Hidden hearing loss in hereditary demyelinating neuropathies: insights from Charcot-Marie-Tooth mouse models.

Hidden hearing loss (HHL) is a recently described auditory neuropathy characterized by normal audiometric thresholds but reduced sound-evoked potentials. It has been proposed that HHL contributes to hearing difficulty in noisy environments in people with normal audiometric thresholds, a widespread complaint. While most studies on HHL pathogenesis have focused on inner hair cell (IHC) synaptopathy, recent research suggests that transient auditory nerve (AN) demyelination may also cause HHL. To test the impact of myelinopathy in a clinically relevant model, we studied a mouse model of Charcot-Marie-Tooth type 1A (CMT1A), the most prevalent hereditary peripheral neuropathy in humans. CMT1A mice exhibit the functional hallmarks of HHL, together with disorganization of AN heminodes near the IHCs with minor loss of AN fibers. Our results support the hypothesis that mild disruptions of AN myelination can cause HHL, and that heminodal defects contribute to the alterations in action potential amplitudes and latencies seen in these models. Also, these findings suggest that patients with CMT1A or other mild peripheral neuropathies are likely to suffer from HHL. Furthermore, these results suggest that studies of hearing in CMT1A patients might help develop robust clinical tests for HHL, which are currently lacking.

Bringing Art to Life: Social and Activity Engagement through Art in Persons Living with Dementia.

OBJECTIVES: The Bringing Art to Life (BATL) program was created as an art therapy intervention to increase social engagement, intergenerational engagement, and creative expression among persons living with dementia (PWD) in an adult day service. This is an evaluation of BATL program efficacy. METHODS: A modified behavioral observation tool was used to quantify meaningful engagements as recorded in ethnographic field notes and test the hypotheses that: 1) engagements increase across art therapy sessions, and 2) engagements were more social than activity or art related. RESULTS: The increase in engagement across session was not significant. However, social engagements (M = 9.61, SD 4.6) were more prevalent than art engagements (M = 5.23, SD = 3.1) in each session (t(99) = 9.52,). A thematic analysis of social interactions recorded in the ethnographic field notes revealed themes related to reminiscence. CONCLUSIONS: The quantitative and qualitative data supported the BATL program as effective in engaging participants in social interaction and art activities. Specifically, quantitative data showed participants were engaged in conversations around reminiscence and discussion of creative expression with students. CLINICAL IMPLICATIONS: Intergenerational social interaction between PWD and college students is a critical and meaningful part of the BATL program, and the intervention created a safe space with focused activity through engagement with art, and this facilitated PWD in self-expression, including humor, advice, and legacy.

Supporting Equity and Inclusion of Deaf and Hard-of-Hearing Individuals in Professional Organizations.

Disability is an important and often overlooked component of diversity. Individuals with disabilities bring a rare perspective to science, technology, engineering, mathematics, and medicine (STEMM) because of their unique experiences approaching complex issues related to health and disability, navigating the healthcare system, creatively solving problems unfamiliar to many individuals without disabilities, managing time and resources that are limited by physical or mental constraints, and advocating for themselves and others in the disabled community. Yet, individuals with disabilities are underrepresented in STEMM. Professional organizations can address this underrepresentation by recruiting individuals with disabilities for leadership opportunities, easing financial burdens, providing equal access, fostering peer-mentor groups, and establishing a culture of equity and inclusion spanning all facets of diversity. We are a group of deaf and hard-of-hearing (D/HH) engineers, scientists, and clinicians, most of whom are active in clinical practice and/or auditory research. We have worked within our professional societies to improve access and inclusion for D/HH individuals and others with disabilities. We describe how different models of disability inform our understanding of disability as a form of diversity. We address heterogeneity within disabled communities, including intersectionality between disability and other forms of diversity. We highlight how the Association for Research in Otolaryngology has supported our efforts to reduce ableism and promote access and inclusion for D/HH individuals. We also discuss future directions and challenges. The tools and approaches discussed here can be applied by other professional organizations to include individuals with all forms of diversity in STEMM.

Accessibility to Hearing Healthcare in Rural and Urban Populations of Alabama: Perspectives and A Preliminary Roadmap for Addressing Inequalities.

Hearing loss is a growing public health concern and has been associated with poor cardiovascular health, diabetes, increased social isolation and poor cognitive functioning. Addressing this issue, especially in rural communities, will require increased awareness of hearing loss and its link to emotional and physical well-being. The purpose of this study was to understand the challenges that those with hearing loss living in rural and urban communities experience and to examine the feasibility of using primary care physicians to assist with improving access to hearing healthcare in rural communities. One hundred thirty-four participants were recruited from rural and urban counties in West Central and South Alabama. All participants completed a hearing evaluation and a Healthcare and Hearing Healthcare Accessibility Questionnaire. Over half of the adults in the study with hearing loss did not have access to hearing healthcare because of distance to a hearing healthcare professional. Other reasons for participants not having access to hearing healthcare included financial constraints, lack of awareness of having a hearing loss, lack of time to see a hearing healthcare provider, and not knowing how to access a provider. Results, however, revealed that most adults in the study had access to a primary care professional. The primary care provider, therefore, could be a valuable resource for the dissemination of information related to hearing healthcare. Collaborative work with primary care providers will help to develop and expand hearing healthcare awareness, research and services provided through the Here Hear Alabama project, a rural outreach initiative in West Central and South Alabama.