Unilateral congenital hearing loss in children: Challenges and potentials

Abstract

The estimated incidence of sensorineural hearing impairment (>40 dB HL) at birth is 1.86 per 1000 newborns in developed countries and 30-40% of these are unilateral. Profound sensorineural unilateral hearing impairment or single sided deafness (SSD) can be treated with a cochlear implant. However, this treatment is costly and invasive and unnecessary in the eyes of many. Very young children with SSD often do not exhibit language and cognitive delays and it is hard to imagine that neurocognitive skills will present difficulties with one good ear. In the current paper we review the most recent evidence on the consequences of unilateral hearing impairment for auditory and neurocognitive factors. While data of both adults and children are discussed, we focus on developmental factors, congenital deafness and a window of opportunity for intervention. We discuss which etiologies qualify for a cochlear implant and present our multi-center prospective study on cochlear implants in infants with one deaf ear. The large, state-of-the art body of research allows for evidence-based decisions regarding management of unilateral hearing loss in children.

Single‐sided deafness and cochlear implantation in congenital and acquired hearing loss in children

“…The estimated incidence of sensorineural hearing impairment (>40 dB HL) at birth is 1.86 per 1000 new‐borns in developed countries and 30%‐40% of these are unilateral . Behavioural and brain findings support the need for an early intervention to optimise the auditory exposure.…”

“…Cochlear implant could be considered as a treatment for patients suffering from profound sensorineural unilateral hearing impairment single‐sided deafness, especially if it is provided at a young age in order to maximise learning opportunities …”

Hearing with One Ear: Consequences and Treatments for Profound Unilateral Hearing Loss

“…Of particular relevance is the provision of CIs for children with SSD. Auditory deprivation negatively affects outcomes with CI, leading to global recognition of the importance of early intervention during the critical auditory development periods [76][77][78][79]. Rerouting solutions have limited utility in pediatric populations, and in most cases are not capable of being readily implemented until after the age of five, when most auditory development has already taken place.…”

“…Unlike rerouting, CI provides stimulation of both auditory pathways. CIs may serve as an important and beneficial alternative, allowing for early intervention by providing additional cues during the critical development phase and before auditory deprivation sets in [77,79,80]. Emerging evidence suggests that the critical period for intervention may play an important role in the long-term outcomes and success of CI in children with SSD [76][77][78][79].…”

Minimal and mild hearing loss in children: Association with auditory perception, cognition, and communication problems

“…Unilateral hearing loss, previously considered a form of minimal hearing loss but not a major clinical concern (Bess et al, 1998), has recently received more attention. That attention has mainly focused on single sided deafness, a severe or profound and important form of unilateral hearing loss, but not considered further here (see Anne et al 2017;van Wieringen et al 2019, for recent reviews). Some studies have also included limited data on mild unilateral hearing loss, but those studies have typically recruited through neonatal screening and, consequently, the samples have been very small (e.g.…”