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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.
WHAT ARE SOME OF THE CAUSES OF INFANT HEARING LOSS?
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
Delayed Onset Sensorineural Hearing Loss Indicators: affects in the inner ear and possibly beyond.
Family history of hereditary childhood hearing loss
In utero infection
Neurofibromatosis Type II and neurodegenerative disorders
Conductive Hearing Loss Indicators: affects the outer and middle ear.
Recurrent or persistent otitis media with effusion
Anatomic deformities and other disorders that affect eustachian tube function
Neurodegenerative disorders
Hearing Loss due to Prenatal Factors: (Congenital Hearing Loss - the child is born with the hearing loss)
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.
Other syndromes that cause hearing loss include:
Congenital Rubella
Toxoplasmosis
AIDS
Herpes I and II
Cytomegalovirus (CMV)
Congenital Rubella
Congenital hearing loss may be also diagnosed with the label "unknown".
Disorders Causing Hearing Loss at Birth:
RH-Incompatibility
Congenital Syphilis
Anoxia or asphyxia at birth
Persistent Fetal Circulation (pulmonary hypertension)
Low Birth Weight
High Forceps Delivery
Violent Uterine Contractions
IF MY CHILD HAS A HEARING LOSS, WHAT DOES THIS MEAN?
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.
WHAT IS UNHS?
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)
Your baby should be able to do the following:
Newborn (0 to 4 months)
A newborn (0 to 4 months) usually reacts suddenly to loud sounds through movements such as widening the eyes, jumping or extending the arms and legs. Parents should look for signs of localization from their child. Localization is eye movement or turning the head towards the direction of the sound source.
3 to 6 months
A baby of 3 to 6 months of age should turn and search out a different sound. They should also be able to respond to the sound of their name. During this particular developmental time, the baby will play with sounds by cooing and babbling. The baby should be smile or stop crying when either of the parents speaks to him/her. In addition, the baby should act differently to the ways the parents talk to him/her (angry, friendly, loving).
6 to 10 months
A baby of 6 to 10 months of age should be able to seek out the sound source. When his or her name is called, the baby should look towards the speaker. In addition, the baby should respond to both soft and loud sounds. Familiar sounds such as a doorbell ringing or a dog barking should get a response from the baby. The baby should also pay attention when the parents talk to him/her.
10 to 15 months
A baby or 10 to 15 months will begin to increase his or her babbling and begin to more closely resemble speech. The baby plays with sounds and is able to put sounds together in different patterns.
15 to 18 months
A child of 15 to 18 months is able to directly localize to most sounds. In addition, the child can understand simple phrases, identify familiar objects such as body parts and follow simple directions. A child at 18 months should have an expressive vocabulary of 20 or more words and short phrases.
First Sounds, North Dakota’s Universal Newborn Hearing Screening Program
(UNHS), First Sounds
is working to provide physiological hearing screenings to over 90% of all
newborns in the state before hospital discharge and refer those identified with
a hearing loss to appropriate early intervention services. In the past months,
the First Sounds project has taken major steps towards fulfilling many of the
outlined objectives and goals stated within the Federal grant proposal. First
Sounds has identified birthing hospitals that do not have the equipment needed
to conduct UNHS. Project members are working to establish an agreement with at
least four health care facilities to serve as regional referral centers.
Referral centers will have clinical OAE equipment and clinical diagnostic ABR
systems with frequency specific and bone conduction capabilities.
A task force that is made up of members of the state Advisory Panel and other stakeholders has been convened in order to review existing systems for managing statewide screening data and other relevant issues. A task force of North Dakota audiologists has been created in order to obtain documentation and pricing information from publishers of the major software programs available. In addition, the Audiology task force is working to determine UNHS protocols for screening and referral. These protocols will be based on validated models from states where UNHS is already in place. Eight of North Dakota’s licensed audiologists have participated in a three day training seminar conducted by the renowned Dr. David Lilly who is an expert in the field of electrophysiology, specifically with ABR and OAE testing of infants and children. In addition, other well-known speakers such as Dr. Richard Seewald and Marlene Bagatto provided training on amplification and fitting procedures with infants and children. The training occurred on September 21 to the 23rd. Additional training will be provided next year for audiologists unable to attend this year’s session. First Sounds is also in the process of developing a press kit for the involved hospitals. A brochure that will provide relevant information for both medical professionals and families is in the finalizing process and will be soon available for distribution. A power point presentation has been created to provide information on infants with hearing loss and the goals of First Sounds. If you have a question about your baby's hearing or language, call First Sounds of North Dakota at 1-800-233-1737 or email ndcpd@minotstateu.edu