John Eichwald

Methodology of a multistate study of congenital hearing loss: Preliminary data from Utah newborn screening

A multistate Centers for Disease Control and Prevention (CDC) study was designed to investigate the etiology of congenital hearing loss in infants ascertained through state‐mandated hearing screening or early hearing loss detection and intervention (EHDI) programs. At least 50% of permanent childhood‐onset hearing loss is due to genetic causes, and approximately 20% of all infants with congenital hearing loss have mutations in the GJB2 gene. Another 1% of childhood hearing loss is due to mitochondrial DNA (mtDNA) mutations. The specific aims of this study are to 1) classify the etiology of congenital hearing loss in infants by doing prospective genetic evaluations of all newborns with permanent hearing loss from defined geographic areas, 2) determine the frequency of mutations in GJB2 and two common mitochondrial mutations in these populations, and 3) establish a model infrastructure linking genetic services to statewide EHDI programs. As of April 2003, Utah is the only center evaluating patients. Study subjects identified through the Utah Department of Health EHDI program are contacted by letter and offered a comprehensive medical genetics evaluation with DNA testing for GJB2 and mitochondrial mutations A1555G and A7445G. To date, 25 probands and their immediate family members have been evaluated. We have identified 20 cases with nonsyndromic hearing loss (7 multiplex and 13 simplex), 4 with syndromic hearing loss, and 1 with presumed cytomegalovirus (CMV)‐induced hearing loss. Six of 19 (32%) nonsyndromic cases with sensorineural hearing loss have mutations of one or both alleles of the GJB2 gene, and 21% are homozygous or compound heterozygotes for the 35delG mutation. No A1555G or A7445G mtDNA mutations have been found. Data reported to date include only children born in Utah, but EHDI programs in Hawaii, Rhode Island, and designated areas of Georgia have begun enrolling children in what is now a multistate collaborative study. This is the first comprehensive investigation to determine the etiology of hearing loss from populations ascertained through EHDI programs. The results of this study will facilitate the incorporation of genetic services into EHDI programs.

Vital Signs: Noise-Induced Hearing Loss Among Adults — United States 2011–2012

Introduction The 2016 National Academies of Sciences report “Hearing Health Care for Adults: Priorities for Improving Access and Affordability” included a call to action for government agencies to strengthen efforts to collect, analyze, and disseminate population-based data on hearing loss in adults. Methods CDC analyzed the most recent available data collected both by questionnaire and audiometric tests of adult participants aged 20–69 years in the 2011–2012 National Health and Nutrition Examination Survey (NHANES) to determine the presence of audiometric notches indicative of noise-induced hearing loss. Prevalence of both unilateral and bilateral audiometric notches and their association with sociodemographics and self-reported exposure to loud noise were calculated. Results Nearly one in four adults (24%) had audiometric notches, suggesting a high prevalence of noise-induced hearing loss. The prevalence of notches was higher among males. Almost one in four U.S. adults who reported excellent or good hearing had audiometric notches (5.5% bilateral and 18.0% unilateral). Among participants who reported exposure to loud noise at work, almost one third had a notch. Conclusions and Implications for Public Health Practice Noise-induced hearing loss is a significant, often unrecognized health problem among U.S. adults. Discussions between patients and personal health care providers about hearing loss symptoms, tests, and ways to protect hearing might help with early diagnosis of hearing loss and provide opportunities to prevent harmful noise exposures. Avoiding prolonged exposure to loud environments and using personal hearing protection devices can prevent noise-induced hearing loss.

CDC Grand Rounds: Promoting Hearing Health Across the Lifespan.

Globally, one in three adults has some level of measurable hearing loss, and 1.1 billion young persons are at risk for hearing loss attributable to noise exposure. Although noisy occupations such as construction, mining, and manufacturing are primary causes of hearing loss in adults, nonoccupational noise also can damage hearing. Loud noises can cause permanent hearing loss through metabolic exhaustion or mechanical destruction of the sensory cells within the cochlea. Some of the sounds of daily life, including those made by lawn mowers, recreational vehicles, power tools, and music, might play a role in the decline in hearing health. Hearing loss as a disability largely depends on a persons communication needs and how hearing loss affects the ability to function in a job. The loss of critical middle and high frequencies can significantly impair communication in hearing-critical jobs (e.g., law enforcement and air traffic control).

Use of Personal Hearing Protection Devices at Loud Athletic or Entertainment Events Among Adults — United States, 2018

Tens of millions of U.S. residents have a range of adverse health outcomes caused by noise exposure (1). During 2011-2012, 21 million U.S. adults who reported no exposure to loud or very loud noise at work exhibited hearing damage suggestive of noise-induced hearing loss (2). In addition to the known risk for hearing damage, nonauditory adverse health outcomes and health risks from excessive environmental sound exposure can include effects on the cardiovascular system, metabolism, blood pressure, body weight, cognition, sleep, mental health, quality of life, and overall well-being (1,3,4). CDC analyzed a representative sample of the U.S. adult population (aged ≥18 years) from a 2018 national marketing survey (50 states and the District of Columbia) that included questions about use of hearing protection devices (HPDs) (e.g., ear plugs or ear muffs) during recreational exposure to loud athletic and entertainment events; approximately 8% of respondents reported consistent use of an HPD at these types of events. Among those adults more likely to wear an HPD, 63.8% had at least some college education, and 49.1% had higher income levels. Women and older adults were significantly less likely to use HPDs. These findings suggest a need to strengthen a public health focus on the adverse health effects of excessive noise exposure at home and in recreational settings as well as a need for continued efforts to raise public awareness about the protective value of HPDs.

Hearing health across a lifespan: Hearing screening from cradle to grave

The 2016 National Academies of Sciences, Engineering, and Medicine report “Hearing Health Care for Adults: Priorities for Improving Access and Affordability” included a call to action for government agencies to strengthen efforts to collect, analyze, and disseminate population-based data on hearing loss in adults. In partial response, the Centers for Disease Control and Prevention (CDC) analyzed the most recent available data collected both by questionnaire and audiometric tests of participants aged 20–69 years in the 2011–2012 National Health and Nutrition Examination Survey to determine the presence of audiometric notches indicative of noise-induced hearing loss. Prevalence of both unilateral and bilateral audiometric notches and their association with self-reported exposure to loud noise were calculated. Nearly one in four adults had audiometric notches, suggesting a high prevalence of noise-induced hearing loss. The prevalence of notches was higher among males. Almost one in four U.S. adults who repor...