Approximately 33 babies are born every day with a significant hearing loss in the United States. Nearly 50% of newborns with hearing loss are not diagnosed until at least the second year of life. Hearing loss among newborns is 20 times more prevalent than phenlyketonuria (PKU), a condition for which all newborns are screened for. The average age that children with hearing loss are initially diagnosed ranges from 12 to 25 months. Studies have shown that when hearing loss is detected later, an important time frame for developing speech and language skills has passed. As a result, speech and language development is delayed and academic and social skills may be adversely affected. Research has confirmed that treatment has the best results when infant hearing loss is identified and intervention is begins before the child reaches six months of age. Less than 20% of the children born in the U.S. are born in hospitals that offer universal newborn hearing screening programs.
    The National Institute of Health, American Academy of Pediatrics, American Academy of Audiology, the Joint committee on Infant Hearing, and the Healthy People 2000 Report recommend that children with congenital hearing loss be identified before six months of age. A unilateral hearing loss that remains undetected will have negative consequences. Even children with a hearing loss in one ear are ten times as likely to be held back by a grade as compared to children with normal hearing in both ears. Infants identified with hearing loss may be fit with hearing amplification as young as four weeks of age. Appropriate and comprehensive early intervention helps these children develop with better language, cognitive, and social skills.

High Risk Criteria For Hearing Loss in Infants (0 to 28 days old) *Indicators associated with sensorineural and/or conductive hearing loss in infants

Family history of hereditary childhood sensorineural hearing loss

Hyperbilirubinemia

Ototoxic medications

Bacterial meningitis

Birth weight less than 1500 grams (3.3lbs)

In utero infections (cytomegalovirus, rubella, syphilis, herpes, and toxoplasmosis)

Craniofacial anomalies (including pinna and ear canal)

Apgar scores of 0-4 at 1 minute or 0-6 at 5 minutes

Mechanical ventilation lasting 5 days or longer

Stigmata or other findings associated with a syndrome known to include a sensorineural and/or conductive hearing loss

Family history of hereditary childhood hearing loss

In utero infection

Neurofibromatosis Type II and neurodegenerative disorders

Recurrent or persistent otitis media with effusion

Anatomic deformities and other disorders that affect eustachian tube function

Neurodegenerative disorders

Prenatal damage to the cochlear may be due to the partial or lack of cochlear development (inner ear), viral or parasitical invasion, spontaneous malformations or inherited syndromes. The most common syndromes are Usher's syndrome and Pendred's syndrome. Usher's syndrome results in a hearing loss that ranges from moderate to profound and a degenerative visual loss. Any child with a profound hearing loss should be evaluated for eye disorders. Pendred's syndrome is a recessive endocrine-metabolic disorder characterized by goiter formation and results in a moderate to profound sensorineural hearing loss that is usually progressive in nature.

Congenital Rubella

Toxoplasmosis

AIDS

Herpes I and II

Cytomegalovirus (CMV)

Congenital Rubella

Congenital hearing loss may be also diagnosed with the label "unknown".

RH-Incompatibility

Congenital Syphilis

Anoxia or asphyxia at birth

Persistent Fetal Circulation (pulmonary hypertension)

Low Birth Weight

High Forceps Delivery

Violent Uterine Contractions

Most children with hearing loss can hear some speech, however, the speech tends to lack clarity since the hearing loss occurs in the higher frequencies that contain most of the consonant and vowel sounds necessary for understanding speech. However, since the child is able to respond to the lower frequencies, he or she will usually be identified later on. As a result of late identification, speech and language skills will have significant delays. Early identification is necessary before sixth months of age in order to catch that specific time frame in which optimal speech and language development occurs. Formal audiological evaluations are needed to rule out less severe but equally disabling degrees of hearing loss.

The goal of Universal Newborn Screenings is to screen the hearing of every infant born before discharge from the hospital. Infants identified with a hearing loss will be referred to appropriate services so early intervention will occur.

PROTOCOLS FOR SCREENING AND REFERRALS: *(IN NORTH DAKOTA)