Hearing Loss & Deafness

Other Names

Hearing impairment

Congenital hearing deficit

Congenital deafness

Diagnosis Coding

H91.90, unspecified hearing loss, unspecified ear

Disorder Category

An other newborn disorder



Loss of hearing sensitivity and acuity, from mild to profound, unilateral or bilateral

Tested By

Automated auditory brainstem response testing (ABR or AABR), 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 entire auditory system, 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 centers, OAE testing is performed first with secondary AABR testing if the OAE is failed.


The Joint Committee on Infant Hearing (JCIH) 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 result from problems involving the auditory nervous system (neoplasm and hypoxia, auditory neuropathy/dyssynchrony).
All infants should have newborn hearing screening prior to one month of age. If immediate newborn screening is not possible (e.g., home birth, birth in a center not offering screening, infant discharge before testing is completed), referral should be made to a pediatric audiologist who can perform 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 and AABR performed as part of their screening; in these patients an OAE alone is insufficient.


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]


More than half of childhood hearing loss is genetic. Approximately 70% of genetic sensorineural hearing loss (SNHL) is nonsyndromic (not associated with other known anomalies or abnormalities).
  • 77% recessive
  • 22% dominant
  • 1% X-linked
More than 400 genes have been identified that are associated with hearing loss.

Clinical Characteristics

With treatment starting by six months of age, children have improved language and speech skills, comparable with peers. 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 one month and, if still positive, additional testing to determine the type of hearing loss and possible causes.

Primary Care Management

Upon Notification of the + Screen

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
    • Availability of early intervention services
  • In Utah, testing for congenital CMV infection was legislatively mandated in 2013 to enable early treatment with hope to mitigate resulting hearing loss
  • Provide information about communication options and hearing technologies
  • Evaluate for possibly-linked medical conditions, such as heart arrhythmias, vision problems, and kidney problems.

Specialty Care Collaboration

Initial consultation and ongoing management with a pediatric audiologist during the newborn period. Consultation with an otolaryngologist if anatomical abnormalities exist or for consideration of cochlear implantation. Consultation and ongoing management with audiology; early intervention; speech and language therapy; and the school system during childhood.


Information & Support

The Portal's Hearing Loss and Deafnessmodule provides management information.

For Professionals

Early Hearing Detection and Intervention (AAP)
Overview of the Early Hearing Detection and Intervention (EHDI) program, including information about EHDI programs, fact sheets, resources and tools, and payment resources; American Academy of Pediatrics and the Early Hearing Detection and Intervention Program.

Newborn Hearing Screening - Lost to Follow-Up Considerations (AAP) (PDF Document 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. Includes health supervision guidelines and other useful resources; a collaboration among the Health Resources & Services Administration, the Maternal and Child Health Bureau, and the American Academy of Pediatrics.

For Parents and Patients

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.

Baby Watch Early Intervention Program (UDOH)
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 non-profit 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)
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

Patient Education

Let's Talk About: What Can Your Baby Hear? (Intermountain Healthcare) (PDF Document 76 KB)
A guide to parental assessment of infant hearing.

What Do I Do Now? (UDOH) (PDF Document 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) (PDF Document 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) (PDF Document)
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) (PDF Document 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 (69) in our database.

Disability/Diagnosis-Specific Advocacy

See all Disability/Diagnosis-Specific Advocacy services providers (112) in our database.

Early Intervention Programs

See all Early Intervention Programs services providers (52) in our database.

Hearing Services

See all Hearing Services services providers (15) in our database.

Pediatric Otolaryngology

See all Pediatric Otolaryngology services providers (9) in our database.

Schools for the Deaf & Blind

See all Schools for the Deaf & Blind services providers (12) 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.

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


Author: Richard Harward, AuD - 8/2009
Content Last Updated: 7/2013

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