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Priority Areas in Hearing and Balance Research
Priority Area 1: Understanding Normal Function
- Development of the Auditory and Vestibular System: Identify the molecules and the genetic and epigenetic changes involved in development of the peripheral and central auditory and vestibular pathways. Understand how auditory neurons establish tonotopic and other organized sensory representations.
- Homeostasis and Microenvironment: Increase understanding of homeostasis in the inner ear (e.g., ionic composition and maintenance, inflammatory response and toxin elimination, blood-labyrinth barrier, microcirculation, hormonal and other control systems), transport of macromolecules through the round window and in the middle ear (e.g., gas exchange, fluid regulation, innate immunity, and gene expression) and how these homeostatic mechanisms are established developmentally.
- Mechanics: Expand knowledge of three-dimensional mechanics in the cochlea (e.g., interaction of hair cell membranes and stereocilia with supporting structures); in the middle ear (e.g., resolve important issues of middle ear mechanics, including tympanic membrane/ossicular coupling and modes of stapes motion); and in the vestibular system (e.g., cupular and otolithic maintenance of posture and equilibrium).
- Sensory Cell Transduction and Innervation: Identify all the molecular constituents of the hair cell transduction process: nanomechanical properties, molecular motors in hair cell membranes and stereocilia, ion channels and pumps; and their integration for hair cell tuning and maintenance. Identify the factors that promote and maintain hair cell afferent synapses.
- Single Cell Analysis: Define the gene expression profile at the single cell level for multiple different cell types and regions in the cochlea over multiple different time points.
- Functional Connectivity: Clarify how afferent and efferent neural circuits process auditory and vestibular peripheral input. Understand how coding schemes influence plasticity and enable attention, cognition, and stress. Incorporate advanced techniques of functional and structural neural imaging and connectivity, ranging from molecular to systems scale. Bridge non-invasive lower-resolution assessments (imaging and electrophysiological methods – ECoG) of complex sounds (speech) obtained in humans with combined invasive/non-invasive higher-resolution assessments in animal models.
- Auditory System: Determine how sound detection, discrimination, and recognition interact with learning, memory, and attention as well as with vision, tactile sensation, and balance to better understand auditory perception in real-world listening environments, especially in conditions with unfavorable low signal-to-noise ratios.
- Vestibular System: Determine how vestibular, visual, and proprioceptive (the sensing of motion or position) systems interact to perceive space and motion and to maintain orientation.
Priority Area 2: Understanding Diseases and Disorders
- Epidemiology: Investigate natural history; genetic and environmental risk factors; racial, ethnic, and gender differences; and practical objective metrics for subpopulations to inform the development of evidence-based treatment strategies. Explore how complex comorbidities create differences in disease phenotypes and treatment outcomes.
- Genetic Causes of Hearing Loss: Leverage new genetic tools and big data to study genotype and phenotype relationships, e.g., genetic risk factors in noise-induced and age-related hearing loss. Test emerging ideas with animal models using cutting-edge gene-editing technologies (CRISPR). Define the spectrum of genetic contributions to inherited, noise-induced and age-related hearing loss and understand the structural and functional consequences of such mutations. Identify the spectrum of mutations in non-coding sequences that contribute to hereditary hearing loss.
- Single Cell Analysis: Define the gene expression profile at the single cell level for multiple different cell types and regions in the cochlea over multiple different time points in diseased or disordered tissue.
- Otitis Media: Improve understanding of susceptibility and pathogenesis related to genetics, prior upper respiratory infection, eustachian tube dysfunction and reflux, bacterial biofilms and microbiome, polymicrobial infections, dysregulation of innate immunity, inflammation and mucus production, mucosal hyperplasia, and dysregulation of the resolution of inflammation and tissue repair. Define immune pathways for effective middle ear protection by vaccines and for identification of new therapeutic targets. Develop animal models of acute and chronic otitis media. Determine impact of vaccination on disease prevalence and infection by other microbes.
- Inflammatory and Autoimmune Responses of the Inner Ear: Identify and characterize first responders to injury in the inner ear. Determine how molecules and cells cross the blood-labyrinth barriers to initiate immune response and autoimmune disease. Identify genetic and epigenetic risk factors. Investigate innate and cognate immunity in resolution of otitis media.
- Tinnitus and Hyperacusis: Validate assays for tinnitus and hyperacusis in animal models. Couple behavior and neurophysiology in animals to probe mechanisms. Use human brain imaging to identify networks that are involved in tinnitus and hyperacusis.
- Other Acquired Disorders: Improve understanding of the pathogenesis and processes of noise-induced, age-related, traumatic, idiopathic, ototoxic, neurotoxic, metabolic, and hereditary and non-hereditary auditory and vestibular dysfunction. Acquired disorders of interest include Ménière's disease, otosclerosis, idiopathic sudden sensorineural hearing impairment, and the slow hearing decline after hearing-preservation cochlear implantation. Leverage the use of human temporal bones to better understand the clinical progression of disease and disease treatment.
- Pathways and Damage: Determine how the peripheral and central auditory and vestibular pathways are reorganized following injury. Define the long-term changes resulting from sensory cell or neuronal loss. Identify molecular, genetic, and anatomical underpinnings of plasticity in normal and hearing-impaired models. Use human imaging and electrophysiological methods to assess effects of hearing loss on central speech representations. Research the central neural pathways to better understand the relevance of hearing loss to balance disorders.
- Changes in Perception with Disease:
- Auditory System: Identify sources of variance contributing to large individual differences in response to similar intervention strategies among people with hearing loss. Improve understanding of the time course, sensitive periods, and complications of hearing loss across the lifespan. Clarify the aspects of perceptual impairment that are primarily caused by cochlear synaptopathy rather than by cochlear hair cell loss.
- Vestibular System: Understand how disease affects perception of motion and spatial orientation, including connections with limbic and autonomic systems.
Priority Area 3: Improving Diagnosis, Treatment, and Prevention
- Genetic Testing: Improve comprehensive genetic testing by developing more affordable and faster Targeted Genomic Enrichment and Massively Parallel Sequencing Platforms integrating single nucleotide (SNV) and copy number (CNV) variation detection in coding and non-coding regions. Develop better variant annotating and pathogenicity prediction tools.
- Regeneration: Develop in vitro systems to identify genes and factors that promote regeneration of specific cellular phenotypes (e.g., hair cells, supporting cells, spiral ganglion neurons, cells of the stria vascularis); understand factors that promote or inhibit hair cell regeneration spiral ganglion neurite extension and hair cell synaptogenesis; and determine which genes and extracellular factors control cell-specific differentiation.
- Pharmacotherapeutics: Develop targeted delivery of viral vectors for gene therapy and gene repair/correction and site-specific, controlled, sustained molecular therapy for both developing and dysfunctional pathways. Develop therapies to improve neuronal stimulation, resist cell damage, and enhance cell repair. Determine rules governing the diffusion or transport of small molecules, macromolecules, and viruses across the round window membrane.
- Gene Therapy and Gene Delivery: Develop therapies to prevent progression of hearing loss and/or restore function after hearing loss has occurred; identify and catalog viral and non-viral vectors with cell-specific inner ear tropism.
- Tinnitus and Hyperacusis: Apply advanced imaging techniques to provide measures of changed neural activity in people with tinnitus and hyperacusis. Identify pharmacologic agents to prevent tinnitus resulting from traumatic, ototoxic, degenerative, and other acquired disorders. Identify behavioral, pharmacological, surgical, and device-based treatments for improving tinnitus and hyperacusis.
- Otitis Media: Develop new vaccines including polyvalent vaccines for middle ear bacterial and viral infections including polymicrobial infections. Develop new therapeutic agents to enhance innate immunity and host defense, suppress uncontrolled inflammation, mucus production, and tissue repair and speed resolution of inflammation for the treatment of otitis media. Develop new drug delivery systems to the middle ear to treat both middle ear and inner ear diseases.
- Noise-Induced Hearing Loss: Use evidence-based research to develop strategies for preventing noise-induced hearing loss for workers in construction and agriculture and from recreational noise exposure.
- Interventions for Hearing Loss:
- Expand or combine databases for high-resolution molecular, neurophysiological, and psychophysical diagnostics for evidence-based therapeutic approaches.
- Examine existing and develop better aural rehabilitation strategies across the lifespan. Investigate how aural rehabilitation strategies are affected by treating comorbid conditions that influence success, such as co-occurring issues in children with hearing impairment, dementia, or diabetes.
- Improve the performance of traditional (external) hearing aids in background noise and other real-world settings.
- Improve the efficacy of bilateral auditory implants, short electrode implants, and hybrid cochlear implant/hearing aids in the same or opposite ear in conjunction with auditory/aural rehabilitation, assistive devices, and sign language in home and educational environments. Develop alternative means of stimulating the auditory nerve to provide greater channel resolution of auditory implants. Improve prediction of outcome and maintenance of outcome over time.
- Interventions for Dizziness and Balance Disorders: Develop safe and effective pharmacological treatments for vertigo. Develop vestibular prosthetic devices and minimally invasive surgery for better control of imbalance and vertigo while preserving hearing and other functions. Develop improved behavioral approaches for the rehabilitation of chronic vestibular disorders. Develop improved methods of systematic diagnosis and delineation of subtypes of dizziness and vertigo to identify subpopulations that might respond best to targeted therapies. Further research is needed to determine the impact of aural therapies on balance disorders, such as the effect of a cochlear implant or hearing aids on balance function, and the connection with vestibular migraines.
- Management of Infants and Children with Hearing Impairment: Improve early hearing detection and intervention (EHDI) and hearing loss management, including screening, treatment, and rehabilitation. Define the underserved population of infants and children for hearing health care. Determine if early access to hearing health care changes health outcomes later in life. Develop and evaluate the effectiveness of screening methods. Test the effectiveness of various types of intervention strategies.
- Management of Older Adults: Improve hearing loss management, including screening, treatment, and rehabilitation. Define the underserved population of older adults for hearing health care. Determine if early access to hearing health care changes health outcomes later in life. Develop and evaluate the effectiveness of screening methods. Reduce risk of falls in older adults due to imbalance. Develop assistive balance aids, remote sensing feedback devices, and training programs to improve stability and posture in the elderly.
Priority Area 4: Improving Outcomes for Human Communication
- Identifying Impact of Hearing Loss and of Hearing Health Care: Identify factors that influence a person’s motivation and perceived need for hearing health care. Examine the impact of organization, financing, and management of health care services on the delivery, cost, access to, and outcomes of services. Develop innovative delivery systems (e.g., mHealth) to increase awareness, access, and affordability. Identify cost-effective approaches for diagnosis and treatment. Determine the impact of hearing loss on quality of life and general physical and mental health and impact of intervention—including hearing aids and other technologies and communication strategies—on the same outcome measures in real-world environment. In addition, the research recommendations from the 2016 National Academies of Sciences, Engineering, and Medicine report on Hearing Health Care for Adults: Priorities for Improving Access and Affordability continue to be a high priority.
- Auditory Ecology: Use mobile technologies to better understand the real-life listening and communication needs of children and adults with mild to profound hearing loss.
- Comparative Effectiveness Research and Evidence-Based Medicine: Through clinical trials and epidemiological studies, identify best treatments for a given medical condition for a defined set of individuals. Develop and use clinical registries, clinical data networks, and other forms of electronic health data to inform the conscientious, explicit, and judicious use of current best evidence in making decisions about hearing health care options. Develop generalizable quality of life measures that allow us to compete with other health care priorities.
- Implementation and Dissemination Research: Improve implementation of “best practices” among health care providers to translate advances into routine community practice. Increase dissemination of health information to the public to promote healthy behaviors, including the need for intervention in individuals with hearing loss and the dangers of acoustic overexposure to the long-term health of the ear.
- Community-Based Participation in Research: Promote community-based research to identify factors that influence outcomes for people with hearing and balance disorders in diverse real-world settings. Engage deaf and hard of hearing individuals in community-based research to aid in developing behavioral interventions to improve their quality of life. Develop methods to address communication disorders in diverse populations, considering variations in care and practice settings.
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