Abstract
Microtia-atresia is a congenital malformation of the external ear, often affecting one side and being associated with severe-to-profound unilateral conductive hearing loss (UCHL). Although the impact of unilateral hearing loss (UHL) on speech recognition, sound localization and brain plasticity has been intensively investigated, less is known about the subjects with unilateral microtia-atresia (UMA). Considering these UMA subjects have hearing loss from birth, we hypothesize it has a great effect on brain organization. A questionnaire on speech recognition and spatial listening ability was administered to 40 subjects with UMA and 40 age- and sex-matched controls. UMA subjects showed poorer speech recognition in laboratory and poorer spatial listening ability. However, cognitive scores determined by the Montreal Cognitive Assessment (MoCA) and Wechsler Intelligence Scale for Children (WISC-IV) did not differ significantly in these two groups. The impact of hearing loss in UMA on brain functional organization was examined by comparing resting-state fMRIs (rs-fMRI) in 27 subjects with right-sided UMA and 27 matched controls. UMA subjects had increased nodal betweenness in visual networks and DMN but decreases in auditory and attention networks. These results indicate that UCHL in UMA causes significant abnormalities in brain organization. The impact of UCHL on cognition should be further examined with a battery of tests that are more challenging and better focused on the cognitive networks identified.
Highlights
Congenital microtia-atresia manifests as developmental defects of the external ear and, in many subjects, of the middle ear (Luquetti et al, 2011)
Previous studies have reported deficits in speech perception and sound localization in patients with unilateral sensorineural hearing loss (USHL) (Asp & Reinfeldt, 2019; Bess & Tharpe, 1988; Bess et al, 1986; Schmithorst et al, 2014), few have focused on the impact of unilateral conductive hearing loss (UCHL) on these functions in children, with none of these studies evaluating the effects of UCHL on hearing function in subjects with congenital unilateral microtia-atresia (UMA)
Because the impact on academic performance was not as profound in children with UMA as in children with USHL (Kesser et al, 2013), this study evaluated whether the changes in resting-state brain activities observed in subjects with USHL were present in subjects with UCHL due to UMA
Summary
Congenital microtia-atresia manifests as developmental defects of the external ear and, in many subjects, of the middle ear (Luquetti et al, 2011). Previous studies have reported deficits in speech perception and sound localization in patients with unilateral sensorineural hearing loss (USHL) (Asp & Reinfeldt, 2019; Bess & Tharpe, 1988; Bess et al, 1986; Schmithorst et al, 2014), few have focused on the impact of unilateral conductive hearing loss (UCHL) on these functions in children, with none of these studies evaluating the effects of UCHL on hearing function in subjects with congenital unilateral microtia-atresia (UMA). Unilateral hearing loss (UHL), including conductive loss, has been shown to have a negative impact on speech recognition and directional discrimination of sound in noisy environments by children (Asp et al, 2018; Griffin et al, 2019), as well as to negatively affect children’s cognition and academic performance (Anne et al, 2017; Lieu., 2018; Rohlfs et al, 2017; van Hovell Tot Westerflier, 2018). The impact of UCHL on hearing, cognitive function and brain organization in children with UMA should be thoroughly investigated and the need for early hearing intervention in these children should be verified
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