title: Audiometric, clinical and educational outcomes in a pediatric symptomatic congenital cytomegalovirus (CMV) population with sensorineural hearing loss.
abstract: OBJECTIVE
  To correlate audiometric findings and outcomes with the clinical, radiological and educational findings in a symptomatic congenital cytomegalovirus (CMV) population with sensorineural hearing loss.
  
  
  METHODS
  A retrospective review of data from 21 symptomatic congenital CMV patients identified in a pediatric hearing impaired database of 1500 patients. Clinical data, audiometric thresholds and outcomes, radiographic abnormalities, communication and educational achievements were used as outcome measures.
  
  
  RESULTS
  Twenty-one patients were identified with symptomatic congenital CMV infection at birth; 5 with unilateral hearing loss and 16 with bilateral hearing loss. The median initial pure-tone average (PTA) for the 21 subjects was 86 dB and the median final PTA was 100 dB. Progression of hearing loss was seen in 9 patients (43%). Neurological and radiological sequelae of symptomatic CMV infection were seen in 81% of affected patients. Children with neurological dysfunction were significantly more likely to rely on special education (p = 0.045). There was a significant correlation between the severity of the initial PTA and the development of a progressive hearing loss (p = 0.0058). Initial hearing thresholds were significantly better in those children with a history of jaundice (p = 0.002), hepatosplenomegaly (HSM) (p = 0.022) and cerebral palsy (CP) (p = 0.013). There was a significant correlation between a less severe final PTA and the presence of CP (p = 0.005). A history of mental retardation in children was significantly associated with poorer communication skills (p = 0.043).
  
  
  CONCLUSIONS
  The severity of neurological manifestations in congenital symptomatic CMV infection was positively correlated with the need for total and manual communication and the reliance on special education. Statistical associations between clinical findings such as hepatic dysfunction, CP and hearing level were identified however plausible mechanisms explaining these associations remain ambiguous and are discussed in the context of this complex population of children with congenital symptomatic CMV.
authors.affiliation: Center of Hearing and Deafness Research, Division of Otolaryngology-Head and Neck Surgery, Department of Pediatric Otolaryngology, OSB-3, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH 45229, USA.
authors.forename: Colm
authors.initials: C
authors.lastname: Madden
authors.forename: Susan
authors.initials: S
authors.lastname: Wiley
authors.forename: Mark
authors.initials: M
authors.lastname: Schleiss
authors.forename: Corning
authors.initials: C
authors.lastname: Benton
authors.forename: Jareen
authors.initials: J
authors.lastname: Meinzen-Derr
authors.forename: John
authors.initials: J
authors.lastname: Greinwald
authors.forename: Daniel
authors.initials: D
authors.lastname: Choo
mesh_terms: D000293:Adolescent; D000704:Analysis of Variance; D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold / Q000502:physiology*; D001921:Brain / Q000000981:diagnostic imaging; D002547:Cerebral Palsy / Q000150:complications / Q000209:etiology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000000981:diagnostic imaging; D012048:Correction of Hearing Impairment / Q000379:methods; D003587:Cytomegalovirus / Q000472:pathogenicity; D003586:Cytomegalovirus Infections / Q000150:complications* / Q000151:congenital / Q000503:physiopathology*; D004519:Education, Special / Q000706:statistics & numerical data; D004522:Educational Status; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000209:etiology* / Q000503:physiopathology*; D006801:Humans; D008297:Male; D011859:Radiography; D012189:Retrospective Studies; D013701:Temporal Bone / Q000000981:diagnostic imaging