The Primary Care Provider’s Guide for Children with Hearing Loss

The Primary Care Provider’s Guide for Children with Hearing Loss

Download the print version of this guide here.

When a child has been identified with a hearing loss, the parents may have many questions and there will be decisions they will need to make.  You are one of the people to whom they will turn for help.  This Guide will assist you in providing the best possible services to the family following the diagnosis of an infant with hearing loss.  Together the Guides for Families, Early Intervention Providers, Physicians and Audiologists comprise a shared plan of care to help parents navigate the first part of their journey with their child with hearing loss.

Following the Diagnosis of Hearing Loss

Understand how hearing loss may influence the child’s development:

Understand the role of the primary care provider in caring for a child with hearing loss:

Soon after the initial diagnosis of hearing loss the following should be performed:

  • Ensure the infant has been tested for possible CMV etiology.
  • A complete history that includes information about hearing loss in the family, pregnancy, infections, and the baby’s history, including type of physiologic auditory screening, gestational age, hyperbilirubinemia, severe metabolic disturbance, and ototoxic drugs.
  • A thorough physical exam, including height, weight, head circumference, birthmarks, facial asymmetry, and otomicroscopic exam of the tympanic membranes
  • Laboratory data, including results of maternal screenings for toxoplasmosis, rubella, and syphilis, as well as the baby’s newborn metabolic screening.
  • An electrocardiogram (long QT is often associated with hearing loss)

Refer the child to Early Intervention:

  • In Virginia, every child with hearing loss is eligible for Early Intervention, also known as Infant and Toddler Connection of Virginia. If the child is not enrolled, encourage the parents to do so.

Ensure that referrals are made for further testing as needed:

  • Pediatric Otolaryngologist
    • for hearing aid clearance;
    • may refer for imaging of the temporal bones (abnormal findings are noted in about 30% of all children with hearing loss, more if the hearing loss is asymmetric).
  • Ophthalmologist
    • 22% of children with sensorineural hearing loss have ophthalmic problems;
    • the rate in children who are deaf is about 50%;
    • children with non-syndromic sensorineural hearing loss have a 2- to 3-fold increased occurrence of ocular abnormalities.
  • Geneticist
    • to identify an explanation for the hearing loss;
    • to become aware of the possible risks of other family members having a hearing loss.
  • Developmental pediatrician, neurologist, cardiologist, and nephrologist as needed.

Discuss with the family:

  • Discuss insurance and financial costs with the family. Most families did not plan on the expenses that accompany having a child with hearing loss.  They may need to call their insurance company and ask about coverage options.
  • If the family is concerned about the cost of initial hearing aids, they may be eligible for free hearing aid use through the VA Hearing Aid Loan Bank (866-596-9367)

Every follow-up visit

  • Encourage the family to continue with a Pediatric Audiologist for follow-up appointments.
    • Early Hearing Detection and Intervention – Pediatric Audiology Links to Services (EHDI-PALS) is a website with a national directory of pediatric audiology facilities and helpful educational resources for families.
  • Talk about communication between parent and child – strengths as well as barriers to communication.
  • Encourage the family to connect with other families whose children are deaf or hard of hearing
    • Family-to-Family Support – VCU – Center for Family Involvement – 1-3-6 Family Educators | 877-567-1122
    • Virginia Hands and Voices of Virginia
  • Refer for case management services if needed

More Information

Critical Window

The critical window for language learning is very early.  Language develops from birth to approximately 3 years of age while brain neuroplasticity is the greatest (Sharma et al., 2002)

The Centers for Disease Control and Prevention (CDC) and the American Academy of Pediatrics (AAP) have set national standards for Early Hearing Detection and Intervention (EHDI).

1 – Screen for hearing loss before one month of age

3 – Diagnose hearing loss before three months of age

6 – Enroll in early intervention programs before six months of age

They reflect the urgency of timely diagnosis and intervention for children with hearing loss.

The goals of the Virginia Early Hearing Detection and Intervention (VA EHDI) Program are based on those standards.  VA EHDI provides tracking for children who need follow-up, support for families, and training and technical assistance for hospitals, audiologists and other providers.

Importance of Early Intervention

The early interventionist helps a child who is deaf or hard of hearing acquire language skills during the first three years of life.   The Infant and Toddler Connection is committed to providing families with the information and support they need as they make decisions for their child. The early interventionist will talk with the parents about communication possibilities for children who are deaf or hard of hearing.  Some of the communication options include:

  • American Sign Language (ASL)
  • Cued Speech
  • Listening and Spoken Language
  • Total Communication

Etiology of Hearing Loss

As children with hearing loss are identified through newborn screening programs, it is important that the cause of hearing loss and associated conditions and syndromes be identified.  Identifying the etiology may influence the care the child should receive.

Currently, research indicates that approximately 60% of children identified with hearing loss have a genetic cause and about 40% have an environmental cause. Hearing loss is one of the known characteristics in some genetic syndromes.  Some examples are Down syndrome (abnormality on a gene), Usher syndrome (autosomal recessive), Treacher Collins syndrome (autosomal dominant), Crouzon syndrome (autosomal dominant), and Alport syndrome (X-linked).

Some non-hereditary causes of early hearing loss include prenatal infections, illnesses, toxins consumed by the mother during pregnancy or other conditions occurring at birth or shortly thereafter. These conditions typically cause sensorineural hearing loss ranging from mild to profound in degree. Examples include:

  • Cytomegalovirus is a common etiology of hearing loss and is treatable if caught early (
  • Intrauterine infections including rubella (German measles) and herpes simplex virus
  • Complications associated with the Rh factor in the blood
  • Prematurity
  • Maternal diabetes
  • Toxemia during pregnancy
  • Lack of oxygen (anoxia)

For further information

Virginia’s Resource Guide for Families of Children with Hearing Loss          

Contact the Virginia AAP Chapter Champion for Early Hearing Detection and Intervention