Congenital heart disease (CHD) is one of the most common types of birth defect. Often newborn hearing screening is not performed for infants with congenital heart disease because of serious health problems at birth. Hearing loss in children with CHD may have late onset due to different stages of CHD treatment. The monitoring of the auditory function is required for early identification of hearing loss in children with CHD, however algorithm of follow-up assessment is not currently defined. Objective – estimation of prevalence, types and time of hearing loss onset in children with congenital heart disease and development a follow-up assessment for CHD children. Results. 148 children with congenital heart disease have been evaluated with auditory brainstem response testing, otoacoustic emissions, impedancemetry, behavioral hearing tests. The patients ranged in age from 7 days to 6 years. 55 children had hearing loss: 28 children with conductive hearing loss and 27 children with sensorineural hearing loss, 2 of them with auditory neuropathy. 6 children had late onset sensorineural hearing loss. The age of delayed hearing loss identification ranged from 4 months to 5 years. Conclusion. Children with CHD require long-term monitoring of the auditory function; an algorithm of follow-up assessment for children with congenital heart disease is proposed. Audiological assessment in children with CHD should include auditory brainstem response registration for auditory neuropathy identification.

Congenital heart disease (CHD) is 1/3 of all congenital malformations and one of the main causes of infant mortality. Hearing loss may be part of syndromes associated with CHD, or the hearing pathology can be a consequence of the various stages of rehabilitation of underlying pathology. Currently, there isn’t any data on the systematic study of hearing impairment in children with congenital cardiac pathologies. Three hundred and six patients aged 2 days to 8.5 months were examined: 96 patients were included in the main group with CHD, and 300 children without CHD made up a comparison group. All children underwent newborn hearing screening. Infants with suspected hearing pathology were tested with a comprehensive audiological examination. In the CHD group hearing impairment was detected in 29% of children, including sensorineural hearing loss (SNHL) in 12%. And in the comparison group, 3.6% of infants had a hearing loss, SNHL was defined in 1%. Analysis of the data revealed the most significant risk factors for hearing impairments in children with CHD and it had showed the difference in the structure of the risk factors between the both groups. In the study group was registered a case of auditory neuropathy spectrum disorders. This case illustrated the importance of conducting hearing screening by means of click-evoked auditory brainstem responses and not only of registration of otoacoustic emissions. One child with CHD was found to have delayed SNHL, which requires long-term monitoring of hearing in children with cardiopathology.