Hearing attention and quality of listening in children with unilateral hearing loss with and without hearing aid.

Objective: To analyse hearing attention and quality of listening in a cohort of children affected by moderate to severe unilateral hearing loss, comparing a group of hearing aid users to children with no hearing aid. Methods: Twenty-four children (12 hearing aid users, and 12 without hearing rehabilitation) underwent audiological evaluation with speech audiometry in quiet and noise, hearing attention tests, and the speech, spatial and quality of hearing questionnaire in the version for parents. Results: Concerning speech audiometry in noise, in the most difficult condition no one in the unaided group achieved a normal speech recognition threshold score (0% vs 11.6 ± 2.7% in the aided group). The selective hearing attention and shifting tests showed fewer errors in the aided group vs. the non-aided group. The questionnaire showed a significant difference between the quality of listening perceived by the parents of the two groups. The mean total scores were 152.8 ± 2.7 in the aided group and 116.1 ± 2.6 in the non-aided group (p > 0.001). Conclusions: Children with unilateral hearing loss without hearing rehabilitation show a lower quality of hearing, especially with regards to divided auditory attention. Hearing rehabilitation should be proposed as soon as possible in children with moderate to severe unilateral hearing loss.

Hyperacusis in Children with Attention Deficit Hyperactivity Disorder: A Preliminary Study.

The association between hyperacusis and developmental disorders such as autism spectrum disorders has been extensively reported in the literature; however, the specific prevalence of hyperacusis in attention deficit hyperactivity disorder (ADHD) has never been investigated. In this preliminary study, we evaluated the presence of hyperacusis in a small sample of children affected by ADHD compared to a control group of healthy children. Thirty normal hearing children with a diagnosis of ADHD and 30 children matched for sex and age were enrolled in the study. All children underwent audiological and multidisciplinary neuropsychiatric evaluation. Hearing was assessed using pure tone audiometry and immittance test; ADHD was diagnosed following the Diagnostic and Statistical Manual of Mental Disorder criteria. Hyperacusis was assessed through the administration of a questionnaire to parents and an interview with children. Hyperacusis was diagnosed in 11 children (36.7%) in the study group and in four children (13.3%) in the control group; this difference was statistically significant (p = 0.03). The preliminary results of this study suggest a higher presence of hyperacusis in children with attention deficit hyperactivity disorder compared to control children. More studies on larger samples are necessary to confirm these results.

Universal newborn hearing screening in the Lazio region, Italy.

BACKGROUND: The introduction of Universal Newborn Hearing Screening (UNHS) programs has drastically contributed to the early diagnosis of hearing loss in children, allowing prompt intervention with significant results on speech and language development in affected children. UNHS in the Lazio region has been initially deliberated in 2012; however, the program has been performed on a universal basis only from 2015. The aim of this retrospective study is to present and discuss the preliminary results of the UNHS program in the Lazio region for the year 2016, highlighting the strengths and weaknesses of the program. METHODS: Data from screening facilities in the Lazio region for year 2016 were retrospectively analyzed. Data for Level I centers were supplied by the Lazio regional offices; data for Level II and III centers were provided by units that participated to the study. RESULTS: During 2016, a total of 44,805 babies were born in the Lazio region. First stage screening was performed on 41,821 children in 37 different birth centers, with a coverage rate of 93.3%. Of these, 38.977 (93.2%) obtained a "pass" response; children with a "refer" result in at least one ear were 2844 (6.8%). Data from Level II facilities are incomplete due to missing reporting, one of the key issues in Lazio UNHS. Third stage evaluation was performed on 365 children in the three level III centers of the region, allowing identification of 70 children with unilateral (40%) or bilateral (60%) hearing loss, with a prevalence of 1.6/1000. CONCLUSIONS: The analysis of 2016 UNHS in the Lazio region allowed identification of several strengths and weaknesses of the initial phase of the program. The strengths include a correct spread and monitoring of UNHS among Level I facilities, with an adequate coverage rate, and the proper execution of audiological monitoring and diagnosis among Level III facilities. Weakness, instead, mainly consisted in lack of an efficient and automated central process for collecting, monitoring and reporting of data and information.

Audiological and radiological characteristics of a family with T961G mitochondrial mutation.

OBJECTIVE: The aim of this study was to describe audiological and radiological characteristics, and other secondary aspects, in a family carrying a T961G mutation in the 12S rRNA mitochondrial gene. DESIGN: Case report. STUDY SAMPLE: Six members of a family participated in an audiological evaluation that included pure-tone audiometry, immittance tests, auditory brainstem responses (ABR), and otoacoustic emissions (OAE). The radiological evaluation was conducted through temporal bone CT scans using a Toshiba 16 channels Aquilon Spirale. Neuropsychiatric evaluation was also administered. RESULTS: Three participants were diagnosed with severe sensorineural hearing loss of cochlear origin and cochlear malformations visible in CT scans. One participant had a mild mixed-hearing loss and no cochlear malformations. Two participants had normal audiological and radiological findings. CONCLUSIONS: We believe our study can provide helpful insight on the clinical findings of a rare mutation, of which few data have been presented in literature.

Modifications of auditory brainstem responses (ABR): observations in full-term and pre-term newborns.

OBJECTIVE: In this study, we have evaluated by means of auditory brainstem responses (ABR), in a population derived from a newborn hearing screening protocol, some aspects of maturation of the auditory pathways in the first months after birth, and the possible repercussions on early treatment. MATERIALS AND METHODS: In this retrospective study newborns were recruited through our hearing screening program, and an ABR evaluation was performed on 339 newborns, that had risk factors or had failed the screening, or both. Such population was divided in two groups for statistical analysis purposes: full-term and pre-term. The initial ABR was pathological in 70 infants. RESULTS: We observed an improvement over time of the estimated hearing threshold in follow-up ABRs in 43 newborns (26 in the full-term group, mean improvement 27.9 dB SPL, and 17 in the pre-term group, mean improvement 34.6 dB SPL); such an improvement might be related to a maturation of the auditory pathways that was not complete at birth. CONCLUSIONS: The auditory system might not be completely developed at birth, and might require some months to complete; hence any early clinical approach should consider the possibility of an overtreatment, and any therapeutic strategy should only be considered once the diagnosis is certain and definitive.