Early intervention is seen as the gold standard for children with hearing loss whose families wish for them to develop spoken language. In ideal situations, intervention should begin as soon as possible after diagnosis. The US Centers for Disease Control has issued 1-3-6 guidelines, recommending hearing loss screenings before 1 month of age, evaluations before 3 months of age, and intervention before 6 months of age.[1]

Age at implantation is a crucial predictor of benefit from cochlear implants,[2] and it is one of the main factors that affects communication outcomes in pediatric cochlear implant patients. There is clear evidence that the best outcomes are found in children who were diagnosed early, fitted with appropriate state-of-the-art technology, and then received good quality intervention based around the development of spoken language.[3]

Despite clear evidence and expert recommendations for early intervention, a substantial number of children still experience late confirmation and intervention, especially infants that present with developmental or medical issues as well as middle ear disorders.[4]

Additionally, the prevalence of late-onset hearing loss sometimes due to congenital infection from cytomegalovirus (cCMV) or genetic disorders,[5] and children missing newborn hearing screenings have led some experts and the World Health Organization to advocate for pre-school hearing screenings for comprehensive detection of hearing loss in early childhood.[6][7]

The Effects of Late Treatment of Hearing Loss in Children

Research has shown that children who do not receive timely diagnosis and hearing loss treatment may experience:

• Delayed speech and language development compared to those who were implanted earlier [8]
• Delayed social development and weaker academic performance [9]
• Limited concentration spans and behavior that is sometimes difficult to manage [10]
• Widespread degeneration in the central auditory system if profound SNHL remains untreated due to prolonged auditory deprivation [3, 11, 12]

For more on the effects of late diagnosis and treatment, Section 1.4 of the World Health Organization’s World Report on Hearing summarizes the impact of unaddressed hearing loss.[7]

Special Characteristics of Children Who Receive Cochlear Implants Late

When beginning rehabilitation after cochlear implantation, children who receive hearing loss treatment later tend to exhibit some characteristics that can be advantageous when developing their individualized rehabilitation program. This is because late-implanted children have typically gained more experience at social events and practicing other skills than their peers who are younger and were implanted earlier.

For instance, they have had the chance to attend many birthday parties, celebrations, and excursions. As a result, their non-verbal cognitive skills may be more developed than those of younger children treated earlier. In addition, their motor skills may be more advanced, so they can be involved in activities such as cutting and drawing. They may have developed some skills in literacy, such as recognizing or writing letters.

Steps to Support Pediatric Patients in Late Cochlear Implantation

Just like children implanted earlier, those implanted later need to go step by step through the stages of auditory development. Remember that those stages may be slower than those of children implanted earlier. To track overall development, MED-EL’s A Child’s Journey Developmental Milestones (Birth – 6 Years) may be useful.

Additional Considerations for Late Pediatric Recipients of Cochlear Implants

Lack of auditory experience in children who are implanted late means that the team responsible for fitting needs to be mindful of certain factors. One is that the child may be overwhelmed by the new sensations of sound. Second, it is crucial, however, that the child wears their processor all waking hours as soon as possible.

While we want to ensure that the child is comfortable wearing the device, we also want to ensure that as soon as possible, they have good access to sound. The aim is for the child to enjoy the full range of sounds that can be provided by the implant system within the first three months following implantation.

Children Benefit Even When Receiving a Cochlear Implant Late

While age at implantation is a significant factor in the potential benefit following cochlear implantation, children who receive a cochlear implant late can still experience an improved quality of life. An intensive, step-by-step approach to rehabilitation that addresses both listening and spoken language goals leads to the optimal success for each individual.

References

1. US Centers for Disease Control and Prevention. (2024, May 15). EHDI 1-3-6 Benchmarks. https://www.cdc.gov/hearing-loss-children/articles/baby-hearing-screening-infographic.html
2. Sharma, S. D., Cushing, S. L., Papsin, B. C., & Gordon, K. A. (2020). Hearing and speech benefits of cochlear implantation in children: A review of the literature. International Journal of Pediatric Otorhinolaryngology, 133, 109984. https://doi.org/10.1016/j.ijporl.2020.109984
3. Ching, T. Y. C., Dillon, H., Leigh, G., & Cupples, L. (2018). Learning from the Longitudinal Outcomes of Children with Hearing Impairment (LOCHI) study: summary of 5-year findings and implications. International Journal of Audiology, 57, S105–S111. https://doi.org/10.1080/14992027.2017.1385865
4. Fitzpatrick, E. M., Santos, J. C. dos, Grandpierre, V., & Whittingham, J. (2017). Exploring reasons for late identification of children with early-onset hearing loss. International Journal of Pediatric Otorhinolaryngology, 100, 160–167. https://doi.org/10.1016/j.ijporl.2017.06.039
5. Lieu, J. E. C., Kenna, M., Anne, S., & Davidson, L. (2020). Hearing Loss in Children. JAMA, 324(21), 2195–2205. https://doi.org/10.1001/jama.2020.17647
6. Mackey, A. R., Persson, A., & Uhlén, I. (2024). Pre-school hearing screening is necessary to detect childhood hearing loss after the newborn period: a study exploring risk factors, additional disabilities, and referral pathways. International Journal of Audiology, ahead-of-print(ahead-of-print), 1–9. https://doi.org/10.1080/14992027.2024.2368571
7. World Health Organization. (2021, March 3). World Report on Hearing. https://www.who.int/publications/i/item/9789240020481
8. Geers, A. E., Nicholas, J., Tobey, E., & Davidson, L. (2016). Persistent Language Delay Versus Late Language Emergence in Children With Early Cochlear Implantation. Journal of Speech, Language, and Hearing Research, 59(1), 155–170. https://doi.org/10.1044/2015_jslhr-h-14-0173
9. Choo, O.-S., Kim, H., Kim, Y.-J., Roh, J., Jang, J. H., Park, H. Y., & Choung, Y.-H. (2021). Effect of Age at Cochlear Implantation in Educational Placement and Peer Relationships. Ear and Hearing, 42(4), 1054–1061. https://doi.org/10.1097/aud.0000000000001000
10. Hentges, R. F., Devereux, C., Graham, S. A., & Madigan, S. (2021). Child Language Difficulties and Internalizing and Externalizing Symptoms: A Meta‐Analysis. Child Development, 92(4), e691–e715. https://doi.org/10.1111/cdev.13540
11. Sharma, A., Gilley, P. M., Dorman, M. F., & Baldwin, R. (2007). Deprivation-induced cortical reorganization in children with cochlear implants. International Journal of Audiology, 46(9), 494–499. https://doi.org/10.1080/14992020701524836
12. Sharma, A., & Mitchell, T. (2013). Deafness. Springer Handbook of Auditory Research, 189–215. https://doi.org/10.1007/2506_2013_7