Hearing Loss Present at Birth

Congenital – meaning present at birth – hearing loss affects babies for a variety of reasons, including low birth weight, infections and pregnancy complications. A genetic (inherited at birth) link impacts more than half of babies born with congenital hearing loss. However, 90% of children with congenital hearing loss are born to parents with normal hearing. Studies show that one out of every 1,000 children born in the United States has moderate to profound hearing loss.

At Penn State Children’s Hospital, we are committed to working with our patients and their families to determine the best treatment option for your child. Our goal is early hearing loss identification and intervention to help each child reach his or her full potential.

Symptoms, Diagnosis and Outlook

In the United States, more than 95% of children have their hearing screened shortly after birth. Almost all states have mandatory hearing screening programs for babies to ensure early identification of a potential congenital hearing issue.

If your child fails the initial hearing screening, a repeat test will be performed. It is typically done during their newborn stay in the hospital or shortly after. Your child will be seen by one of our Penn State Children’s Hospital otolaryngologists if hearing loss is identified. We will take a careful medical history and perform a physical exam, looking for the cause of the hearing loss. In many cases, the source is ear infections or chronic effusion (fluid behind eardrums). Other children have sensorineural (nerve) hearing loss, which requires further testing.

Your child’s specialist may order further testing (CT and MRI ) to identify the hearing loss cause and examine the ear and hearing nerve. Further testing may be ordered for other systems that are relevant to hearing loss; such as electrocardiogram and kidney USG. Testing options can include:

  • Computed tomography (CT) scan
  • Electrocardiogram (ECG or EKG)
  • Kidney ultrasound
  • Magnetic resonance imaging (MRI) scan

Children with congenital hearing loss may also be referred to a pediatric ophthalmologist and possibly a geneticist. Once testing is complete, your child might be fitted for hearing aids.

If your child has profound hearing loss and does not benefit from hearing aids, a cochlear implant may be an option. These are computerized devices surgically placed in the ear. Children with cochlear implants have positive outcomes and greatly improved hearing.

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Why Choose Penn State Children’s Hospital for Care

Comprehensive Cochlear Implant Program

Penn State Children’s Hospital offers a comprehensive cochlear implant program for children not benefiting from hearing aids. Cochlear implants provide an opportunity to restore your child’s functional hearing. The cochlear implant program at Penn State Children’s Hospital focuses on children with congenital or early onset deafness.

Our cochlear implant team includes:

  • Audiologists
  • Dedicated pediatric otolaryngology and otology surgeons
  • Developmental pediatrics
  • Social workers
  • Speech therapy
  • Genetic specialist

Our highly skilled cochlear implant team is here to help families address their child’s deafness during the early language years to maximize success.

Comprehensive Hearing Screening and Diagnosis

Auditory brainstem response (ABR) screening measures the response of sound from the ear to the brain’s hearing center, allowing for accurate measurement of your child’s hearing. Many causes of hearing difficulties, such as sensorineural hearing loss from meningitis, cytomegalovirus and systemic infections at birth, can be treated at birth with antibiotics. Other causes of congenital hearing loss include jaundice at birth, certain medications, head trauma and certain syndromes.

Commitment to Research

Penn State Children’s Hospital frequently participates in clinical trials for medical conditions like congenital hearing loss and treatments like cochlear implants. For more information on clinical trials at Penn State Children’s Hospital, please visit StudyFinder.

Support Groups

Support groups provide children and their families an opportunity to connect with others in similar situations. Learn more about the support groups offered at Penn State Children’s Hospital.

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