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Office of Newborn Screening

Healthcare Providers – Hearing Screening and EHDI

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The Arizona Early Hearing Detection and Intervention Program (EHDI) includes newborn hearing screening follow-up under the Office of Newborn Screening. Follow-up is conducted to meet the goals of:

  • Screening all infants for hearing loss by one month of age
  • Completing diagnostic testing before three months of age for children who fail the newborn hearing screen
  • Enrollment in Early Intervention services as soon as possible (prior to 6 months of age) after diagnosis of hearing loss

In order for the Program to know where infants are in the process of early identification and to provide appropriate follow up, it is critical that providers know when and how to report screening and diagnostic results.

Screening

If infants do not pass their newborn hearing screen in the hospital, it is important that the babies are screened again as soon as possible (no later than 1 month of age). Most can return to the birthing hospital for an outpatient rescreen within the first week or two after discharge. If it is not possible or convenient (or for insurance coverage), the provider can refer the infant to another screening location.

If the infant was screened with the AABR, it is best if AABR is used for the rescreening. NICU graduates (more than a 5-day stay) or infants with risk indicators for hearing loss:

  • Screening Locations
  • Do not rescreen
  • Refer to a pediatric audiologist for diagnostic testing
  • Older infants may need sedation/anesthesia

Diagnostic Testing

Refer to a pediatric audiologist if the infant fails an outpatient screen or is a NICU graduate that failed the inpatient screen. Infants may need a referral from the primary care provider.

Late Onset and Progressive Hearing Losses

Between the newborn period and school age the prevalence of significant hearing loss doubles. This increase in hearing loss is due to:

  • Late onset losses
  • Progressive losses
  • False negative screens
  • Missed newborn screens or loss to follow-up

Although some children present with hearing loss and have no risk factors, there are conditions known to cause hearing loss. The Joint Committee on Infant Hearing recommends that these children receive, at minimum, a diagnostic assessment by 24 to 30 months of age. Risk indicators that are marked with an asterisk are of greater concern for delayed-onset hearing loss and should receive earlier and/or more frequent assessment.

  • Caregiver concern regarding hearing, speech, language, or developmental delay
  • Family history* of permanent childhood hearing loss
  • Neonatal intensive care of more than 5 days or any of the following regardless of length of stay: ECMO*, assisted ventilation, exposure to ototoxic medications (gentimycin and tobramycin) or loop diuretics (furosemide/Lasix), and hyperbilirubinemia that requires exchange transfusion.
  • In utero infections, such as CMV*, herpes, rubella, syphilis, or toxoplasmosis.
  • Craniofacial anomalies, including those that involve the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies.
  • Physical findings, such as white forelock, that are associated with a syndrome known to include a sensorineural or permanent conductive hearing loss.
  • Syndromes associated with hearing loss or progressive or late-onset hearing loss*, such as neurofibromatosis, osteopetrosis, and Usher syndrome. Other frequently identified syndromes include Waardenburg, Alport, Pendred, and Jervell and Lange-Nielson.
  • Neurodegenerative disorders*, such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich ataxia and Charcot-Marie-Tooth syndrome.
  • Culture-positive postnatal infections associated with sensorineural hearing loss*, including confirmed bacterial and viral (especially herpes viruses and varicella) meningitis.
  • Head trauma, especially basal skull/temporal bone fractures* that requires hospitalization.
  • Chemotherapy

Beyond the Newborn Period

The state is required by law to monitor screening, diagnostic, and early intervention results for all Arizona infants through two years of age. Beyond the Newborn Period provides resources to capture data for older infants.

Hearing Screening Guidelines

Statewide and national best practice standards for Universal Newborn Hearing Screening are detailed in the links below:

Family Checklists

Family Checklists have been developed as a type of "road map" for parents and providers of children who have failed their newborn hearing screening tests. There are checklists for well-babies and NICU graduates or babies with risk factors for hearing loss and include developmental milestones, a timeline for when various assessments should be completed, and appropriate next steps.

These checklists may be given to families at the hospital and are included with letters to parents sent from the Office of Newborn Screening Follow-up.

Provider Resources

Evidence based practice resources and competency based training curriculum for screening.

Family-Friendly Resources

Resources to share with families in English and Spanish, including stories and family-to-family support networks.