The case for universal newborn screening

An editiorial from the American Family Physician

In the United States, approximately 12,000 infants per year (or one to three per 1,000 newborns) are born deaf or hard of hearing, making hearing loss one of the most common potentially disabling conditions present at birth.

Exposure to visual or spoken language is vital during infancy and early childhood. Without full exposure to language and early intervention services, children with late-onset or undiagnosed hearing loss, including mild and unilateral loss, can experience considerable delays in development of language, social, and academic skills.

The Early Hearing Detection and Intervention (EHDI) program is a national initiative promoting identification of hearing loss among infants and young children, and facilitating enrollment in early intervention services that help ensure language development. The objectives in Healthy People 2010 support this initiative by calling for an increase in the proportion of newborns who are screened for hearing loss before one month of age, have an audiologic evaluation before three months of age, and are enrolled in appropriate intervention services before six months of age.

Without EHDI programs, the average age of identification for hearing loss is approximately 30 months, which is past the start of the critical period for optimal language acquisition.

Composed of representatives from the fields of otolaryngology, pediatrics, audiology, speech-language pathology, deaf education, and deaf advocacy, the Joint Committee on Infant Hearing (JCIH) has been influencing EHDI program policy since 1969 and providing internationally recognized clinical practice recommendations.

In 2007, the JCIH released its current position statement outlining the principles of the EHDI program, which include recommendations that expand on the Healthy People 2010 objectives. Updates for screening, diagnostic, medical, early intervention, surveillance, and infrastructure recommendations were also included in the statement.

In July 2008, the U.S. Preventive Services Task Force (USPSTF) revised its recommendations on universal newborn hearing screening. Based on an increasing body of evidence from outcome studies of children with early identification of hearing loss, the USPSTF now recommends hearing screening for all newborns.7 In 2006, hearing screening was documented for more than 90 percent of infants born in the United States.

However, newborn screening is only the first step in the EHDI process. Diagnosis and provision of early intervention services are essential for the care of infants and children with hearing loss. Despite their importance, adequate documentation of timely diagnosis and initiation of effective intervention does not exist for many of these children.

 In 2006, nearly two thirds of infants who did not pass their hearing screening did not have a documented diagnosis reported to their state EHDI program. Additionally, documentation of receiving Part C intervention services (i.e., services for infants and toddlers provided by states through the Individuals with Disabilities Education Improvement Act of 2004) existed for only one half of infants diagnosed with hearing loss.

 These numbers represent infants who might not be receiving necessary services and are at risk of language delays. In its 2007 statement, the JCIH highlighted “pediatricians, family physicians, and other allied health care professionals” as constituting the medical home of infants and children with hearing loss.

 The JCIH's recommended guidelines outlined roles and responsibilities of the medical home that will help ensure children with hearing loss receive the follow-up services that are critical to their care. The guidelines also emphasized that health care professionals within the medical home should work as partners with parents of infants identified with any degree of hearing loss. Together, they can identify and access appropriate services to develop a global plan of necessary health and habilitative services. These services include audiologic and early intervention services, as well as referrals to an otolaryngologist, geneticist, and ophthalmologist.

For family physicians, a commitment to become the medical home for these infants and their families is no small task. It requires a collegial interaction with audiologists, interventionists, educators, and various physician consultants. It also requires an awareness of one's own knowledge gaps in the rapidly evolving medical field and a willingness to broaden a commitment to “just-in-time” learning. Additionally, it requires a sense of urgency in guiding families through timely rescreening, completion of audiologic diagnostic testing, and initiation of appropriate intervention—all within the first few months of life.

These actions are critical to the language development of newborns who are deaf or hard of hearing. The availability of new screening technology brings the responsibility of ensuring that appropriate steps are taken for affected infants and their families. More information on newborn hearing screening and the medical home, as well as billing and legislation, is available at http://www.medicalhomeinfo.org/screening/hearing.html.

The link between hearing loss and dementia

Hearing health care is one of the most neglected areas of treatment. There are no pills for hearing loss, the doctor can't write a script. And 15 minutes in a doctor's office, which is what most of us usually get, is not enough to determine whether a person has hearing loss bad enough to require treatment.

Most doctors evaluate hearing by simply asking the patient "how's your hearing?" Many people, particularly men, are dishonest about this because they don't want to be saddled with hearing devices. As a result, they often go undiagnosed until they really can't hear.

So you can imagine the poor folks who may have hearing loss, but who also might be suffering the first signs of dementia. There is, unquestionably, a link between hearing loss and dementia. It's one of those chicken and egg situations. A person who might have dementia might be mistaken for someone who is hard of hearing. Or a person experiencing the first signs of dementia might become more isolated because of hearing loss.

Audiologists can play an important role in detecting early onset dementia or Alzheimer's Disease because they spend more time with patients and they should start adding cognitive testing to their stable of routine tests on their elderly patients, according to Marilyn Reed, an audiologist at Baycrest in Toronto.

Her team was surprised last summer when a psychology student came to Baycrest, a facility for elderly patients, to do cognitive testing on patients. She found that the majority of patients had some degree of cognitive problems.

“We didn’t know that such a huge percentage of our patients had cognitive impairment,” she says. “We didn’t know because most of them behave normally. Since mild cognitive impairment affects memory but doesn’t necessarily affect their behavior, you wouldn’t notice it during the time you spend with them in a clinic for one appointment.”

The 2011 World Dementia Report stated that the world is facing a "global epidemic" of Alzheimer's Disease, with more than 60% of cases going undetected and undiagnosed by health practitioners.
It is evident, therefore, that health care professionals need to do more cognitive testing on elderly patients.

Mild cognitive impairment often goes undetected for months, sometimes years. Audiologists begin to notice it when patients start repeatedly complaining that their hearing aids aren’t working.

“They’re getting frustrated, upset; they’re not wearing their hearing aids. They’re forgetting to change the battery or forgetting to clean them. It’s then we realize they are having difficulty with cognitive impairment.”

But there is good news on the horizon.

The Baycrest Audiology team was awarded a clinical research grant at the fall meeting of the Canadian Academy of Audiology to assess the cognitive status of their patient s and determine a) if this corresponds to the audiologists’ impression and b) if it influences management protocols. A research assistant will use cognitive tests such as the Montreal Cognitive Assessment (MoCA) to assess the cognitive status of audiology patients. The team will report back to the CAA meeting in St. John’s, Newfoundland with the results.

Failure at the Primary Level

Missing the warning signs of early cognition problems come at a price to our society.

 

• The 85 plus population is the fastest growing segment of the Canadian population.

• The prevalence of Alzheimer’s and other forms of dementia increases with age. Its rate doubles every five years.
• ·It is a serious public health concern, as the 7^th leading cause of death in Canada with no prevention or cure.
• It costs the Canadian economy $2 billion a year in health care costs.

Patients and their families also suffer.

• The association between age-related hearing loss and social isolation is commonly accepted and profoundly important.
• Direct causal and neurobiological pathways link loneliness with psychological pathology.
• The more social stimulation and communication opportunities a person with Alzheimer’s Disease has, the more likely surviving brain cells are to restore connections.

Detecting cognitive problems in the early stages isn’t easy.

“The big problem is that people generally in health care find it difficult to tell the difference between cognitive impairment and severe hearing loss because they present themselves similarly. People give inappropriate responses or don’t respond much at all. It may be hearing; it may be cognition.  How do you know?”