• Colm Madden, Susan Wiley, Mark Schleiss, Corning Benton, Jareen Meinzen-Derr, John Greinwald, and Daniel Choo.
• 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.
• Int. J. Pediatr. Otorhinolaryngol. 2005 Sep 1; 69 (9): 1191-8.

ObjectiveTo correlate audiometric findings and outcomes with the clinical, radiological and educational findings in a symptomatic congenital cytomegalovirus (CMV) population with sensorineural hearing loss.MethodsA 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.ResultsTwenty-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).ConclusionsThe 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.

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