Longitudinal investigation of hearing disorders in children with congenital cytomegalovirus.

This investigation consisted of a longitudinal study of the effects of congenital cytomegalovirus (CMV) infection on hearing sensitivity in 860 children with documented asymptomatic or symptomatic congenital CMV infection. Of the 651 children with asymptomatic CMV infection, 48 (7.4%) developed sensorineural hearing loss (SNHL), compared to 85 (40.7%) of the children with symptomatic CMV infection. Children in both groups experienced latent effects consisting of delayed onset of loss, threshold fluctuations, and/or progressive loss of hearing. It can be concluded that congenital CMV infection is a leading cause of SNHL in children. The late onset and progression of loss necessitates continued monitoring of hearing sensitivity in this population.

Newborn hearing screening: will children with hearing loss caused by congenital cytomegalovirus infection be missed?

OBJECTIVE: To predict whether universal newborn auditory screening will identify most infants with sensorineural hearing loss (SNHL) caused by congenital cytomegalovirus (CMV) infection. STUDY DESIGN: A cohort of 388 children born between 1980 and 1996 at one hospital and identified during the newborn period as having congenital CMV infection received repeated hearing evaluations to assess whether hearing loss had occurred. RESULTS: SNHL was detected in 5.2% of all infants at birth. Late-onset SNHL occurred among the children throughout the first 6 years of life. By the age of 72 months, the cumulative incidence of SNHL was 15.4% in the cohort. Children with clinically apparent disease at birth had significantly more SNHL than children without any apparent disease (22.8% vs 4.0% at 3 months and 36.4% vs 11.3% at 72 months of age). CONCLUSIONS: Universal screening of hearing in neonates will detect less than half of all SNHL caused by congenital CMV infection. Because most infants with congenital CMV infection are without symptoms at birth, these children are unlikely to be recognized as being at risk for SNHL and will not receive further hearing evaluations to detect late-onset hearing loss. A combined approach of universal screening of neonates for hearing, as well as for detection of congenital CMV infection, needs to be considered.

Progressive and fluctuating sensorineural hearing loss in children with asymptomatic congenital cytomegalovirus infection.

OBJECTIVE: To determine the prevalence and temporal changes of sensorineural hearing loss (SNHL) among children with clinically inapparent (asymptomatic) congenital cytomegalovirus (CMV) infection identified from a cohort of newborn infants screened for congenital CMV infection. METHODS: The study population consisted of 307 children with documented asymptomatic congenital CMV infection, 76 uninfected siblings of children with asymptomatic congenital CMV infection, and 201 children whose neonatal screen for congenital CMV infection showed negative results. Audiologic evaluations were completed for all children to determine their hearing status. RESULTS: SNHL occurred only in children with congenital CMV infection. Of the children with asymptomatic congenital CMV infection, 22 (7.2%; 95% confidence interval, 4.5% to 10.6%) had SNHL. Among the children with hearing loss, further deterioration of hearing occurred in 50.0%, with the median age at first progression at 18 months (range, 2 to 70 months). Delayed-onset SNHL was observed in 18.2% of the children, with the median age of detection at 27 months (range, 25 to 62 months). Fluctuating SNHL was documented in 22.7% of the children with hearing loss. CONCLUSIONS: Asymptomatic congenital CMV infection is likely a leading cause of SNHL in young children. The continued deterioration of hearing and delayed onset of SNHL in these children emphasizes the need for continued monitoring of their hearing status.

Hearing loss and congenital symptomatic cytomegalovirus infection: a case report of multidisciplinary longitudinal assessment and intervention.

More than 6000 children born annually in this country have hearing loss resulting from congenital cytomegalovirus (CMV) infection, the leading nonhereditary congenital cause of hearing loss in children. This exemplary congenital symptomatic CMV case focuses on the results of longitudinal audiologic, educational, medical, psychological, and visual evaluations and intervention. Decreased ocular motor control and visual acuity were observed as was bilateral deterioration of hearing from 3 days though 9 years of age. Treatment with dexamethasone and histamine resulted in almost complete reversal of the most recent progression of hearing loss in the left ear.

Audiological findings in children with neonatal herpes.

Hearing sensitivity was assessed in 20 children who were diagnosed as having symptomatic neonatal herpes simplex virus (HSV) infection. The subjects were seen as part of an interdisciplinary project that included audiological, medical, visual, and developmental assessments. Audiologic procedures consisted of behavioral and/or auditory brain stem tests and immittance audiometry. Eighteen children were found to have normal hearing and 2 were documented as having sensorineural hearing impairments. Similarities in the manner in which HSV and congenital cytomegalovirus affect the auditory mechanism are discussed.

Hearing and otologic disorders in children with Down syndrome.

Hearing impairment and otologic problems have been reported to be prevalent among individuals with Down syndrome. This study was designed to determine whether children with Down syndrome have a higher prevalence of auditory abnormalities than do children with other forms of mental retardation. Hearing, impedance, and otoscopic examinations were performed on 30 children with Down syndrome and 30 children without Down syndrome matched for CA and IQ. Results indicated that the children with Down syndrome had a significantly higher prevalence of hearing impairment and otologic disorders.

Progressive hearing impairment in children with congenital cytomegalovirus infection.

Audiological assessment of 86 children with congenital cytomegalovirus infection revealed progressive hearing loss in four of 12 subjects with sensorineural hearing impairments. Case descriptions are presented documenting the progression of the hearing loss. In view of the findings, children with congenital cytomegalovirus should be monitored closely to insure detection of possible delayed or progressive hearing impairment and delivery of appropriate habilitative services.

Auditory and visual defects resulting from symptomatic and subclinical congenital cytomegaloviral and toxoplasma infections.

Sensorineural hearing loss was present in ten of 59 (17%) patients with congenital cytomegalovirus (CMV) infection (three of eight born with symptomatic and seven of 51 born with subclinical infection). The defect was bilateral in eight, moderate to profound in eight, and of progressive nature in two. Hearing loss did not occur in 21 patients with natal CMV infection nor in seven of 12 patients with congenital toxoplasmosis. Histopathologic and immunofluorescent studies of the inner ear in two of three neonates who died with severe infection revealed that viral antigens were widely distributed in cochlear structures. Eye pathology was associated only with congenital Toxoplasma (nine of 12) and CMV (seven of 43) infections. Visual impairments were more prominent and severe in those born with symptomatic infections, exclusively so with CMV. However, ocular defects, in particular chorioretinitis, developed after birth in five of eight patients born with asymptomatic congenital toxoplasmosis. These data firmly establish clinically inapparent congenital CMV infection as a major public health problem and confirm the fact that congenital toxoplasmosis may be associated with late-appearing, debilitating chorioretinitis.