Sensorineural Hearing Loss and Cochlear Implants in Children
by Marlee Henderson and Heather Schieffler
Sensorineural Hearing Loss and Cochlear Implants in Children
How Do We Hear?
In order for us to hear, sound waves travel through the ear canal and vibrate the eardrum which passes those vibrations through small bones in the middle ear to the cochlea, a snail-shaped structure of the inner ear1. The cochlea then translates the vibrations into electrical signals that are transmitted up the auditory (hearing) nerve to the brain.
What is Sensorineural Hearing Loss?
There are two main types of hearing loss – Conductive and Sensorineural. Conductive hearing loss occurs when sound is unable to be transmitted through the ear canal to the eardrum and small bones of the middle ear2.
Sensorineural Hearing Loss (SNHL) is a hearing impairment caused by damage to the cochlea or auditory nerve3. As explained in the following video, tiny hair cells in the cochlea of the inner ear perceive sound and transmit it to the auditory nerve, which then carries the information to the brain to be processed and understood4. Click HERE to watch Hearing Loss
Causes of Sensorineural Hearing Loss
SNHL has a variety of causes and can be either congenital, meaning present during pregnancy or at the time of birth, or acquired at any time in one’s life after birth5.
● Infections during pregnancy such as rubella, toxoplasmosis, syphilis, and cytomegalovirus among others
● Genetic syndromes including (but not limited to) Alport, Waardenburg, and Usher syndrome
● Prenatal drug exposure
● Oxygen deprivation during birth
● Trauma to the head during birth
● Chronic Ear Infections
● Inflammation of the brain (encephalitis)
Levels of Hearing Loss
Hearing is evaluated by measuring the sound intensity level (loudness) that can be heard using the units decibels Hearing Level (dB HL)6. For children, the degrees of hearing loss are categorized as follows:
-10 to 15 dB HL normal hearing range
16 to 25 dB HL slight or borderline hearing loss
26 to 45 dB HL mild hearing loss
50 to 65 dB HL moderate hearing loss
70 to 90 dB HL severe hearing loss
91+ dB HL profound hearing loss6.
The following video simulates hearing at each degree of hearing loss. Make sure you do not adjust the volume on your speakers once it begins:
Click HERE for the video: “What Hearing Loss Sounds Like”
Identifying Children with Hearing Loss
Approximately 3 infants per 1,000 are born with moderate, profound, or severe hearing loss making it one of the most frequently occurring birth defects7. Hearing loss is even more common in infants admitted to intensive care units at birth7. Because hearing examination data is lacking for children 0-5 years old, newborn hearing screening data was used to estimate congenital hearing loss at a prevalence of 30,000 children under the age of six8. Undetected and/or untreated hearing loss can delay speech, language, and cognitive development and can result in significant educational costs and learning difficulties7. The National Center for Hearing Assessment and Management (NCHAM) has reported that detecting and treating hearing loss at birth for one child can saves $400,000 in special education costs by the time that child graduates from high school7. Louisiana legislation dictates that all newborn infants are required to receive a hearing screening prior to discharge from the hospital7.
The Effects of Hearing Loss on Speech
SNHL limits the capability to hear faint sounds and can also distort sounds so they are not heard correctly9. Children with profound hearing loss or deafness will have insufficient development of auditory skills including:
● Auditory speech perception – the ability to perceive and understand speech
● Speech production – the ability to produce speech
● English Language skills – the ability to use and understand reading, writing, and speaking, as well as the ability listen10.
Without the assistance of a hearing aid or cochlear implant, residual hearing, which is the small amount of hearing remaining in the presence of SNHL, is typically insufficient due to the effect of distortion on speech sounds, and the lack of acoustic cueing–the ability to differentiate sounds3. Some children with profound hearing loss are able to acquire practical communication skills by using conventional aids, but most do not10.
What are Cochlear Implants?
According to ASHA11, “a cochlear (koe-klee-er) implant is a device that provides direct electrical stimulation to the auditory nerve in the inner ear” (para.1). Implantation of a cochlear device allows sound to bypass damaged inner hair cells and directly stimulate the auditory nerve11. Cochlear implants may help children with SNHL that do not benefit from hearing aids that amplify and direct sound through the ear canal.
Who Can Get Cochlear Implants?
Cochlear implants have been approved by the U.S. Food and Drug Administration for use in children with severe to profound SNHL in both ears and who get limited benefit from hearing aids starting at 12 months of age8. Generally, a hearing loss measured at 90 dB HL or above indicates candidacy for cochlear implantation10. As of 2012, approximately 38,000 children in the United States were implanted with cochlear devices, with 324,000 children registered with implants worldwide8.
Benefits of Cochlear Implants
Research has shown that speech perception, speech production, and speech intelligibility (the “understandability” of speech) improve in children with cochlear implants as compared to their non-implanted peers who use hearing aids10. Children who receive cochlear implants between 6 to 12 months typically demonstrate significant progress in vocabulary development and receptive and expressive spoken language abilities10. In those children deafened before developing language skills (pre-lingually), continual progress in speech recognition and speech production has been shown after the cochlear implants are received10. Overall, sound detection and auditory perception improve after receiving cochlear implantation10.
U.S. Department of Health and Human Services, National Institutes of Health, National Institute on Deafness and Other Communication Disorders. (2015). How do we hear?. Retrieved from https://www.nidcd.nih.gov/health/how-do-we-hear
American Speech-Language-Hearing Association. (n.d.). Conductive hearing loss. Retrieved from http://www.asha.org/public/hearing/Conductive-Hearing-Loss/
Halliday, L. F., Tuomainen, O., & Rosen, S. (2017). Auditory processing deficits are sometimes necessary and sometimes sufficient for language difficulties in children: Evidence from mild to moderate sensorineural hearing loss. Cognition, 166 139-151. doi:10.1016/j.cognition.2017.04.014
[Siemens]. (2014, August 5). Hearing loss [Video File]. Retrieved from https://www.youtube.com/watch?v=2rhRo73F324&feature=youtu.be
American Speech-Language-Hearing Association. (n.d.). Causes of hearing loss in children. Retrieved from http://www.asha.org/public/hearing/Causes-of-Hearing-Loss-in-Children/
DeBonis, D. A., & Donohue, C. L. (2008). Survey of audiology: Fundamentals for audiologists and health professionals (2nd ed.) Boston, MA: Pearson Education, Inc
National Conference of State Legislatures. (2011). Newborn hearing screening laws. Retrieved from http://www.ncsl.org/research/health/newborn-hearing-screening-state-laws.aspx
U.S. Department of Health and Human Services, National Institutes of Health, National Institute on Deafness and Other Communication Disorders. (2005). Statistical report: Prevalence of hearing loss in u.s. children. Retrieved from https://www.nidcd.nih.gov/workshops/statistical-report-prevalence-hearing-loss-us-children/2005#sup3
Nelson, W. N. (2010). Language and literacy disorders: Infancy through adolescence. Boston, MA: Pearson.
American Academy of Audiology. (n.d.). Cochlear implants in children. Retrieved from https://www.audiology.org/publications-resources/document-library/cochlear-implants-
American-Speech Language-Hearing Association. (n.d.-b). Cochlear implants. Retrieved from http://www.asha.org/public/hearing/Cochlear-Implant/