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Beyond hearing loss: exploring neurological and neurodevelopmental sequelae in asymptomatic congenital
Meghan R Swanson1, Lauren D Haisley2, William B Dobyns3
1Masonic Institute for the Developing Brain, Center for Neurobehavioral Development, and Institute for Child Development, Division of Clinical Behavioral Neuroscience, University of Minnesota Medical School Minneapolis, Minneapolis, MN, USA. swanson1@umn.edu.
Insights
Congenital cytomegalovirus (cCMV) infection is common, with most infants having clinically inapparent cCMV. Further research is needed to understand the full spectrum of neurodevelopmental outcomes in these infants.
Area of Science:
- Pediatrics
- Infectious Diseases
- Neurodevelopmental Disorders
Background:
- Congenital cytomegalovirus (cCMV) is a common infection affecting approximately 1 in 200 births.
- While many infants with cCMV are asymptomatic, a subset develops clinically apparent CMV disease (CACMV) with known long-term disabilities.
- Clinically inapparent cCMV (CICMV) poses a less understood risk for neurodevelopmental issues, despite its increased identification through universal screening.
Purpose of the Study:
- To highlight the diagnostic and prognostic uncertainties surrounding clinically inapparent congenital CMV infections.
- To emphasize the need for further research into the neurodevelopmental outcomes of infants with CICMV.
- To inform clinical practice and research priorities in the era of universal cCMV screening.
Main Methods:
- Review of existing literature on cCMV prevalence, clinical manifestations, and neurodevelopmental outcomes.
- Analysis of the implications of universal newborn screening for cCMV identification.
- Identification of knowledge gaps in the evaluation and management of CICMV.
Main Results:
- Universal cCMV screening identifies a majority of infections as CICMV.
- The long-term neurodevelopmental impact of CICMV is not fully characterized, potentially being more extensive than previously recognized.
- Current guidelines lack comprehensive recommendations for diagnostic evaluations, CNS imaging, and developmental assessments for CICMV.
Conclusions:
- Adverse neurodevelopmental outcomes in CICMV may be underestimated.
- Further research is crucial to define the scope of neurocognitive disability associated with CICMV.
- New findings will aid in refining the understanding and management of cCMV in infants identified through universal screening.
Abstract:
Congenital cytomegalovirus (cCMV) infection is common, and usually clinically inapparent. The prevalence of infection is approximately 1:200 births, but only 10-15% of infants have clinically apparent CMV disease (CACMV) as newborns. The most common long-term disability is sensorineural hearing loss (SNHL), which occurs in 10-15% of all cases. Infants with CACMV are also at increased risk for intellectual disability, cerebral palsy, learning disabilities, ocular and cortical blindness, seizure disorders, developmental delay, and autism spectrum disorders. Although infants with clinically inapparent cCMV (CICMV) are at risk for SNHL, the spectrum of other adverse neurodevelopmental outcomes is less clear, since few studies have tracked neurodevelopment in this setting. With the advent of universal cCMV screening, most cCMV infections will now be identified in infants with CICMV. These infants require serial audiologic monitoring, but many questions are unanswered, including what kinds of diagnostic evaluations are required; what kinds of central nervous system (CNS) imaging studies are recommended; what the utility and value of developmental assessments is; and whether there are biomarkers that can inform the long-term prognosis and direct anticipatory guidance in monitoring for neurologic and neurodevelopmental adverse outcomes. IMPACT: Universal newborn screening for congenital CMV (cCMV) infection has been implemented in many US states and Canadian provinces. Most infants identified by universal screening have CICMV infections. All require audiologic monitoring, but there is minimal experience to direct other evaluations, including laboratory tests, brain imaging and neurodevelopmental assessments. Adverse neurodevelopmental outcomes in CICMV may be more extensive than previously appreciated. Research is needed to define the full range of potential neurocognitive disability. New knowledge generated by studying CICMV infections may aid in reclassification of the scope of disease in an emerging era of universal cCMV screening.
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