Multiple congenital anomalies and adverse developmental outcomes are associated with neonatal intensive care admission and unilateral hearing loss

Abstract

AimTo determine congenital and developmental outcomes of children with Unilateral Hearing Loss (UHL) who were admitted to the Neonatal Intensive Care Unit (NICU).MethodRetrospective, single-site study that followed 25 children with permanent congenital UHL and a NICU admission to a NICU of Nottingham University Hospital. Birth and two-year developmental follow-up data were collected. They were compared to matched control group who had a NICU admission but no hearing loss (matched on gestational age, weight and sex).ResultsThe median birthweights, gestational ages and number of days spent on the NICU for the UHL population were 2510 g, 36 weeks, and 12 days respectively. Most children (20/25; 80%) with UHL and a NICU admission were diagnosed with a congenital anomaly within the first two years of life. Only half (13/25) of these children were diagnosed with a congenital anomaly at discharge. Children with UHL and a NICU admission were more likely than the matched group (NICU admission only; p < .001) to have multiple congenital anomalies. We found a positive association between multiple congenital anomalies and developmental impairment for the NICU graduates with UHL (p = .019). This UHL-NICU group were also more likely than the matched NICU children to have developmental impairment (7/25 vs. 0/25; p = .01), speech and language therapy (13/25 vs. 1/25; p < .001), inner ear malformations (14/25 vs. 0/25, p < .001) or craniofacial anomalies (12/25 vs. 2/25; p = .004).InterpretationChildren with UHL and a NICU admission were at high risk of congenital anomalies and certain adverse developmental outcomes. Improved congenital anomaly screening is needed at birth for this population. Having multiple congenital anomalies suggests closer developmental monitoring is needed. This study contributes towards producing clinical screening and management guidelines to ensure consistent high-quality care for this unique population.

Characterisation of the treatment provided for children with unilateral hearing loss

“…The genetic testing revealed four pathogenic variants, and two had variants of unknown significance. The genetics data have previously been cited in a recent study ( 44 ).…”

Unilateral Sensorineural Hearing Loss in Children: Etiology, Audiological Characteristics, and Treatment

“…Specifically, single-sided deafness (SSD) refers to profound sensorineural HL or nonfunctional hearing in one ear, while the other ear maintains normal hearing [ 3 ]. The prevalence of congenital UHL is about 0.3–1 per 1000 newborns, with a remarkable increase (1.2–4.6 per 100) in newborns admitted to the neonatal intensive care unit [ 4 ]. However, it is important to underline that most children with mild HL and those with late-onset or acquired HL are not identified by universal newborn hearing screening [ 1 , 5 ].…”

UK and US risk factors for hearing loss in neonatal intensive care unit infants

“…Previous data indicate that other neonatal factors; e.g; mechanical ventilation [26] oxygen therapy [27] and hyperbilirubinemia [28] are also associated with HL. Recently there has been interest in co-morbidities of multiple risk factors for HL [14] and Chant et al, 2022 have shown cumulative doses of ototoxic medication are associated with increased risk of HL in preterm infants.…”

“…There are serious negative consequences associated with undetected HL such as speech, language and cognitive delays, poor social adjustment, and academic achievement [11][12][13]. Physical and psychological morbidities may also co-exist with HL [14] and more than a quarter of children with HL have additional disabling conditions [15,16]. An aetiologic factor can be identified in 50-60% of HL cases -a genetic cause is present in 60% of infants with HL, peripartum problems in 21%, and cytomegalovirus (CMV) infection in 19% [17][18][19][20][21].…”

“…Previous data indicate that other neonatal factors; e.g; mechanical ventilation [26] oxygen therapy [27] and hyperbilirubinemia [28] are also associated with HL. Recently there has been interest in comorbidities of multiple risk factors for HL [14] and Chant et al, 2022 have shown cumulative doses of ototoxic medication are associated with increased risk of HL in preterm infants. There is international consensus that early diagnosis and follow-up of HL in children is important for them to achieve satisfactory language outcomes [13,29,30] Highly successful implementation of automated auditory brainstem response (AABR) hearing screening has been demonstrated in the NICU setting and there is good evidence that newborn hearing screening programmes (NHSP) achieve early identification of HL [31].…”