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Audiological findings in children with a congenital rubella syndrome
Insights
Congenital rubella virus infection can cause hearing loss in children. Audiometry reveals varied hearing loss patterns, not a typical configuration, challenging diagnosis.
Area of Science:
- Otolaryngology
- Pediatrics
- Virology
Background:
- Congenital rubella virus infection is a significant cause of sensorineural hearing loss in children.
- Diagnosing congenital rubella retrospectively can be challenging, often relying on a combination of laboratory tests and physical deformities.
- Affected infants may present with cardiac, central nervous system, ocular, and inner ear abnormalities.
Observation:
- This report details hearing assessments in 22 children with confirmed intrauterine rubella virus infection.
- Audiometric findings in these children were highly differentiated, lacking a consistent or typical pattern.
- High-frequency hearing (up to 20 kHz) was evaluated in a subset of severely hearing-impaired children.
Findings:
- The study did not replicate findings from a previous US report suggesting preserved high-frequency hearing in some profoundly deaf rubella patients.
- Audiometric results showed significant variability, with no specific configuration indicative of congenital rubella.
- Severely hearing-impaired children in this cohort did not exhibit the expected good high-frequency hearing.
Implications:
- The diverse audiometric profiles underscore the complexity of hearing impairment associated with congenital rubella.
- Accurate retrospective diagnosis of congenital rubella requires careful consideration of multiple factors beyond audiometry.
- Further research may explore potential differences in hearing loss patterns between rubella-induced and hereditary hearing impairments.
Abstract:
This is a report of the hearing in 22 consecutive children seen in an audiology clinic because of intrauterine infection with rubella-virus. The diagnosis of congenital rubella may be difficult and sometimes impossible to make retrospectively. It is often a probability diagnosis made by a combination of laboratory tests and various deformities in the heart, the central nervous system, the eyes, and the inner ear. Audiometrically the findings are very much differentiated, and there is no typical configuration. The results reported in a scientific paper from USA mentioning a relatively good high-frequency hearing in some "profoundly deaf" patients have not been found by audiometry up till 20 kHz in a small group of severely hearing impaired rubella-children. But there is reason to suppose that some persons with a hereditary type of hearing impairment may have a relatively well preserved hearing in the high-frequency field.