Home > Newborn Disorders > Hearing impairment
Hearing impairment
Screening
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.
ICD-9
389, Hearing loss
389 is a non-specific code reflecting the clinical finding that does not specify a diagnosis. The diagnosis and code are
determined by the cause of the hearing loss. See Hearing Impairment ICD-9
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for details.
Overview
- 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).
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]
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.
Prevalence
Incidence of neonatal hearing loss in the United States is 1.1/1000 and varies by state from 0.22 to 3.61/1000 [Mehra: 2009]; (Utah 2.2 - 2.4/1000)Inheritance
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
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 on positive screening test
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
- Contact the family and evaluate the infant for potential causes, including infections and syndromes;
- To confirm the diagnosis, work with the following service(s): Audiology and Pediatric Otolaryngology
- see all Audiology services providers (52) in our database
- see all Pediatric Otolaryngology services providers (9) in our database
- For evaluation and ongoing collaborative management, the following service(s) may be helpful: see all Audiology services providers (52) in our database, see all Pediatric Otolaryngology services providers (9) in our database, see all Hearing Services services providers (9) in our database. 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
- availability of early intervention services
- 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 Management
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.Resources
Information & Support
For Professionals
Newborn Hearing Screening (eMedicine)
An article for physicians providing an overview of screening, including methods of screening, information about early intervention,
and references.
Newborn Hearing Screening - Guidelines for the Medical Home (NCHAM)
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A flow chart and information for newborn hearing screening and follow-up from the National Center on Hearing Assessment and
Management.
National Center for Hearing Assessment and Management
Based at Utah State University, NCHAM is the National Resource Center for Early Hearing Detection and Intervention (EHDI)
systems. The site offers numerous resources for providers, including training materials and links to state and national information.
Also provides links to family support organizations.
National Newborn Screening & Genetics Resource Center
Provides information for families and professionals about genetic screening; links to support and advocacy groups; links to
state genetic contacts; newsletters; fact sheets; data reports; publications; and more.
For Parents and Patients
my baby's hearing
Information for families about hearing screening, follow-up, amplification options, and more (a Spanish version of the site
is linked from the home page). Resources for providers include patient education materials, fact sheets, hearing aid information,
Spanish public service announcements, and more. From Boys Town National Research Hospital and the National Institute on Deafness
and Other Communication Disorders.
Hearing, Speech and Vision Services, Utah
HSVS is a public health program of the Utah Department of Health's Community and Family Health Services Division. It provides
statewide consultation, education, and clinical services in the areas of communicative disorders (speech-language development
and hearing) and preschool vision screening.
Congenital Hearing Loss Module (NNSGRC)
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From the National Newborn Screening & Genetics Resource Center, this 17-page learning module for primary care clinicians provides
information about newborn hearing screening, testing, interventions, case examples, and references. Developed as part of
Genetics in Primary Care Training Program.
Communicate with Your Child
Links to brochures in 6 languages providing information about communication options, technology options, and more for parents
of children with hearing loss, from the National Center for Hearing Assessment and Management.
AG Bell, Utah Chapter
A membership organization and information center focusing specifically on children with hearing loss through the auditory
approach. Provides newsletters, journals, and information relating to oral education and the use of technology. Financial
aid programs available for children with hearing loss.
Baby Watch Early Intervention Program
Utah's network of service for children ages birth to three with developmental delays or disabilities.
Utah Parent Center
A statewide non-profit organization, founded in 1984, to provide 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. The Center provides information on support and
advocacy for families of children with special health care needs.
Technical Assistance Alliance for Parent Centers
Links to local Parent Centers which serve as resources for education and training for parents of children with disabilities;
provide local conferences; provide support groups; provide autism information; assist parents in advocacy and finding school
and other local services; and more. Funded by OSEP.
Patient Education
"What Do I Do Now?"
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A brochure for parents of infants who have failed newborn hearing screening, from the Utah Department of Health.
What Can Your Baby Hear?
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A guide to parental assessment of infant hearing from Primary Children's Medical Center.
Services
Disability/Diagnosis-Specific Advocacy
See all Disability/Diagnosis-Specific Advocacy services providers (54) in our database.
Early Intervention Programs
See all Early Intervention Programs services providers (33) in our database.
Schools for the Deaf & Blind
See all Schools for the Deaf & Blind services providers (5) in our database.
For other services related to this condition, browse our Services categories or search our database.
Helpful Articles
ACMG.
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
Samson-Fang, L., Simons-McCandless, M., and 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
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