Vestibular Assessment in Infants with Congenital or Early Onset Sensorineural Hearing Loss: Is Neonatal Screening Required?

Background/Objectives: Children with congenital or early onset sensorineural hearing loss (SNHL) are at a greater risk of vestibular dysfunction (VD), hypothesised to occur from the close embryological relationship between the cochlear and vestibular systems. Even with increasing focus on early detection and rehabilitation through Universal Newborn Hearing Screening Programmes in many countries, few studies have focused on the prevalence and feasibility of vestibular assessment on infant populations. The objectives of this review are to 1. Identify the prevalence of VD infants with congenital or early onset SNHL, 2. Identify which vestibular assessment tests/protocols are conducted on this population, 3. Report sensitivity and specificity values for identified vestibular assessment tests/protocols. Methods: Studies including infants aged 0-12 months, with congenital or early onset SNHL of any laterality, degree and configuration and underwent any method of vestibular assessment. The review adhered to the Joanna Briggs Institute (JBI) guidance and the PRISMA-ScR extension statement. Results: A total of 14 studies were included in the review. All articles reported that infants with congenital or early onset SNHL are at a greater risk of VD, particularly those with bilateral severe-profound SNHL. The cVEMP test was the most frequently identified vestibular assessment tool utilised within this age demographic. Conclusions: Results from the included articles coincide with results from literature assessing older paediatric populations. cVEMPs have been reported to be a feasible, sensitive, and specific screening tool in infants with congenital or early onset SNHL. The prevalence of VD in infants with congenital or early onset SNHL justify further investigation on the feasibility of establishing a pathway for vestibular assessment for all infants referred by UNHS programmes.