Progressive hearing loss in children with congenital cytomegalovirus

Case studies of five children with congenital cytomegalovirus (CMV) who were referred for audiological evaluation are presented. Significant deterioration of hearing was noted in all cases within the first 4 years of life. It is essential that neonates with congenital CMV be referred for hearing assessment and that the child's hearing be monitored on a long-term basis. Early intervention is vital for the speech and language development of the hearing-impaired child.     

A wider role for congenital cytomegalovirus infection in sensorineural hearing loss

BACKGROUND: Diagnostic problems in identifying congenital infection cases in infancy have thus far impaired the assessment of the role of congenital cytomegalovirus (CMV) infection in the etiology of sensorineural hearing loss (SNHL). OBJECTIVE: To estimate the impact of congenital infection in children with SNHL by detection of CMV DNA in stored samples of neonatal dried blood (dried blood spots test). METHODS: The Guthrie cards of 130 children with hearing loss >40 dB hearing loss were retrieved from the regional screening center. CMV DNA was extracted by thermal shock and amplified by PCR. RESULTS: The percentage of SNHL cases attributable to congenital CMV infection was 10% (9 of 87) in infants whose SNHL had been diagnosed in their first 2 months of life and 34.2% (13 of 38) in children with deafness of unidentified cause that was diagnosed in early childhood. In the latter group 42.7% (12 of 28) of the children with a hearing loss of >70 dB were CMV-positive. CONCLUSIONS: The results suggest that congenital CMV infection has a more relevant role in the etiology of SNHL than previously reported. The data obtained in both groups suggest that 20 to 30% of all deafness cases are caused by CMV. The percent of congenital CMV cases alone appears to account for all the cases previously attributed to all congenital infections. More than 40% of deafness cases with an unknown cause, needing rehabilitation, are caused by congenital CMV.     

Neuropsychological functioning in patients with asymptomatic congenital cytomegalovirus infection

Congenital cytomegalovirus (CMV) infection has an affinity for the central nervous system and has been implicated in a variety of neurological impairments. Analysis of cognitive functioning in children with asymptomatic congenital CMV infection, however, has revealed no general intellectual deficits. The present study was designed to explore neuropsychological test performance in these children, compared with healthy control subjects, providing data from more sensitive measures of neurocognitive functioning. The sample consisted of 109 children diagnosed with asymptomatic congenital CMV infection and 173 control subjects who were compared on tests measuring various aspects of perceptual and motor functioning, memory, problem solving, and traditional intelligence measures. Young (41NDASH6 yr) control patients performed significantly better on the Full-Scale but not the Verbal or Performance intelligence quotient (IQ) measures than patients with asymptomatic congenital CMV infection, without accompanying consistent neuropsychological performance differences. However, no IQ or neuropsychological differences were found between groups of older children. The present study adds to the existing literature finding no reliable, lasting differences in IQ scores and adds to our knowledge by finding no reliable, lasting differences in neuropsychological test performance.     

Intellectual development in school-aged children with asymptomatic congenital cytomegalovirus infection

Congenital cytomegalovirus infection occurs in about 1% of live births. Although symptomatic congenital infection often results in severe developmental deficits and mental retardation, about 90% have asymptomatic infection. Previous studies of the intellectual development in children with asymptomatic congenital cytomegalovirus have resulted in mixed findings. To control for the effects of hearing impairment (which occurs in about 15% of asymptomatic children) on intelligence scores, we tested 18 prospectively followed, normally hearing, school-aged children with asymptomatic congenital cytomegalovirus (15 black, ten male) and 18 controls matched for age, sex, race, school grade, and socioeconomic status. Children were tested via the Wechsler Intelligence Scale for Children-Revised, the Kaufman Assessment Battery for Children, and the Wide Range Achievement Test. Multivariate analysis revealed no differences between groups on intelligence scores or subscales, achievement scores, or incidence of learning disabilities (defined as significant discrepancy between intelligence and achievement), and mean scores for both groups were very close to national norms. It is concluded that the 25,000 children born in the United States each year with asymptomatic congenital cytomegalovirus and normal hearing are not likely to be at increased risk of mental impairment.     

先天性症状性巨细胞病毒感染脑脊液病毒载量与听力损伤相关性

目的 探讨症状性先天性巨细胞病毒(cytomegalovirus,CMV)感染新生儿脑脊液(cerebrospinal fluid,CSF) CMV DNA载量与感音性听力损伤(sensorineural hearing loss,SNHL)的相关性.方法 36例先天性症状性CMV感染患儿,PCR法检测CSF CMV DNA载量,并于出生1个月内、生后6个月及1年左右行脑干听觉诱发电位检测.结果 (1)36例患儿,其中CSF CMV DNA阳性15例,阳性率41.2％,SNHL17例,SNHL发生率47.2％.(2) CSF CMV DNA阳性组SNHL发生率60.0％(9/15);阴性组SNHL发生率38.1％ (8/21),两组发生率比较差异无统计学意义(P =0.194).(3)CSFCMVDNA阳性组中,SNHL组与听力正常组CSF CMV DNA载量为3.35 ±0.68和3.17±0.56,两组载量比较,差异无统计学意义(P=0.36).结论 先天性症状性CMV感染患儿CSF CMV DNA是否阳性及其载量不是预测SNHL的指标.     

Congenital cytomegalovirus infection: a cause of sensorineural hearing loss.

Prospective studies have suggested that about 108 children with congenital cytomegalovirus (CMV) infection and bilateral sensorineural hearing loss are born each year in England and Wales; this represents about 12% of all children with congenital sensorineural hearing loss. Over a nine year period 1644 children aged between 6 months and 4 years who were attending the Nuffield Hearing and Speech Centre were screened for CMV infection. The prevalence of CMV in the urine of children with sensorineural hearing loss but no immediate family history of deafness was nearly twice that (13%) found in other children with impaired hearing and those with normal hearing (7%). These findings indicate the importance of CMV as a cause of hearing loss.     

Ganciclovir therapy for congenital cytomegalovirus infection in six infants

BACKGROUND: Congenital cytomegalovirus (CMV) infection is common, and its morbidity rate is high. Ganciclovir (GCV) treatment has been used for congenital CMV infection, but there are few reports on viral loads associated with GCV therapy. METHODS: A real-time PCR assay was used to monitor viral load in 6 cases of symptomatic CMV infection that received GCV therapy. Initially GCV was given at a dose of 5-12 mg/kg/d for 2-7 weeks. In 2 cases, additional doses were given as symptoms returned. RESULTS: After GCV administration, active signs of chorioretinitis, thrombocytopenia and anemia disappeared or improved in all cases. During GCV therapy, viral loads decreased while patients improved clinically and increased again when GCV therapy was stopped. Although CMV DNA continued to be detectable for a long period, clinical findings did not always worsen. In 2 cases, an improvement of hearing loss was observed. CONCLUSION: GCV therapy transiently suppresses the CMV concentrations. Subsequent increases of viral titers do not appear to be correlated with the clinical course or neurologic outcome.     

先天性巨细胞病毒感染对婴幼儿的影响

为探讨先天性巨细胞病毒(CMV)感染对婴幼儿的影响,采用瞬态诱发性耳声发射(TEOAE)对65例先天性CMV感染儿及82例非感染儿于新生儿期进行听力筛查,并于生后6个月至4岁进行听力及智力随访.感染组发育商落后8例,对照组仅1例,二组差异显著.感染组新生儿期、随访时分别有7例9耳、9例11耳未通过TEOAE听力测试,对照组均为1例1耳未通过,未通过率感染组明显高于对照组.新生儿期、随访时双耳反应能量感染组明显低于对照组.14例进行了脑干听觉诱发电位(ABR)检测,感染组9例17耳听力异常,对照组1例1耳听力异常.提示先天性CMV感染对听力、智力的影响应引起长期关注.     

巨细胞病毒感染新生儿听力损害与尿液病毒负荷量的相关性研究

目的：研究新生儿巨细胞病毒(CMV)负荷量与听力损害的关系。方法：分析我院2006 年 4 月至 2010 年 1 月确诊的22例CMV感染新生儿的尿液中CMV病毒含量及其听力损失情况,并对其中20例进行为期3~6月的随访,复查脑干听觉诱发电位,分析病毒负荷量与听力损害的相关性并绘制受试者工作曲线(ROC),确定诊断听力损害的病毒负荷界点。结果：CMV感染新生儿脑干听觉诱发电位(BAEP)异常组尿液病毒负荷量平均值显著高于BAEP正常组（5.06±1.50 vs 3.73±0.86, P<0.05）,建立的ROC曲线下面积为0.752,界点为5.1时（即CMV DNA指数为1.27×105拷贝/mL）预测病毒负荷量导致听力损伤的灵敏度为54.5%,特异度100%,5.1以上组听力损害率显著高于5.1以下组(P<0.05)。听力损伤类型与病毒含量的高低（以病毒负荷量5.1为界点）无相关性(P＞0.05)。随访的20例中6例出现听阈损失,与病毒含量在5.1以下组相比,5.1以上组听力损害更严重(P<0.05)。结论：新生儿尿液CMV负荷量的水平可提示导致听力损伤的几率大小;当病毒负荷量达1.27×105拷贝/mL时,更容易出现听力损害。     

Screening infants for hearing loss.

A computerised child health register was used to study the coverage, referral rate, and false positive rate of the eight month hearing distraction test in a cohort of 1990 births to residents of one district during an eight month period. Coverage by the age of 9 months was under 60% and varied with ethnic group and immunisation record. The true problem rate among those referred was 48%. None of the three children in the cohort who had a sensorineural hearing loss was picked up by screening, although it did identify children with conductive loss. The findings question the value of the distraction test as currently used, and underline the usefulness of computerisation, even if limited to child registration, in the evaluation of screening tests.     

Hearing loss in children with congenital cytomegalovirus infection born to mothers with preexisting immunity

OBJECTIVE: To define hearing outcomes in children with congenital cytomegalovirus (CMV) infection born to mothers with non-primary CMV infection. STUDY DESIGN: A cohort of 300 children with congenital CMV infection identified by newborn virologic screening at the University of Alabama Hospital and a private community hospital in which the type of maternal infection could be classified constituted the study population. Maternal infections were categorized by analyzing serum samples. Children were followed prospectively and underwent serial audiologic evaluations. RESULTS: The frequency of hearing loss was not different between children born to mothers with non-primary infection (10%) and those with primary infection (11%). Significantly more children in the primary infection group had progressive and severe/profound hearing loss compared with children in the non-primary group. The frequency of bilateral, delayed onset, high-frequency, and fluctuating hearing loss was not different between the 2 groups. The mean age of diagnosis of hearing loss was 39 +/- 53 months for children born to mothers with non-primary infection and 13 +/- 21 months for the primary infection group (P = .16). CONCLUSIONS: Maternal preexisting seroimmunity to CMV does not provide complete protection against hearing loss in infants with congenital CMV infection.     

先天性无症状Ⅱ型单纯疱疹病毒感染患儿近期预后观察

为观察先天性无症状Ⅱ型单纯疱疹病感染患儿近期生长发育情况，对12例感染婴儿及12例对照婴儿为期1年的跟踪随访，检查内容包括体格测量，Bayley婴幼儿发育量表，头颅B超和脑干听觉诱发电位（BAEP）等，结果显示感染组婴儿体格发育与对照组相比，差异无显著性意义，但智能发育的差异有显著性意义（P＜0．05），感染组3个月时的头颅B超异常率高于对照组，差异有显著性意义（P＝0．034），12个月时的头颅B超异常率虽高于对照组，但差异无显著性意义，感染组BAEP的异常率高于对照组，差异有显著性意义（P＝0．023），提示先天性II期单弛疱疹病毒感染对新生儿及婴儿的神经系统发育可能造成一定的不良影响，应普及孕妇HSV－II感染的筛查，对感染者及早予抗病毒治疗。     

先天性巨细胞病毒感染对婴幼儿听力及智力影响的研究

目的　探讨先天性巨细胞病毒 (CMV)感染对婴幼儿生长发育、听力及智力的影响。方法　对 65例先天性CMV感染儿及 82例非感染儿于新生儿期进行生长发育及听力的检测 ,并于生后 6个月至 4岁进行随访。随访内容包括 :体格检查、发育商测试及听力测试。结果　两组生长发育状况无显著性差异。感染组发育商落后8例 ,对照组仅 1例 ,二者有显著性差异。感染组新生儿期、随访时分别有 7例 9耳、9例 11耳未通过TEOAE听力测试 ,对照组均为 1例 1耳未通过 ,未通过率感染组明显高于对照组。新生儿期、随访时双耳反应能量感染组明显低于对照组。 14例进行了ABR检测 (感染组 12例 ,对照组 2例 ) ,其中感染组 9例 17耳听力异常 ,对照组 1例 1耳听力异常。结论　先天性CMV感染对婴幼儿生长发育可能无明显影响 ,但对听力、智力的损害却不容忽视