Approximately one to three babies per 1,000 births are born deaf or hard of hearing, and about 90% of those children are born to hearing parents.
Universal newborn hearing screening was developed to ensure that all babies are screened at birth to identify potential hearing loss. Babies should be screened no later than one month of age, diagnosed no later than three months of age and receive early intervention no later than six months of age.
Each baby's Primary Health Care Provider plays a key role in ensuring the appropriate follow-up occurs for those babies who did not pass the initial hearing screening and those needing audiologic assessments and evaluations by medical specialists.
The Role of the Primary Health Care Physician
The primary health care provider (PHCP), in cooperation with the audiologist, directs and coordinates, as needed, the evaluation and referral process within the child's medical home by:
Referring a newborn that does not pass a hearing screening to a pediatric audiologist for a diagnostic evaluation.
Providing a statement to parents stressing the importance of follow-up, the time and location of the follow-up appointment, and the telephone number of the screening audiology center.
Referring a baby identified as deaf or hard of hearing to appropriate agencies capable of providing intervention services (http://edn.ne.gov/cms for Nebraska residents) and to appropriate medical specialists (i.e., otolaryngologist and geneticist) as may be indicated by the diagnostic audiologic evaluation.
Monitoring individual cases to assure that the diagnostic evaluation was completed and facilitating the infant's receipt of amplification if needed and linkage to the Early Intervention services.
Providing updates regarding the infant's hearing status to the Nebraska Early Hearing Detection and Intervention Program Department of Health and Human Services.
Providing on-going monitoring and surveillance of ALL children, especially those with risk factors for late-onset and progressive hearing lossJCIH2007-Risk Factors. If the child has any risk factors for late onset or progressive hearing loss, those risk factors need to be reported to NE-EHDI using the Risk Factor Reporting Form.
How is the Hearing Screening Performed
The two screening technologies currently available are Auditory Brainstem Response (ABR) and Otoacoustic Emissions (OAE).
ABR is an electrophysical measure of the auditory nervous system's response to sound. A sound (e.g. click or tone bursts) is presented to the ear via earphones or probe tips, and electrodes record the response as the nerve impulse travels from the cochlea (inner ear) through the auditory nerve to the brainstem. ABR is typically performed by an audiologist as part of a diagnostic evaluation. ABR test results are best interpreted by an audiologist to determine if further testing is needed.
For the purposes of hearing screening, Automated ABR (A-ABR) equipment has been developed. A-ABR screening equipment provides fully automated results interpretation (only a 'pass'/'not pass' outcome). Consequently, the A-ABR allows for others besides audiologists to perform the hearing screening. Other personnel that can be trained include nurses, midwives, technicians, support staff and volunteers. A-ABR may miss a small percentage of hearing losses, such as a mild hearing loss or a low frequency or high frequency hearing loss. A-ABR is sensitive to identifying those children at risk for conductive, sensory, or neural (Auditory Neuropathy Spectrum Disorder) hearing disorders.
OAE evaluates the integrity of the outer hair cells in the cochlea. Thus, the test can identify infants at risk for sensory hearing loss. A sound is presented into the ear canal and a small microphone measures the response from the infant's cochlea (inner ear) that is evoked by the test signal(s). The audiologist analyzes the response to determine how well the inner ear sensory cells are functioning. There are two types of OAE technologies: Transient Evoked Otoacoustic Emissions (TEOAE) and Distortion Product Otoacoustic Emissions (DPOAE).
OAE testing can be fully automated for the purposes of hearing screening and provide a 'pass/not pass' outcome.
OAE responses indicate the status of the peripheral auditory system extending to the out hair cells of the cochlea.
OAE may miss a mild hearing loss in children that are at risk for Auditory Neuropathy Spectrum Disorder.
OAE results are sensitive to sensory hearing loss greater than 40 dB in addition to transient outer ear or middle ear conductive hearing loss.
What to do if patient does not pass the hearing screening
A child not passing the hearing screening should be referred to a pediatric audiologist for an outpatient diagnostic evaluation that typically begins with a second hearing screening. It is important that parents understand that this does not necessarily mean their baby is deaf or hard of hearing. Information about hearing screening should be provided to parents in a professional and compassionate manner while stressing the importance of prompt and appropriate follow-up. Hearing screenings should be completed for an infant within one month of age and a full diagnostic audiologic evaluation should be completed before three months of age.
Which Specialists Should I Refer my Patients
A referral to a specialist such as an otolaryngologist and geneticist may be indicated by the diagnostic evaluation. The majority of children who are born deaf or hard of hearing have a genetic component, even if they do not have syndrome or dysmorphic features.
Risk Factors for Late Onset & Early Childhood Hearing Loss?
The Joint Committee on Infant Hearing (JCIH) 2007 recommends that infants who have a risk factor for late onset or progressive hearing loss should have at least one comprehensive audiologic evaluation by 24 to 30 months of age. Testing should occur any time there is a parent, caregiver, or professional concern regarding communication development.
How Do I Help Families Find Parental Support
Hands and Voices/Guide By Your Side of Nebraska (GBYS)
is a support program for families of children and toddlers who are identified as deaf or hard of hearing. GBYS gives these families an opportunity to meet face-to-face and/or talk with a Parent Guide; a trained and experienced parent of a child who is deaf or hard of hearing. http://www.handsandvoicesne.org
Parent Training Institute Nebraska (PTI) is a statewide resource for families of children with disabilities or special health care needs, enabling parents to have the capacity to improve educational outcomes for their children. http://www.pti-nebraska.org
JCIH Selected Physician Sections
Regional Programs' Roles and Map
Nebraska Audiological Confirmatory Testing Facilities