May 2020

In 2013 Utah enacted legislation requiring infants failing newborn hearing screening be tested for cytomegalovirus infection. As a result, cytomegalovirus-infected infants are being identified because of hearing deficits. Retrospective medical record review was used to identify patients seen at the University of Utah and Primary Children’s Hospitals in Salt Lake City, Utah, who failed newborn hearing screens. A cohort of patients with congenital cytomegalovirus infection, brain MRI and sedated auditory brainstem response testing was studied.

Seventeen patients were identified; eleven (65%) were female. Confirmatory auditory brainstem response testing, performed at a median of 29 days old, showed profound hearing loss in eight subjects (47%), severe loss in two (12%), moderate loss in two (12%), and mild loss in three (18%); two (12%) had normal hearing. The diagnosis of cytomegalovirus infection was made at a median of 23 days old. Brain imaging was performed at a median of 65 days old. Ten subjects (59%) had one or more neuroimaging abnormality. White matter lesions were found in eight (47%), cysts in three (18%), and stroke in two (12%). Polymicrogyria was identified in two (12%). Seven (41%) had normal brain MRIs.

These results indicate that the majority of infants whose CMV infections were identified after failing newborn hearing screening had abnormal brain MRIs. Our results suggest that brain MRIs should be considered in infants with congenital CMV infections who are identified through hearing screening programs.

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