞病毒筛查模式必不可少。