A Case of Delayed-onset Sensorineural Hearing Loss with Congenital Cytomegalovirus Infection

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Sensorineural hearing loss (SNHL) due to congenital cytomegalovirus (CMV) infection can either be present at birth or develop later in life. We report a 3 years 7 months old boy who gradually developed speech until 3 years of age, but later showed no responses to verbal stimuli. He was first referred to our clinic for the evaluation of motor delay at 9 months of age and was found to have left hemiparesis. Brain MRI revealed polymicrogyria in the right frontal region. Epileptic seizures began at the age of 13 months. Verbal development was assessed annually using intellectual function testing. He responded to his name at 9 months of age, and imitated words and pointed to objects when asked at 21 months. He could speak several words at 34 months of age. However, around 3 years of age, he stopped saying meaningful words. Audiologic evaluation revealed severe SNHL at 3 years 7 months old. There was no family history of hearing loss, past medical history of ototoxic medications or bacterial meningitis, or craniofacial/auditory anomalies, as possible factors related to the hearing loss. At the age of 7 years 5 months, examination of a dried umbilical cord specimen revealed the presence of CMV DNA. Thus, this patient was diagnosed as having delayed-onset SNHL with asymptomatic congenital CMV infection. A prospective study is necessary for early detection of delayed-onset SNHL in patients with asymptomatic congenital CMV infection. Testing for the presence of CMV DNA in neonatal urine is performed in cases in which pregnant women are seronegative for CMV-IgG antibody or seropositive for CMV-IgM antibody during the first trimester of pregnancy. Infants diagnosed as having congenital CMV infection are evaluated by a newborn hearing screening test, head CT, ophthalmologic evaluations, and intellectual function testing. Follow-up hearing assessments are performed by both the auditory brainstem response and behavioral audiometry.

A Case of Delayed-onset Sensorineural Hearing Loss with Congenital Cytomegalovirus Infection

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