Poor speech recognition, sound localization and reorganization of brain activity in children with unilateral microtia-atresia

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.

Intelligence among ear deformities and cleft lip and/or alveolus and/or cleft palate patients during 50 years in Israel

“…33 In contrast, more subtle and less severe developmental errors are probably associated with auricle anomalies, including disorganized brain function measured by MRI. 34 Third, post-surgical repair quality of life assessments show that individuals after palate and lip repair show improved satisfaction but report low-quality life scores for speech and speech-related 35 outcomes. Some subsequently undergo several surgical interventions.…”

“…This causes malfunction of associative areas of the brain, such as the parietal–temporal‐occipital, frontoparietal and prefrontal regions 33 . In contrast, more subtle and less severe developmental errors are probably associated with auricle anomalies, including disorganized brain function measured by MRI 34 . Third, post‐surgical repair quality of life assessments show that individuals after palate and lip repair show improved satisfaction but report low‐quality life scores for speech and speech‐related 35 outcomes.…”

Impact of unilateral hearing loss in early development

“…These findings appear to comport with a connectome model where hearing loss, whether unilateral or bilateral, leads to synaptic changes and altered coupling patterns in the brain which may result in cascading effects on other neurocognitive skills [20,59]. More recent findings reveal that even children with untreated unilateral conductive hearing impairments, as a result of unilateral aural atresia, exhibit significant abnormalities in brain organization, with connectome-level changes involving multiple large-scale networks involved in sensory and higher-level cognitive functions [60 ▪ ].…”

Risk factors for Microtia and preventive approaches

“…In classic microtia, there is usually ear canal atresia and auditory ossicular disorders -Konigsmark syndrome (microtia, ear canal atresia and conductive hearing loss). ( 27) (28) Middle ear deformities can range from a narrow canal and minor auditory ossicular disorder to hypoplastic fused auditory ossicles and poor aeration of the mastoid bone. It should be noted that since patients with atresia have ear canals like everyone else, they can cause middle ear inflammation (otitis media) even if they do not have an external auditory canal.…”

“…It is also critical to monitor the middle ear status in the typically formed ear to ensure that optimal hearing is maintained for the better hearing ear. (28) Hearing screening: When infants have microtia and aural atresia, the affected ears will generally fail the newborn hearing screening test (NHS). Regardless of the NHS results, these infants should be referred directly to an audiologist for outpatient diagnostic auditory brainstem response testing.…”