Get More Help in UtahHearing Loss & Deafness
Guidance for primary care clinicians receiving a positive newborn screen result
Screening
Abnormal Finding
Tested By
Description
The Joint Committee on Infant Hearing endorses early hearing detection and intervention for infants with hearing loss. The goal is to maximize linguistic competence and literacy development for children who are deaf or hard of hearing. Without early detection and opportunities for language development, children with hearing loss will fall behind their peers in communication, cognition, reading, and social-emotional development. [Joint: 2019]
- Conductive hearing disorders involve outer and middle ear structures (congenital anomalies, otitis media, etc.).
- Sensory hearing disorders involve the cochlea and inner ear structures (maternal and acquired infections).
- Neural hearing disorders involve problems of the auditory nervous system (neoplasm and hypoxia, auditory neuropathy/dyssynchrony).
All infants should have a newborn hearing screening prior to 1 month of age. If immediate newborn screening is not possible (e.g., home birth, birth in a center not offering screening, or the infant is discharged before testing is completed), a referral should be made to a pediatric audiologist who can perform a screening within the first month of life. Repeat newborn hearing screening is recommended if an infant is readmitted to the hospital for sepsis, meningitis, or hyperbilirubinemia requiring exchange transfusion.
Infants admitted to the NICU for 5 days, or more are at increased risk for auditory neuropathy and should have both an AABR and OAE performed as part of their screening; in these patients, an OAE alone is insufficient.
Clinical Characteristics
Incidence
Inheritance
- 77% recessive
- 22% dominant
- 1% X-linked
Primary Care Management
Next Steps After a Positive Screen
- Contact the family and evaluate the infant for potential causes, including infections and syndromes.
- Refer to an audiologist for repeat testing within 1 month and, if still positive, diagnostic testing to determine the type of hearing loss and possible causes.
Confirming the Diagnosis
If the Diagnosis is Confirmed
- For evaluation and ongoing collaborative management,
the following service(s) may be helpful:
- Audiology (see UT providers [21]) for consultation and ongoing management during the newborn period
- Pediatric Otolaryngology (ENT) (see UT providers [9]) if anatomical abnormalities exist or for consideration of cochlear implantation
- Speech and Hearing Services (see UT providers [29])
- Early Intervention for Children with Disabilities/Delays (see UT providers [51])
- Treatment should be initiated by 6 months of age.
- Educate the family regarding the need for:
- Speech - Language Pathologists (see UT providers [65])
- Hearing amplification to overcome hearing loss
- Evaluation by Pediatric Ophthalmology (see UT providers [4])
- Early Intervention for Children with Disabilities/Delays (see UT providers [51])
Resources
Information & Support
Related Portal Content
Hearing Loss and Deafness
Assessment and management information for the primary care
clinician caring for the child with medium-chain acyl-CoA dehydrogenase deficiency.
Hearing Loss and Deafness (FAQ)
Answers to questions families often have about caring for their
child with hearing loss and deafness.
After a Diagnosis or Problem is Identified
Families can face a big change when their baby tests positive for
a newborn condition. Find information about A New Diagnosis; Caring for Children
with Special Health Care Needs; Assistance in Choosing Providers; Partnering
with Healthcare Providers; Top Ten Things to Do After a
Diagnosis.
For Professionals
Resources for Professionals (My Baby's Hearing)
Contains information about hearing aid validity verification and genetics, and resources for nurses and midwives; Boys Town
National Research Hospital.
Hereditary Hearing Loss and Deafness Overview (GeneReviews)
Detailed information addressing clinical characteristics, diagnosis/testing, management, genetic counseling, and molecular
pathogenesis; from the University of Washington and the National Library of Medicine.
For Parents and Patients
My Baby's Hearing (NIDCD)
Information for families about hearing screening and hearing loss, follow-up, amplification options, and more. A link to the
site in Spanish is on the home page; Boys Town National Research Hospital and the National Institute on Deafness and Other
Communication Disorders.
CMV Public Health Initiative (UDHHS)
Information related to House Bill 81, enacted in 2013, that mandates screening all infants who fail newborn hearing screening
for congenital cytomegalovirus infection, with the intent to enable early treatment to mitigate the impact on hearing; Utah
Department of Health & Human Services (Formerly UDOH).
Universal Newborn Hearing Screening (My Baby's Hearing)
Information about specific aspects of newborn hearing screening, as well as information for families who have recently received
a diagnosis; Boys Town National Research Hospital.
Communicate with Your Child (NCAM)
Links to brochures in 6 languages providing information about communication and technology options for children with hearing
loss; National Center for Hearing Assessment and Management.
Baby Watch Early Intervention Program (UDHHS)
Provides excellent information on Utah's network of services for children (birth to 3 years) with developmental delays or
disabilities; Utah Department of Health (Formerly UDOH), Bureau of Child Development.
Utah Parent Center
A nonprofit organization that provides training, information, referral, and assistance to parents of children and youth with
all disabilities including physical, mental, hearing, vision, learning, behavioral, and emotional. Staff consists primarily
of parents of children and youth with disabilities.
Center for Parent Information and Resources (DOE)
Parent Centers in every state provide training to parents of children with disabilities and provide information about special
education, transition to adulthood, health care, support groups, local conferences, and other federal, state, and local services.
See the "Find Your Parent Center Link" to find the parent center in your state.
National Center for Hearing Assessment and Management (USU)
A national resource center for the implementation and improvement of comprehensive and effective Early Hearing Detection and
Intervention (EHDI) systems; Utah State University.
Patient Education
Let's Talk About: What Can Your Baby Hear? (Intermountain Healthcare) (
76 KB)
A guide to parental assessment of infant hearing.
What Do I Do Now? (UDHHS) ( 256 KB)
A brochure for parents of infants who have failed newborn hearing screening; Utah Department of Health & Human Services (Formerly
UDOH).
Tools
1-3-6 Newborn Hearing Checklist (AAP) ( 105 KB)
Checklist for assuring early detection and intervention for infants born with hearing loss; American Academy of Pediatrics
and the Early Hearing Detection and Intervention Program.
ACT Sheet for Congenital Hearing Loss (ACMG) ( 283 KB)
Contains short-term recommendations for clinical follow-up of the newborn who has screened positive; American College of Medical
Genetics.
Confirmatory Algorithms for Hearing Loss (ACMG) ( 184 KB)
An algorithm of the basic steps involved in determining the final diagnosis of an infant with a positive newborn screen; American
College of Medical Genetics.
Services for Patients & Families in Utah (UT)
| Service Categories | # of providers* in: | UT | NW | Other states (3) (show) | | NM | NV | RI |
|---|---|---|---|---|---|---|---|---|
| Audiology | 21 | 3 | 22 | 8 | 24 | |||
| Disability Rights Groups | 26 | 7 | 17 | 15 | 12 | |||
| Early Intervention for Children with Disabilities/Delays | 51 | 3 | 34 | 31 | 13 | |||
| Pediatric Ophthalmology | 4 | 1 | 6 | 6 | 8 | |||
| Pediatric Otolaryngology (ENT) | 9 | 1 | 11 | 5 | 7 | |||
| Special Education/Schools | 43 | 3 | 82 | 9 | 35 | |||
| Speech and Hearing Services | 29 | 7 | 36 | 12 | 20 | |||
| Speech - Language Pathologists | 65 | 4 | 23 | 11 | 32 | |||
For services not listed above, browse our Services categories or search our database.
* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.
Studies
Otitis Media with Effusion (ClinicalTrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.
Childhood Hearing Loss (ClinicalTrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.
Cochlear Implants and Children (ClinicalTrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.
Helpful Articles
PubMed search for deafness or hearing loss in children and neonatal screening, last 2 years.
American College of Medical Genetics Newborn Screening Expert Group.
Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss. Genetic Evaluation of Congenital Hearing
Loss Expert Panel. ACMG statement.
Genet Med.
2002;4(3):162-71.
PubMed abstract / Full Text
Guideline reaffirmed by the ACMG in 2005
Samson-Fang, L., Simons-McCandless, M., & Shelton, C.
Controversies in the field of hearing impairment: Early identification, educational methods, and cochlear implants.
Infants and Young Children.
2000;12(4):77-88.
PubMed abstract
Kadambari S, Williams EJ, Luck S, Griffiths PD, Sharland M.
Evidence based management guidelines for the detection and treatment of congenital CMV.
Early Hum Dev.
2011;87(11):723-8.
PubMed abstract / Full Text
Page Bibliography
American College of Medical Genetics Newborn Screening Expert Group.
Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss. Genetic Evaluation of Congenital Hearing
Loss Expert Panel. ACMG statement.
Genet Med.
2002;4(3):162-71.
PubMed abstract / Full Text
Guideline reaffirmed by the ACMG in 2005
Hall JW 3rd, Smith SD, Popelka GR.
Newborn hearing screening with combined otoacoustic emissions and auditory brainstem responses.
J Am Acad Audiol.
2004;15(6):414-25.
PubMed abstract
Provides sensitivity and specificity for newborn hearing screening.
Joint Committee on Infant Hearing.
Year 2019 position statement: Principles and guidelines for early hearing detection and intervention programs.
Journal of Early Hearing Detection and Intervention.
2019.
/ Full Text
Kadambari S, Williams EJ, Luck S, Griffiths PD, Sharland M.
Evidence based management guidelines for the detection and treatment of congenital CMV.
Early Hum Dev.
2011;87(11):723-8.
PubMed abstract / Full Text
Llanes EG, Chiong CM.
Evoked otoacoustic emissions and auditory brainstem responses: concordance in hearing screening among high-risk children.
Acta Otolaryngol.
2004;124(4):387-90.
PubMed abstract
National Center on Birth Defects and Developmental Disabilities.
Research and Tracking of Hearing Loss in Children.
Centers for Disease Control and Prevention; (2019)
https://www.cdc.gov/ncbddd/hearingloss/research.html. Accessed on April 2022.
Samson-Fang, L., Simons-McCandless, M., & Shelton, C.
Controversies in the field of hearing impairment: Early identification, educational methods, and cochlear implants.
Infants and Young Children.
2000;12(4):77-88.
PubMed abstract