Diagnosis and management of hearing loss in congenital Rubella Syndrome: A literature review

Congenital Rubella Syndrome (CRS) is a condition that results from maternal rubella infection during pregnancy, particularly in the first trimester. This infection can lead to a variety of clinical manifestations in the infant. The virus can cross the placenta and affect fetal development, leading to a range of birth defects. Because of this risk, rubella infection during pregnancy is a major public health concern.

Globally, around 236,000 cases of Congenital Rubella Syndrome (CRS) occur each year in developing countries, with Indonesia reporting a rise in incidence from 3.2 in 2015 to 5.6 per 100,000 in 2017. The risk of CRS is 85% when infection occurs within 12 weeks of gestation and may cause severe congenital defects, including congenital heart defects, cataracts, intrauterine growth retardation with sensorineural hearing loss (SNHL) being the most prevalent. It may be unilateral or bilateral and is often not detected until delays in language development become apparent. SNHL in children impairs speech and language development if untreated, necessitating early intervention. Therefore, newborn hearing screening is essential in infants with suspected or confirmed CRS.

Rubella-induced damage to the cochlea and auditory nerve underlies SNHL, diagnosed through audiological assessments like otoacoustic emissions (OAE), brainstem evoked response audiometry (BERA), and auditory steady-state response (ASSR). Management involves hearing aids, cochlear implants, and speech therapy, with early intervention by 6 months critical to optimize outcomes.

Prevention relies on MMR vaccination and universal newborn hearing screening, particularly in resource-limited settings like Indonesia, where enhanced immunization and screening access are essential to reduce CRS burden.