Audiometric, clinical and educational outcomes in a pediatric symptomatic congenital cytomegalovirus (CMV) population with sensorineural hearing loss.

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.