Cortical Auditory Evoked Potential Testing in Children With Auditory Neuropathy Spectrum Disorder.

PURPOSE: In the present report, we reviewed the role of cortical auditory evoked potentials (CAEPs) as an objective measure during the evaluation and management process in children with auditory neuropathy spectrum disorder (ANSD). METHOD: We reviewed the results of CAEP recordings in 66 patients with ANSD aged between 2 months and 12 years and assessed the relationship between their characteristics (prevalence, morphology, latencies, and amplitudes) and various clinical features, including the mode of medical management. RESULTS: Overall, the CAEPs were present in 85.2% of the ears tested. Factors such as prematurity, medical complexity, neuronal issues, or presence of syndromes did not have an effect on the presence or absence of CAEPs. CAEP latencies were significantly shorter in ears with cochlear nerve deficiency than in ears with a normal caliber nerve. Three different patterns of CAEP responses were observed in patients with bilateral ANSD and present cochlear nerves: (a) responses with normal morphology and presence of both P1-P2complex and N2 components, (b) responses with abnormal morphology and presence of the N2 component but undefined P1-P2complex peak, and (c) entirely absent responses. None of the patients with normal, mild, or moderate degree of hearing loss had a complete absence of CAEP responses. No significant differences were uncovered when comparing the latencies across unaided and aided children and children who later received cochlear implants. CONCLUSIONS: The CAEP protocol used in our ANSD program did inform about the presence or absence of central auditory stimulation. Absent responses typically fit into an overall picture of complete auditory deprivation and all of these children were ultimately offered cochlear implants after failing to develop oral language. Present responses, on the other hand, were acknowledged as a sign of some degree of auditory stimulation but always interpreted with caution given that prognostic implications remain unclear.

Public Health Learning Agenda for Systems Change Toolkit: National Pilot Evaluation Results.

CONTEXT: There have been multiple calls in the United States for public health workforce development approaches that expand practitioner skill sets to respond to profound inequities and improve population health more effectively. However, most workforce models address individual competencies that instead focus on collective approaches to systems change. PROGRAM: In response to this opportunity, the HRSA-funded Regional Public Health Training Centers (PHTCs) and the University of Illinois Chicago Policy, Practice, and Prevention Research Center (P3RC) released Creating a Learning Agenda for Systems Change: A Toolkit for Building an Adaptive Public Health Workforce (the Toolkit) in December 2020. We later supplemented the Toolkit with additional learning activities to launch the Learning Agenda Toolkit Pilot Test (Toolkit Pilot). IMPLEMENTATION: From June to August 2021, 24 diverse teams piloted the Toolkit. Teams completed a multistep process simulating the development of a learning agenda aimed at addressing community health issues and impacting systems change. EVALUATION: We conducted an evaluation process to assess the usability and impact of the Toolkit Pilot to inform its improvement and future implementation. An evaluation subcommittee analyzed worksheets completed by the Pilot Teams that are aligned to the Learning Agenda steps and conducted and analyzed 12 key informant interviews using concepts from the Toolkit Pilot Logic Model. FINDINGS AND DISCUSSION: Evaluation results suggest that most Pilot Teams found that the Toolkit Pilot offered a step-by-step process toward a clear vision that produced a concrete product on how to address community challenges through learning and systems change. Pilot Teams noted that the Toolkit Pilot provided exposure to and a unique focus on systems thinking; however, prior knowledge of systems thinking and systems change was important. Building readiness for systems change and having more time, resources, and technical assistance would be needed for future versions of the Learning Agenda Toolkit.

Cochlear implantation in a patient with mucopolysaccharidosis IVA.

Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation. Although severe progressive sensorineural hearing loss is a common feature of mucopolysaccharidosis IVA, no detailed description of cochlear implantation for mucopolysaccharidosis IVA has been reported. To review the effectiveness and special considerations associated with cochlear implantation in patients with mucopolysaccharidosis IVA, we here report the case of cochlear implantation in mucopolysaccharidosis IVA by a multidisciplinary team. A retrospective chart review was conducted on a 34-year-old female with mucopolysaccharidosis IVA, who received a cochlear implant. Audiometric thresholds, speech perception scores, and cochlear implant processor mapping information were reviewed during the first 12 months following cochlear implantation. The results of audiological tests indicate improved hearing thresholds as well as remarkable enhancement of speech perception skills over 12 months of cochlear implant use. Cochlear implantation improved auditory performance in a mucopolysaccharidosis IVA patient with postlingually severe-to-profound sensorineural hearing loss. The benefits of cochlear implantation could be meaningful for other Morquio patients with progressive hearing loss, although the risks of surgery and anesthesia should be carefully considered by a multidisciplinary team of experts during the cochlear implant candidacy process.

Tablet-Based Hearing Test Among Child Clinical Populations: Performance and Preference.

Background and Introduction: Tablet-based automated audiometry applications are a recent alternative method to perform pure-tone hearing testing. Children, in particular, might benefit from such apps because of the game-like interface. However, how children perceive this alternative approach has not been well studied. This study examined children's preference of hearing test and a relationship between their test preference and hearing test results. Materials and Methods: Forty children 6-12 years of age completed a pure-tone hearing test in both the tablet-based automated (tablet) method and the conventional method. Hearing thresholds were measured at 0.5, 1, 2, 4, 6, and 8 kHz in each ear. An exit interview was conducted to obtain each child's test preference. Results: We found that 59% of the children preferred the tablet method when compared to the conventional method. Preference to the tablet method was stronger in the younger children (6 years) than older children (7-12 years). The linear regression analyses suggest that test preference does not affect the hearing test results in the conventional method, but does negatively affect the results in the tablet method. In addition, poor performance was found among children with a clinical diagnosis, in particular, in the tablet method. Discussion: These results suggest that hearing thresholds may be overestimated in children, especially those with clinical diagnosis such as attention or behavioral issues in the tablet method. Future work is needed to determine which clinical populations have potential benefit from a tablet method. Conclusions: Children's test preference is not a good index of hearing test accuracy.