Late-Onset Hearing Loss Awareness

Late-Onset Hearing Loss Awareness

Author: Diana Hanson, MS CCC-A

Late Onset Hearing Loss Awareness Week was established in May 2021 to shine a spotlight on a population of young children who are often undiagnosed until later in childhood. In 2022, we celebrate LOHL Awareness Week from May 4-10. Audiologist Diana Hanson answers our questions.

How is late-onset hearing loss (LOHL) defined/classified?
LOHL is defined as hearing loss that follows a passed newborn hearing screening up until age 5-6 years. 

What are some of the consequences/impacts associated with LOHL?
Some of the consequences associated with LOHL are speech delay, language delay, and a delay in receiving appropriate amplification and intervention services. Additionally, kids can withdraw from interacting in conversation and have behavior issues. 


How common is LOHL in children with CMV?
LOHL is very common.  15-20% of all hearing loss in children is caused by CMV.  33-50% are a late-onset hearing loss. Two-thirds of children with hearing loss at 6 years of age are not identified by 1 year of age. 

What are some signs of LOHL that parents should look for?
Some signs to look for are a delay in speech and developmental milestones, lack of progress in speech development, and an overall less responsiveness to sound. 

What steps can be taken to reduce LOHL? Steps can be taken to mitigate the impact of LOHL?
The PCP or infectious disease physician can help to guide parents on whether medications are effective in ensuring that hearing loss will not develop later.  To mitigate the effect of LOHL a parent will want to follow up with diagnostics and the recommended devices or therapy needed.  

How can audiologists and other professionals work together in LOHL?
Most importantly, parents, physicians, audiologists, and early interventionists should work together to monitor these children aggressively in the first several years of life.  If children with CMV pass their initial hearing screenings, from 3 months to 18 months, they should be retested by an audiologist, every 2 months.  From 18 months to 6 years they should be seen every 3-6 months if they initially passed.  If there are any abnormal test results, further diagnostics should be completed to identify the specifics of that hearing loss.  Any child with CMV should have early intervention services, even if their hearing is normal to monitor developmental milestones. 

How can parents meet the needs of their LOHL children?
Parents need to be proactive with their PCP and ask for this testing to be routinely completed.  If they are concerned about a change in hearing, they need to let their PCP know about it.  Don't be dismissed, follow up. 

Where can families find more info regarding LOHL treatment, therapy, and those specifically working with CMV kids and LOHL?
Any pediatric audiologist and SLP or pediatrician should be well-versed in CMV and its impact on children and families. 

Do you have a LOHL success story you could share?
A patient of mine was born in 2014 and initially passed her hearing screenings, but later did not and was referred to me. She was confirmed to have CMV early on and ultimately had many developmental and health issues. Her first ABR showed moderate hearing loss. She had middle ear issues at the same time and was fit with hearing aids at age 5 months. She had a repeat ABR at age 6 months and it was normal, so she stopped using her hearing aids. She on and off passed OAE testing at times. At 9 months, she had another ABR which showed hearing loss again and went back to using her hearing aids. At one year, she had a more severe hearing loss and each time her hearing aids were reprogrammed. A year later her parents reported adverse reactions to her hearing aids and another ABR showed mild hearing loss. For several years, her testing in the sound booth showed results similar to her last ABR. Finally, in 2019, testing in the booth showed a possible change in hearing. She had an ABR that showed a profound loss, bilaterally and received her first cochlear implant in September of 2019 (5 years of age).  In December of 2019, she got her 2nd CI for the other ear. This illustrates how patients can have fluctuating hearing loss and lose all of their hearing 5 years later. It also emphasizes the need to keep retesting. This patient is non-verbal, so she was not able to tell us about her hearing. This can make diagnosing hearing loss even more of a challenge. 

Is there a list of resources you would like to share in the blog?
I am sharing my favorite article. I think it is easy to read and good for parents. Download now.


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About the Author

Diana Hanson received her Bachelor of Science degree from the University of Iowa and her Masters in Audiology from the University of Wisconsin-Madison. She received her Certificate of Clinical Competence in Audiology from the American Speech-Language-Hearing Association. Diana has worked as an audiologist since 1990, and specializes in working with pediatric patients for hearing aid and cochlear implant services. She also has extensive experience in assessment using Auditory Brainstem Evoked Response (ABR) testing. She has previously served on the State of Iowa Licensure Board for Speech Pathology and Audiology, and currently serves on the Early Hearing Detection and Intervention (EHDI) board. Diana is a member of the American Speech-Language-Hearing Association and the Iowa Speech-Language Hearing Association. She also has a hearing aid specialists license. When not in the office, she enjoys reading, golfing and spending time with her family.
 
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References
 
1. Pass RF, Fowler KB, Boppana S. Clinical importance of cytomegalovirus infection: an overview. In: Excerpta Medica International Congress Series, Progress in Cytomegalovirus Research 1991:3–10.
 
2. Fowler KB, Dahle AJ, Boppana SB, Pass RF. Newborn hearing screening: will children with hearing loss due to congenital cytomegalovirus infection be missed? J Pediatr 1999; 135:60–4.
 
3. Dollard SC, Grosse SD, Ross DS. New estimates of the prevalence of neurological and sensory sequelae and mortality associated with congenital cytomegalovirus infection. Rev Med Virol 2007; 17:355–63.
 
4. Fowler KB, Boppana SB. Congenital cytomegalovirus (CMV) infection and hearing deficit. J Clin Virol 2006; 35:226–31.
 
5. Morton CC, Nance WE. Newborn hearing screening—a silent revolution. N Engl J Med 2006; 354:2151–64.
 
6. Fowler KB, Stagno S, Pass RF, Britt WJ, Boll TJ, Alford CA. The outcome of congenital cytomegalovirus infection in relation to maternal antibody status. N Engl J Med 1992; 326:663–67.
 
7. Foulon I, Naessens A, Foulon W, Casteels A, Gordts F. A 10-year prospective study of sensorineural hearing loss in children with congenital cytomegalovirus infection. J Pediatr 2008; 153:84–8.
 
8. Yamamoto AY, Mussi-Pinhata MM, Isaac M de L, et al. Congenital cytomegalovirus infection as a cause of sensorineural hearing loss in a highly immune population. Pediatr Infect Dis J 2011; 30:1043–6.
 
9. Ross SA, Fowler KB, Ashrith G, et al. Hearing loss in children with congenital CMV infection born to mothers with preexisting immunity. J Pediatr 2006; 148:332–6.
 
10. Dahle AJ, Fowler KB, Wright JR, Boppana SB, Britt WJ, Pass RF. Longitudinal investigation of hearing disorders in children with congenital cytomegalovirus. J Am Acad Audiol 2000; 11:283–90.
 
11. Fowler KB, McCollister FP, Dahle AJ, Boppana S, Britt WJ, Pass RF. Progressive and fluctuating sensorineural hearing loss in children with asymptomatic congenital cytomegalovirus infection. J Pediatr 1997; 130:624–30.
 
12. Fowler KB, Tolan RW, Ahmed A, et al. Interim findings of the importance of congenital cytomegalovirus infection in the etiology of childhood hearing loss. In: Abstract Proceedings of the 13th International CMV/BetaHerpesvirus Workshop, Nuremberg, Germany, 14–17 May 2011.
 
13. Joint Committee on Infant Hearing. Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics 2007; 120:898–921.