Physicians

What is universal newborn hearing screening?
Universal newborn hearing screening was developed to ensure that all babies are screened at birth to identify potential hearing loss. Babies should be screened before 1 month of age, diagnosed before 3 months of age and receive early intervention before 6 months of age.

What is the incidence of hearing loss in newborns?
Approximately 1 to 3 babies per 1,000 are born with a hearing loss. Of babies with hearing loss, about 90% are born to hearing parents.

How is the newborn hearing screening performed?
The two screening technologies currently available are Auditory Brainstem Response (ABR) and Otoacoustic Emissions (OAE):

ABR
ABR is an electrophysiological measure of the auditory nervous system's response to sound.
A sound (e.g. click or tone bursts) is presented to the ear via earphones or probe tips, and electrodes record the response as the nerve impulse travels from the cochlea (inner ear) through the auditory nerve to the brainstem. ABR is typically performed by an audiologist as part of a diagnostic evaluation. ABR test results are best interpreted by an audiologist to determine if further testing is needed.

For the purposes of hearing screening, Automated ABR (A-ABR) equipment has been developed. A-ABR screening equipment provides fully automated results interpretation (only a 'pass'/'not pass' outcome). Consequently, the A-ABR allows for others besides audiologists to perform the hearing screening. Other personnel that can be trained include nurses, midwives, technicians, support staff, and volunteers.

• A-ABR may miss a small percentage of hearing losses, such as a mild hearing loss or a low frequency or high frequency hearing loss.
• A-ABR is sensitive to identifying those children at risk for conductive, sensory, or neural (Auditory Neuropathy Spectrum Disorder) hearing loss/disorders.

OAE
OAE evaluates the integrity of the outer hair cells in the cochlea. Thus, the test can identify infants at risk for sensory hearing loss. A sound is presented into the ear canal and a small microphone measures the response from the infant's cochlea (inner ear) that is evoked by the test signal(s). The audiologist analyzes the response to determine how well the inner ear sensory cells are functioning. There are two types of OAE technologies: Transient Evoked Otoacoustic Emissions (TEOAE) and Distortion Product Otoacoustic Emissions (DPOAE).

• OAE testing can be fully automated for the purposes of hearing screening and provide a 'pass'/'not pass' outcome.
• OAE responses indicate the status of the peripheral auditory system extending to the outer hair cells of the cochlea.
• OAE may miss a mild hearing loss and children that are at risk for Auditory Neuropathy Spectrum Disorder.
• OAE results are sensitive to sensory hearing loss greater than 40 dB in addition to transient outer ear or middle ear conductive hearing loss.

What is the role of the primary care physician in the Early Hearing Detection and Intervention Program?
The primary care provider (PCP) in cooperation with the audiologist directs and coordinates, as needed, the evaluation and referral process within the child's medical home by:

• Referring a newborn that does not pass a hearing screening to a pediatric audiologist for a diagnostic audiologic evaluation.
• Providing a statement to parents stressing the importance of follow-up, the time and location of the follow-up appointment, and the telephone number of the screening audiology center.
• Referring a baby diagnosed with hearing loss to appropriate agencies capable of providing intervention services and to appropriate medical specialists (i.e., otolaryngologist and geneticist) as may be indicated by the diagnostic audiologic evaluation.
• Monitoring individual cases to assure that the diagnostic audiologic evaluation was completed, and facilitating the infant's receipt of amplification if needed and linkage to Early Intervention services.
• Providing updates regarding the infant's hearing status to the Department of Health upon request.
• Providing on-going monitoring and surveillance of ALL children, especially those with risk factors for late-onset and early childhood hearing loss. (See "What are the risk factors for late-onset and early childhood hearing loss?" for a list of these risk factors) To review the complete Newborn Hearing Screening Program Guidelines, Click HERE.

If my patient did not pass his/her hearing screening, what should I do?
A baby who did not pass his/her hearing screening should be referred to a pediatric audiologist for an outpatient follow-up diagnostic evaluation that typically begins with a second hearing screening. It is important that parents understand that this does not necessarily mean their baby has a hearing loss. Information about hearing screening should be provided to parents in a professional and compassionate manner while stressing the importance of prompt and appropriate follow-up. Hearing screenings should be completed before an infant is one month of age and a full diagnostic audiologic evaluation should be completed before 3 months of age.

What are the types of hearing loss?
There are 4 types of hearing loss:
1) conductive hearing loss,
2) sensorineural hearing loss,
3) mixed hearing loss and
4) Auditory Neuropathy Spectrum Disorder.
For information on these types of hearing loss in easy to understand, non-technical terms, Click HERE.

What specialists should I refer my patient to if he/she is diagnosed with a hearing loss?
Referrals to appropriate medical specialists such as an otolaryngologist and geneticist* may be indicated by the diagnostic audiologic evaluation. * The majority of children with hearing loss have a genetic component even if they do not have a syndrome or dysmorphic features.
Additional medical referrals may need to be made to neurology, cardiology, nephrology and developmental pediatrics. See "What are the risk factors for late-onset and early childhood hearing loss?" for a list of syndromes commonly associated with hearing loss.

How do I help families find parent support?
The Hands & Voices Guide By Your Side Program of PA (GBYS) is a support program for families of infants and toddlers who are diagnosed with a hearing loss. GBYS gives families who recently learned of their child's hearing loss an opportunity to talk to and meet face-to-face with a Parent Guide, a trained and experienced parent of a child with hearing loss. The program is available to all Pennsylvania families with children up to age 3 at no cost. Contact Anne Gaspich, GBYS program coordinator at 1-800-360-7282, ext. 3908 or email: agaspich@pattan.net
Parent to Parent of Pennsylvania provides support to families of children with special health care needs, including children with hearing loss, by linking them to a parent who has a child with the same special health care need. Contact them by calling 1-888-727-2706.

What are the risk factors for late-onset and early childhood hearing loss?
Joint Committee on Infant Hearing (JCIH) 2007 Risk Indicators Associated with Permanent Early Onset and or Late Progressive Hearing Loss in Childhood (* Indicates risk indicators are of greater concern for delayed onset hearing loss):

• Parent or caregiver concern* regarding hearing, speech, language or developmental delay.
• Family history* of permanent childhood hearing loss.
• Neonatal intensive care of >5 days, which may include extracorporeal membrane oxygenation* (ECMO) assisted ventilation, exposure to ototoxic medications (gentamicin and tobramycin) or loop diuretics (furosemide/lasix), and hyperbilirubinemia requiring exchange transfusion.
• In-utero infections such as cytomegalovirus*, herpes, rubella, syphilis, and toxoplasmosis.
• Craniofacial anomalies, including those involving the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies.
• Physical findings such as white forelock, associated with a syndrome known to include sensorineural or permanent conductive hearing loss.
• Syndromes associated with hearing loss or progressive or late onset hearing loss* such as neurofibromatosis, osteopetrosis, and Usher syndrome. Other frequently identified syndromes include Waardenburg, Alport, Pendred, and Jervell and Lange-Nielson.
• Neurodegenerative disorders*, such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich's ataxia and Charcot-Marie-Tooth syndrome.
• Culture positive postnatal infections associated with sensorineural hearing loss*, including confirmed bacterial and viral (especially herpes and varicella) meningitis.
• Head trauma, especially basal skull/temporal bone fracture* requiring hospitalization.
• Chemotherapy*.
  

JCIH (2007) recommends that infants who have a risk factor for late onset hearing loss should have at least one comprehensive audiologic evaluation by 24- to 30-months of age. Testing should also occur any time there is parent, caregiver or professional concern regarding communication development.

What are the speech and language milestones for babies?
Here is a link to the NIDCD Fact Sheet – Speech and Language Developmental Milestones: Click HERE