title: Investigation of the role of congenital cytomegalovirus infection in the etiology of enlarged vestibular aqueducts.
abstract: OBJECTIVE
  To determine whether congenital cytomegalovirus (CMV) infection is an etiologic factor in the pathogenesis of enlarged vestibular aqueducts (EVA).
  
  
  METHODS
  Two different cohort studies. Subjects The study population comprised 19 subjects with a history of congenital CMV infection and sensorineural hearing loss (cohort 1); 39 subjects with nonsyndromic EVA and their unaffected mothers (cohort 2); and 16 control subjects with EVA associated with Pendred syndrome and bi-allelic mutations of the SLC26A4 gene and their unaffected mothers.
  
  
  RESULTS
  In cohort 1, we detected EVA in 0 of 19 subjects with congenital CMV infection and sensorineural hearing loss. In cohort 2, anti-CMV serologic profiles were consistent with possible congenital CMV infection in 10 (26%) of 39 subjects with nonsyndromic EVA and 6 (38%) of 16 control subjects with Pendred syndrome (P = .52). These seroprevalence rates are similar to those expected in the general population (40%).
  
  
  CONCLUSIONS
  In spite of their auditory phenotypic similarities, congenital CMV infection is not a significant factor in the etiology of EVA.
authors.affiliation: Hearing Section, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA.
authors.forename: Shannon P
authors.initials: SP
authors.lastname: Pryor
authors.forename: Gail J
authors.initials: GJ
authors.lastname: Demmler
authors.forename: Anne C
authors.initials: AC
authors.lastname: Madeo
authors.forename: Yandan
authors.initials: Y
authors.lastname: Yang
authors.forename: Chris K
authors.initials: CK
authors.lastname: Zalewski
authors.forename: Carmen C
authors.initials: CC
authors.lastname: Brewer
authors.forename: John A
authors.initials: JA
authors.lastname: Butman
authors.forename: Karen B
authors.initials: KB
authors.lastname: Fowler
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Griffith
mesh_terms: D000293:Adolescent; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003586:Cytomegalovirus Infections / Q000150:complications* / Q000151:congenital* / Q000235:genetics; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000821:virology; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009154:Mutation; D014723:Vestibular Aqueduct / Q000821:virology*