Children with Late Onset Hearing Loss


Increasing Awareness and Understanding


By Krystyann Krywko, Ed.D.

“Can you say juice? J-U-I-C-E. How about please? P-L-E-A-S-E.” There I was on the floor with my face mere inches from my 26-month-old son – pleading with him to at least attempt to repeat some sort of word that I might recognize.

At this point I believed my son had a speech delay. As a former early childhood educator, I had taught numerous children who didn’t talk until their third birthday. My pediatrician told me not to worry.

VV_18(5)_Krywko.1The author’s son, Henry, and his teacher Miss Jackie work on language development in school. Henry was identified with a late onset hearing loss at age 3. Credit: Krystyann Krywko   

My son had passed the universal newborn hearing screening test (UNHS) and was hitting other developmental milestones; he was simply a late talker. It wasn’t until he was two months shy of his 3rd birthday that his preschool teacher suggested we take him for a hearing test. Six weeks later my son was diagnosed with a moderate-to-severe bilateral hearing loss.

Reflecting on our experiences, I realize that I appear incredibly naïve not to connect my son’s delayed speech with the possibility of a hearing loss, but at the time I had absolutely no familiarity with the concept as I didn’t know any other families who were dealing with hearing loss. However, more importantly, our pediatrician, as a trusted professional, missed an opportunity to write out a simple referral for a hearing test.

Hearing loss affects 1.4 of every 1,000 U.S. born children every year, thereby making it the most common sensory birth difference (CDC, 2011). This number represents the children who do not pass the UNHS. However it leaves out the number of children who are diagnosed with a late-onset hearing loss. Hearing loss can occur at any time in a child’s life for a variety of reasons, and passing the UNHS at birth has absolutely no bearing on what will happen to the child’s hearing in the future. Despite the best intentions of practitioners, once a child who is born to parents with typical hearing has passed the UNHS, hearing ability is often overlooked in a child’s development.

As babies and young children learn to talk primarily through imitating the voices of adults and other caregivers in their lives, even a small degree of hearing loss has the potential to interfere with speech and language development. Some hearing losses are so mild that it can be difficult to determine whether that is really the root cause of the delay; however, hearing loss should be one of the first things to be ruled out. Parents or caregivers are usually the first to suspect that their child has a hearing loss. Their suspicions are based upon something not “quite right” with their child’s development, including limited use of speech, inconsistent responses, their child becomes frustrated when trying to communicate or a change in the child’s behavior. Parental concerns such as these need to be treated as “red flags” and should lead to a referral for a hearing test.

Although it can be difficult to determine exactly when a child loses his or her hearing, it is important to distinguish between the misdiagnosis of hearing loss and late onset hearing loss. “Misdiagnosis of hearing loss would be when the parent was told the child passed the UNHS and, in fact, did not,” says Dr. Michelle Kraskin, audiology supervisor at Weill Cornell Medical College in New York City. She goes on to explain that with a diagnosis of late onset hearing loss, the child passed the UNHS, but at some point after their discharge from the hospital, up to the time of diagnosis, there was some form of progressive hearing loss that occurred. Although children who have congenital severe-to-profound hearing loss are most likely identified earlier through UNHS, it is more difficult to ascertain mild to moderate losses. These losses are generally not detected through UNHS and are instead identified at a later age when significant gaps are noticed in the child’s speech and language development.

Due to the uncertain timeframe of when late onset hearing loss first occurs it can be difficult to ascertain what speech and language gaps exist, as each child has a different experience. “While it is inevitable that there will be some language gaps in a child diagnosed with late onset hearing, the degree of those gaps depends on numerous variables,” states Meredith Berger, head of Clarke School NYC. Therefore it is important not to be overwhelmed by statistics from online research and searches. Ms. Berger suggests that the following variables need to be taken into account when assessing communication gaps in children with late onset hearing loss:

  • The age of the child when hearing loss was diagnosed
  • The degree of hearing loss and the quality of residual hearing the child has unaided
  • If child was immersed in language rich, stimulating home environment prior to diagnosis
  • The child’s personality (whether they are engaged, curious, and active, or more shy and withdrawn)

Because many late onset hearing losses are of a mild to moderate nature it is important not to dismiss the impact that hearing loss has on a child’s development. “Minimal is often connected with insignificant,” states Dr. Carol Flexer, distinguished professor emeritus of audiology at The University of Akron, “however, a minimal hearing loss can have major implications in a classroom setting.” 

VV_18(5) Krywko.2Despite an onset hearing loss, Henry is thriving and developing listening and spoken language abilities. Credit: Krystyann Krywko 

Spoken language is such an important component of learning that even a mild hearing loss can cause a child to miss up to 25% of classroom instruction (Flexer, 1995). Therefore, it is important to have access to consistent appropriate therapies and amplification options. “Hearing is not optional,” continues Dr. Flexer, “it’s not something that is nice to have. Every child needs to have consistent access to clear speech and acoustic detail in order to succeed.” No matter what the degree of hearing loss, children need access to enriched environments, enriched conversations and enriched language both at home and at school.

While it can be difficult for parents to accept their child’s hearing loss, it is important that there is immediate access to appropriate interventions and that the child be fitted for hearing aids and/or cochlear implants, if spoken language is chosen. “The diagnosis of hearing loss is an emotional time,” cautions Ms. Berger, “but the family really should deal with the emotional issues while they continue to move forward with treatment.” The wearing of hearing aids and/or cochlear implant processors need to become a non-negotiable part of a daily routine, along with any prescribed auditory-verbal and/or speech therapy. Ongoing audiological management and support is necessary to ensure that child’s aids or implant are working and also to follow up to determine whether the hearing loss is progressive or if it is stable.

Technological advances in hearing aids and cochlear implants allow children who are hard of hearing to connect to the world in ways that they never could before. However, children who develop late onset hearing loss often fall into a grey area, as parents and health care providers fail to recognize that the child has a hearing loss. The intricate link between speech and hearing is a fundamental cornerstone of a child’s academic and social success, and ruling out hearing loss earlier is necessary for continued development. With appropriate early intervention and parental support, children with hearing loss can be mainstreamed in regular elementary and secondary education classrooms.

Etiology of Late Onset Hearing Loss

The etiology of late onset hearing loss can be difficult to determine, however it may be related to one of the categories below:
  • Hereditary (genetic) – A hereditary or genetic loss may be related to syndrome such as Ushers, Downs and Waardenburgs syndromes. Or it might be passed onto child if there is a family history of hearing loss.
  • Pathological (result of disease) –The most common childhood illness that can lead to hearing loss is congenital cytomegalovirus (CMV). However, viral and bacterial meningitis, mumps, measles and other viral infections that cause high fever can lead to hearing loss in young children. Having your child vaccinated will reduce the risk of these infections.
  • Structural – some structural irregularities  might interfere with hearing and will lead to late onset hearing loss. Irregularities include include stenosis (a narrowing of the ear canal) or Mondini dysplasia (a malformation of the cochlea).
  • Acquired – head trauma and exposure to loud noises, such as fireworks, can also lead to hearing loss; exposure to ototoxic medication (such as that used for chemotherapy) might also lead to hearing loss.
  • Idiopathic (unknown origin) – It can be difficult to pinpoint the exact cause of a child’s hearing loss. And the audiologist might label it as being of unknown origin.  
 

References


Centers for Disease Control and Prevention (CDC). (2011). Data and statistics. Retrieved July 12, 2011, from http://www.cdc.gov/ncbddd/hearingloss/data.html.

Flexer, C. (1995). Classroom management of children with minimal hearing loss. The Hearing Journal. 48(9), 54-58.