AABR measurements reflect the function of the auditory system up through the brainstem, including the peripheral auditory system, the eighth nerve, and the brainstem auditory pathway. OAEs reflect the status of the peripheral system extending to the cochlear outer hair cells. In some clinics, OAE testing is performed first with secondary AABR testing if the OAE is failed.
- 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).
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.
- 77% recessive
- 22% dominant
- 1% X-linked
- Contact the family and evaluate the infant for potential causes, including infections and syndromes.
- Confirmatory testing should be completed by the time the infant is 3 months of age. To confirm the diagnosis, work with the following service(s):
- For evaluation and ongoing collaborative management, the following service(s) may be helpful:
- Once hearing loss is confirmed, treatment should be initiated by 6 months of age.
- Educate the family regarding the need for:
- Speech and language therapy
- Hearing amplification to overcome hearing loss
- Evaluation by an ophthalmologist
- Early intervention services
- In Utah, testing for congenital CMV infection was legislatively mandated in 2013 to enable early treatment to help mitigate the effects of the resulting hearing loss.
- Provide information about communication options and hearing technologies.
- Evaluate for the possibility of linked medical conditions, such as heart arrhythmias, vision problems, and kidney problems.
Early Hearing Detection and Intervention (AAP)
Enhances clinical knowledge of the Early Hearing Detection and Intervention (EHDI) program and screening guidelines, and helps to ensure that newborn screening results are communicated to families and reported according to state laws; American Academy of Pediatrics and the Early Hearing Detection and Intervention Program.
Newborn Hearing Screening - Considerations for the Medical Home (AAP) ( 105 KB)
Outlines specific follow-up and reporting that the medical home should do when a newborn or infant does not pass hearing screening tests; American Academy of Pediatrics.
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.
Genetics in Primary Care Institute (AAP)
The goal of this site is to increase collaboration in the care of children with known or suspected genetic disorders. It includes health supervision guidelines and other useful resources; represents a collaboration among the Health Resources & Services Administration, the Maternal and Child Health Bureau, and the American Academy of Pediatrics.
My Baby's Hearing (NIDCD)
Information for families about hearing screening, 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.
Deafness and Hereditary Hearing Loss Overview (GeneReviews)
An expert-authored, peer-reviewed, current disease description that applies genetic testing to diagnosis and management information; sponsored by the U.S. National Center for Biotechnology Information, U.S. National Library of Medicine.
Newborn Hearing Screening (My Baby's Hearing)
Information about specific aspects of newborn hearing screening; 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.
Center for Parent Information and Resources (DOE)
A large resource library related to children with disabilities. Parent Centers in every state provide training to parents of children with disabilities. Lists local conferences, support groups, advocacy tips, and suggestions for finding schools and other local services; Department of Education, Office of Special Education.
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.
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? (UDOH) ( 256 KB)
A brochure for parents of infants who have failed newborn hearing screening; Utah Department of Health.
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) ()
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)
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.
Early Hearing Detection and Intervention Guidelines (AAP) ( 243 KB)
One-page algorithm for assuring documentation of normal newborn hearing screening or appropriate follow-up if negative; American Academy of Pediatrics.
See all Audiology services providers (13) in our database.
See all Disability/Diagnosis-Specific Advocacy services providers (5) in our database.
See all Early Intervention Programs services providers (13) in our database.
See all Hearing Services services providers (14) in our database.
See all Pediatric Otolaryngology services providers (9) in our database.
See all Schools for the Deaf & Blind services providers (1) in our database.
For other services related to this condition, browse our Services categories or search our database.
Childhood Hearing Loss (clinicaltrials.gov)
Clinical trials involving hearing loss. Trials may be recruiting subjects - compensation offered in some cases; registered with the National Institutes of Health.
Cochlear Implants (clinicaltrials.gov)
Listing of clinical trials of which may be recruiting subjects; National Institutes of Health.
Otitis Media with Effusion (clinicaltrials.org)
Clinical trials related to OME in children; National Institutes of Health.
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
|Author:||Richard Harward, AuD - 8/2009|
|Content Last Updated:||1/2017|
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 2007 position statement: Principles and guidelines for early hearing detection and intervention programs.
Pediatrics. 2007;120(4):898-921. 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
Mehra S, Eavey RD, Keamy DG Jr.
The epidemiology of hearing impairment in the United States: newborns, children, and adolescents.
Otolaryngol Head Neck Surg. 2009;140(4):461-72. PubMed abstract