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Early Hearing Detection and Intervention Program

Risk Factors & Genetics

Risk factors for hearing loss

When an infant passes their newborn hearing screening, it means they have normal hearing at that point in time. However, if your child has any of the below health concerns (risk factors), it is possible that they are at higher risk for developing a hearing loss later on.  Be sure to monitor your child’s developmental milestones closely and speak with their pediatrician or audiologist if you have questions about their risk factors or developmental milestones.

 

According to the National Center for Hearing Assessment and Management (NCHAM), a child may be “at risk” for hearing loss if any of the following apply:

  • Caregiver concern* regarding hearing, speech, language, or developmental delay.

  • Chemotherapy*

  • Craniofacial anomalies, including those involving the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies.

  • Culture-positive postnatal infections associated with sensorineural hearing loss*, including confirmed bacterial and viral (especially herpes viruses and varicella) meningitis.

  • Family history* of hearing loss.

  • Head trauma, especially basal skull/temporal bone fracture* requiring hospitalization.

  • In-utero infections, such as CMV*, herpes, rubella, syphilis, and toxoplasmosis.

  • Neurodegenerative disorders,* such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich ataxia and Charcot-Marie-Tooth syndrome.

  • NICU stay of five or more days, or any of the following regardless of length of stay: ECMO*, assisted ventilation, exposure to ototoxic medications (gentamycin and tobramycin) or loop diuretics (furosemide/lasix), and hyperbilirubinemia (jaundice) requiring exchange transfusion.

  • Physical findings, such as white forelock, associated with a syndrome known to include a 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.

*Risk indicators that are marked with an asterisk are of greater concern for delayed-onset hearing loss.

According to the Centers for Disease Control and Prevention (CDC), if a child is “at risk” for hearing loss, hearing should be checked every 6 months by a pediatric audiologist.  Be sure to talk to your child’s pediatrician to determine the best follow-up schedule for your child if one or more risk factor is identified.

 

The Family Plan of Care for Infants\Children who are Deaf or Hard of Hearing (Family Plan of Care) can help you keep track of any risk factors your child may have for developing hearing loss.  You can use this form to keep all hearing related medical information in one place to facilitate sharing with your pediatrician and pediatric audiologist.

 

Genetics causes of hearing loss

According to the CDC, 50% to 60% of hearing loss in children is due to genetic causes. Some of the genetic causes of childhood hearing loss, include:

  • Having family members who also have a hearing loss.
  • Having a syndrome or other conditions in addition to the hearing loss, such as Down syndrome or Usher syndrome.
  • Having gene changes or mutations.

If your child has a hearing loss, you should talk to your child’s pediatrician or audiologist about genetic testing. Genetic testing for children who are identified with a hearing loss can provide families with valuable information, such as:

  • The reason for the hearing loss.
  • Chances of having future babies with hearing loss.
  • How to best treat hearing loss.
  • If certain antibiotics should not be given.
  • If there are any other medical concerns.

Your child’s pediatrician or other health care providers can assist you in arranging to have genetic evaluation and testing done.  In Connecticut, genetic testing can be done at the following centers:

University of Connecticut                               
Division of Human Genetics
11 South Road, Suite 230
Farmington, CT 06030
Telephone: (860) 679-1440
Fax: (860) 679-6005
Yale University School of Medicine
Department of Human Genetics
333 Cedar Street, Room WWW305
New Haven, CT 06520
Telephone: (203) 785-2660
Fax: (203) 785-3404
 

To learn more about the genetics of hearing loss click on the CDC’s A Parent’s Guide to Genetics & Hearing Loss.

 

Non-genetic causes of hearing loss

The CDC reports that 25% or more of hearing loss in babies is due to outside factors such as, the child:

  • Was exposed to infection, such as cytomegalovirus, herpes, rubella, syphilis and toxoplasmosis, before birth.

  • Spent 5 days or more in a hospital neonatal intensive care unit (NICU) or had complications while in the NICU.

  • Needed a special procedure like a blood transfusion to treat bad jaundice.

  • Has head, face or ears shaped or formed in a different way than usual.

  • Has a condition like a neurological disorder that may be associated with hearing loss.

  • Had an infection around the brain and spinal cord called meningitis.

  • Received a bad injury to the head that required a hospital stay.

  • Was exposed to chemotherapy.

In some cases, the cause of childhood hearing loss is unknown.

 

Signs of hearing loss

Remember that if you have ANY concerns about your child’s speech or hearing abilities, you should talk to your child’s pediatrician.  Some of the signs of hearing loss are:

 

Signs in babies

  • Does not startle at loud noises.

  • Does not turn to the source of a sound after 6 months of age.

  • Does not say single words, such as “dada” or “mama” by 1 year of age.

  • Turns head when he or she sees you but not if you only call out his or her name. This sometimes is mistaken for not paying attention or just ignoring, but could be the result of a partial or complete hearing loss.

  • Seems to hear some sounds but not others.

Signs in children

  • Speech is delayed.

  • Speech is not clear.

  • Does not follow directions. This sometimes is mistaken for not paying attention or just ignoring, but could be the result of a partial or complete hearing loss.

  • Often says, “Huh?”

  • Turns the TV volume up too high.

For more information and resources on developmental milestones for children, visit the CDC’s website at cdc.gov/ncbdd/actearly/milestone.

 

The Connecticut Early Hearing Detection and Intervention Task Force handout How’s My Child Doing? How Hearing, Language, & Speech Are Connected is another resource for families.

 

If you are concerned about your child’s ability to hear and/or their response to sound, trust your instincts and speak with your child’s pediatrician.  Don’t wait!  The earlier children with hearing loss are identified and start getting services, the more likely they are to reach their full potential.