Hearing Loss & Deafness
Screening
Tested By
Automated auditory brainstem response testing (AABR or ABR), sensitivity=100%, specificity=99.7% [Hall: 2004] or otoacoustic emission testing (OAE), sensitivity of 76.9%, specificity of 90% [Llanes: 2004]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.
Overview
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: 2007]- 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.
Incidence
Incidence of neonatal hearing loss in the United States is about 1:1,000 and varies by state from 0.22:1,000 to 3.61:1,000. [Mehra: 2009]Inheritance
More than half of childhood hearing loss is genetic:- 77% recessive
- 22% dominant
- 1% X-linked
Clinical Characteristics
With treatment starting by 6 months of age, children have improved language and speech skills. Without treatment, children have impaired ability to communicate; decreased speech and language development; and problems with emotional and social development.Follow-up Testing after Positive Screen
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.Primary Care Management
Upon Notification of the + Screen
- 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.
If the Diagnosis is Confirmed
- 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.
Specialty Care Collaboration
Initiate consultation and ongoing management with a pediatric audiologist during the newborn period. Consult with an otolaryngologist if anatomical abnormalities exist or for consideration of cochlear implantation. Consider consultation and ongoing management with audiology; early intervention; speech and language therapy; and the school system during childhood.Resources
Information & Support
For Professionals
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. This site also has links to state chapter EHDI experts
and resources.
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)
Contains health supervision guidelines and other useful resources for the care of children with genetic disorders; American
Academy of Pediatrics.
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 (UDOH)
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.
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.
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 (UDOH)
Provides excellent information on Utah's network of services for children (birth to 3 years) with developmental delays or
disabilities; Utah Department of Health, 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 link for Download a List of Parent Centers across the USA to find the parent center in your state; 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.
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? (UDOH) ( 256 KB)
A brochure for parents of infants who have failed newborn hearing screening; Utah Department of Health.
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) ()
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.
Services in Utah
Early Intervention for Children with Disabilities/Delays
Early Intervention for Children with Disabilities/Delays (see UT providers [49])
For other services related to this condition, browse our Services categories or search our database.
Studies
Childhood Hearing Loss (ClinicalTrials.gov)
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
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