The Feasibility of Performing Vestibular Newborn Screening

In this issue of Pediatrics, Martens et al¹  report on their 3-year progress using cervical vestibular evoked myogenic potentials (cVEMPs) to screen for vestibular loss in infants diagnosed with sensorineural hearing loss in Flanders (northern Belgium). The cVEMP is a myogenic response measured at the contracted sternocleidomastoid muscle in response to high intensity, short duration, acoustic stimuli. The authors used tone bursts (500 Hz) elicited with a standard clinical bone oscillator. The cVEMP reflects inferior vestibular nerve and saccule function and can be recorded in infants as young as a few days with a head turn.^2–5  cVEMPs are measured with the same evoked potential equipment as auditory brainstem responses, which are often used to confirm the presence of hearing loss, thus making cVEMP the ideal tool for screening vestibular function in infants.

Martens et al¹  propose using cVEMPs to screen all children diagnosed with sensorineural hearing loss. In the last 3 years of the screening program, 35 of 254 (13.8%) infants with sensorineural hearing loss had abnormal cVEMP results. Of those, 32 (91.4%) had severe to profound sensorineural hearing loss. Additionally, abnormal cVEMPs were more likely to occur in children with early onset (eg, from congenital cytomegalovirus or meningitis) versus congenital hearing loss. These data provide support that vestibular screening can be completed as part of the newborn hearing screening process, that cVEMPs are an adequate tool for screening, and that the primary population of children at risk for vestibular loss are those with severe to profound, acquired sensorineural hearing loss.

There are several benefits of integrating vestibular testing at the time of universal newborn hearing screening. First, children who fail their vestibular screening could be referred for a comprehensive vestibular evaluation involving tests of semicircular canal and gross motor function to better characterize the extent and functional effects of vestibular loss. Second, infants and young children can begin vestibular rehabilitation to work on gross motor skill acquisition immediately (J. Christy, PhD, e-mail communication, March 18, 2022). Third, parents can be counseled on the effects of vestibular loss and the importance of vestibular rehabilitation at an early age. However, despite these benefits, there are several obstacles for implementing a large-scale screening/evaluation process. The primary obstacle is the paucity of audiologists and physical therapists with experience and training in the assessment and rehabilitation of pediatric vestibular loss, particularly in infants. Given this critical limitation, it may not be feasible to immediately implement large scale screening practices; however, trends in the data provide a framework for implementing pediatric vestibular testing for infants at higher risk.

Vestibular loss has previously been associated with hearing loss and gross motor delay in children^6,7  and is more likely to occur with specific etiologies and increasing severity of hearing loss.^8–10  Additionally, children with vestibular loss are late to acquire gross motor skills such as holding their head upright, sitting, and walking independently.^9,11  Given these trends, it seems reasonable that vestibular testing be recommended in the following groups of children: (1) children with severe to profound sensorineural hearing loss, which justifies testing all children before cochlear implantation; (2) children who pass their newborn hearing screening and acquire hearing loss or have progressive hearing loss; and (3) children with any degree of hearing loss and concomitant gross motor delay.

This modified approach to pediatric vestibular testing is dependent upon several action items that are urgently needed to address this problem. First, the number of qualified practitioners who assess and treat pediatric vestibular loss is lacking and needs to increase. At minimum, having at least 1 qualified center in every state or geographic region that can complete pediatric vestibular testing and rehabilitation is recommended. Second, the best tool for quantifying the presence and degree of vestibular loss in infants needs to be determined. In addition to cVEMP, rotary chair or video head impulse testing using a remote camera are also options.^12–16  Third, the effect of pediatric vestibular loss needs to be adequately characterized. Like adults, children with vestibular loss demonstrate reduced dynamic visual acuity, increased imbalance, falls, and even reading and academic deficits;^9,11,17–29  however, additional work is needed to characterize the interactions among these consequences, determine whether there are additional consequences (ie, reductions in quality of life), and the cascading effects of these consequences on children and families; particularly defining how vestibular loss affects the academic performance of these children once they reach school age. Lastly, additional work is needed investigating the effectiveness of vestibular rehabilitation in remediating these functional effects in children. Defining the consequences of vestibular loss and the effectiveness of treatment provides further justification for the importance of early diagnosis and rehabilitation.

Overall, we commend Martens et al for their pioneering efforts in moving the field of pediatric vestibular assessment and intervention forward. If implementing vestibular screening in all children with sensorineural hearing loss is not feasible, then at minimum, vestibular screening for children with severe-to-profound and/or acquired/progressive sensorineural hearing loss should be considered; especially those with gross motor delay.

cVEMP

cervical vestibular evoked myogenic potential