Prevalence and Profiles of Late-Onset Hearing Loss in Preschool Children with Autism Spectrum Disorder Who Passed Newborn Hearing Screening in a South East Asian Population.

Prevalence of hearing loss in children with autism spectrum disorder (ASD) is uncertain, as it is more challenging to assess hearing function in children with developmental difficulties (DD). We aimed to determine the prevalence and profiles of hearing loss in preschool children with ASD in a Southeast-Asian population who passed newborn hearing screening. A retrospective study of preschool children with DD (ASD, Global Developmental Delay (GDD), and Speech and Language Delay (SLD)) attending the Child Development Unit (CDU) at our hospital was performed. Three hundred and thirty-three children (ASD: n = 129; GDD: n = 110; and SLD: n = 94) underwent hearing assessments. Of these, 10.8% of children (n = 36, comprising 15 with ASD, 12 with GDD and 9 with SLD) had confirmed hearing loss. Hearing loss was predominantly bilateral in children with ASD and GDD; in those with SLD, unilateral and bilateral hearing loss were equally common. Conductive hearing loss occurred as frequently as sensorineural hearing loss in children with ASD and SLD, but was the dominant subtype in those with GDD. Moderate to severe hearing loss (n = 2) was noted only in children with ASD. Children with ASD and GDD required significantly more audiology visits and procedures to obtain conclusive hearing test results, compared to those with SLD. The need to identify hearing loss and monitor for resolution is particularly important in vulnerable populations with communication deficits, such as in those with ASD.

Do all infants with congenital hearing loss meet the 1-3-6 criteria? A study of a 10-year cohort from a universal newborn hearing screening programme in Singapore.

OBJECTIVE: To evaluate 1) the outcomes of a local universal newborn hearing screening (UNHS) programme and its audiological follow-ups at 3- and 6-month points, 2) the education outcomes of children with congenital hearing loss (CHL). DESIGN: Retrospective study that analysed data containing hearing screening, diagnosis, intervention and schooling information from electronic databases. STUDY SAMPLE: Children aged 5 to 15 years old who were born between 2004-2014 and underwent UNHS in a local hospital. RESULTS: Over a 10-year cohort, 99.4% of 29,972 newborns underwent UNHS; approximately 90% of them were screened by 1 month of age. However, only 10% of the cohort strictly fulfilled the 1-3-6 criteria recommended by the Joint Committee on Infant Hearing. Lost to follow-up (LTF) rate was highest at post-diagnosis (35%). 80% of infants who were intervened between 6 and 48 months of age went to mainstream schools. The remaining 20% had additional disabilities or family factors. CONCLUSIONS: A high UNHS coverage rate may not translate to meeting the 1-3-6 criteria. Despite ease of access to our healthcare system, LTF at post-diagnosis remained high. In the absence of additional disabilities or family factors, infants intervened during the sensitive window could still potentially make it into mainstream schools.

Quality of life outcomes for children with hearing impairment in Singapore.

OBJECTIVES: This study aimed to investigate the hearing-related quality of life (hearing-QOL) of children with hearing loss in Singapore using hearing aids (HAs) and/or cochlear implants (CIs). Their health-related QOL (health-QOL) as well as their families' health-QOL were compared with normally hearing (NH) children and their families. METHODS: This cross-sectional study recruited families (i.e., children aged 2-18 years and their parents) of NH children (n=44), children wearing HAs (n=22) and children wearing CIs (n=14). Hearing-QOL was assessed using the parent-reported Children Using Hearing Devices QOL questionnaire. General health-QOL was assessed using parent and child-reported measures from the PedsQL inventory consisting of the Generic Core Scales, General Well-being Scale and Family Impact Module. The questionnaires were self-administered for children aged 8-18 years, and interviewer-administered for children aged 5-7 years. RESULTS: The NH children and their families had significantly higher general health-QOL scores compared to the children with hearing loss and their families. This indicates that hearing loss significantly impacts on the well-being of children and their families. Congruence between parent and child reports was only observed in the NH group. Parents of children wearing HAs rated their child's overall hearing-QOL significantly higher than parents of children wearing CIs. Family household income was the only significant predictor of child hearing-QOL scores with parents from the middle income families rating their child's hearing-QOL significantly poorer than parents from high income families. CONCLUSION: Overall, NH children and their families have higher self-rated general health-QOL than children with hearing loss and their families, with children using HAs providing higher hearing-QOL than those using CIs. Evaluating both general health and hearing specific QOL from both the child and their parent/caregiver is worthwhile, allowing a more holistic measure of real-life outcomes and better individualised clinical care.

Non-verbal sound processing in the primary progressive aphasias.

Little is known about the processing of non-verbal sounds in the primary progressive aphasias. Here, we investigated the processing of complex non-verbal sounds in detail, in a consecutive series of 20 patients with primary progressive aphasia [12 with progressive non-fluent aphasia; eight with semantic dementia]. We designed a novel experimental neuropsychological battery to probe complex sound processing at early perceptual, apperceptive and semantic levels, using within-modality response procedures that minimized other cognitive demands and matching tests in the visual modality. Patients with primary progressive aphasia had deficits of non-verbal sound analysis compared with healthy age-matched individuals. Deficits of auditory early perceptual analysis were more common in progressive non-fluent aphasia, deficits of apperceptive processing occurred in both progressive non-fluent aphasia and semantic dementia, and deficits of semantic processing also occurred in both syndromes, but were relatively modality specific in progressive non-fluent aphasia and part of a more severe generic semantic deficit in semantic dementia. Patients with progressive non-fluent aphasia were more likely to show severe auditory than visual deficits as compared to patients with semantic dementia. These findings argue for the existence of core disorders of complex non-verbal sound perception and recognition in primary progressive aphasia and specific disorders at perceptual and semantic levels of cortical auditory processing in progressive non-fluent aphasia and semantic dementia, respectively.