Welcome to my “Cliff Notes” for understanding all the terminology you will start to hear from ear doctors, audiologists, speech therapists, etc.
It’s like drinking from a firehose, so I thought you should enjoy a kitchen dance party first, as through technology, your deaf and hard of hearing baby can hear the world just like you and boogie woogie one day, too!
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or take bite sized info as you are ready.
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Types of Hearing Loss
Sensorineural Hearing loss (snhl)
Sensorineural hearing loss occurs when tiny hair cells within the inner ear (the cochlea) are damaged. This hearing loss is caused by damage to the inner ear (cochlea) or to the nerve pathways to the brain. Most of the time, SNHL cannot be medically or surgically corrected. This is the most common type of permanent hearing loss.
Sudden sensineural hearing loss can be caused by head trauma (like a car accident), acoustic trauma (like being too close to gun fire), viral infections, tumors and other things.
Typically people with sensineural hearing loss receive hearing aids to make up for their hearing loss. But people with profound sensorineural hearing loss can be good candidates for cochlear implants.
SNHL reduces the ability to hear faint sounds. Even when speech is loud enough to hear, it may still be unclear or sound muffled. Some possible causes of SNHL are:
Hearing loss that runs in the family (genetic or hereditary)
Aging
Drugs that are toxic to hearing
connexin 26
Connexin 26 mutations are the most common cause of congenital sensorineural hearing losses. Connexin 26 is actually a protein found in the gap junction beta 2 (GJB2) gene. This protein is needed to allow cells to communicate with each other. If there is not enough Connexin 26 protein, the potassium levels in the inner ear become too high and damage hearing.
Everyone has two copies of this gene, but if each birth parent has a flawed copy of the GJB2/Connexin 26 gene, the baby may be born with a hearing loss. In other words, this is an autosomal recessive mutation.
Populations at Risk for Connexin 26 Mutations
Connexin 26 mutations occur most often in Caucasian and Ashkenazi Jewish populations. There is a 1 in 30 carrier rate for Caucasians and 1 in 20 carrier rate for those of Ashkenazi Jewish descent. [2]
Conductive hearing loss
A reduction of sound being sent to the inner ear caused by a problem in the outer or middle ear. Conductive hearing loss is the most common type of hearing loss in children and is usually acquired. In a minority of cases, this type of hearing loss is congenital.
Hearing loss can also be a mix of both of these types, sensorineural and conductive, affecting both the inner ear and outer/middle ear. In rare cases, hearing loss can be a result of a nerve dysfunction known as auditory neuropathy or auditory dyssynchrony, in which sounds enter the inner ear normally, but the signals between the ear and brain are impaired. [3]
Hearing Tests
hearing tests for children: Abr, oae, tympanometry
None of the below test are risky or painful. Each of these tests measures a different part of the auditory system, so they all have to be taken together to know whether your child has a hearing loss, how much loss exists, and whether it is permanent or temporary (like an ear infection).
Auditory Brainstem Response (ABR): This hearing loss test involves placing electrodes on your child’s head and reading the brainwave responses to sound - similar to an EKG reading of your heart rhythm. For the most accurate results, an audiologist and pediatric ear nose throat specialist might suggest your child be sedated for the ABR to be administered. (Read here about my initial issues with this follow-up process, and how much we learned when eventually administered properly.) While the ABR does not help you explain how your child processes sound, it can help you identify their loss and what type of amplification or technology solution could be give your child the best access to sound.
Otoacoustic Emissions (OAE): An easier test, but less credible, an earplug is placed in the ear. Sounds are administered and test tones are recorded. Your child has to be quiet for this test, either awake or asleep.
Tympanometry Tests: Objective tests that are measured without your child’s participation and often require your child to be asleep
Intervention to emphasize speech and language development is necessary, so that progress can be assessed. Lack of progress means either that the hearing thresholds have changed, or that the hearing aid is not providing enough amplification. If the aids are fit to accurate hearing thresholds, but don’t provide enough information for auditory development, then the child would be considered a cochlear implant candidate. [1]
What is the difference between hearing screening tests and diagnostic tests?
A hearing screening test is when a device (such as OAE, ABR) provides sound at different pitch levels that a person with normal hearing would hear or a normal auditory system would respond to. If a baby’s responses are within a normal range, they pass the screen. The screening doesn’t give comprehensive information abut hearing - the baby might be able to hear much soften sounds, or might might just barely hear the test sound. If the responses aren’t in the test range, they can’t say whether there is a mild or profound loss, or whether it’s a middle ear problem or an inner ear problem. It just means that the baby needs a diagnostic test.
A diagnostic test is when you determine the very softest sounds a baby will respond to, at all the different pitches of interest. During the diagnostic evaluation, the pediatric audiologist will analyze and compare several tests, such as an air and bone conduction ABR, OAE, and tympanometry to determine what level and type of hearing loss the baby may have.
[1; Christine Eubanks, PhD, Audiologist - Director of Audiology; Co-Director, Cochlear Implant program at Virginia Commonwealth University Health System]
language acquisition
The process of a child learning words and being able to understand their meaning. Simply, acquiring words to build language - expressive and receptive.
I’d never given two thoughts about how easy it was for my hearing kids to “acquire language.” They just did. But with Charlotte, it tool focused effort and years to get her up to speed with her peers. Now, at 6 years old, we are working on her understanding that she will be learning synonyms for words she “already thinks she knows” and new words for the rest of her life.
She kinda just wants to have one word and use only it, but as we all know - that would create a very limited vocabulary. Thankfully she is beginning to read and that motivation to understand what the story is has now become a secondary input for her language acquisition.
Auditory Verbal Therapy / Listening and Spoken Language Therapy
Aural (re)habilitation therapy is provided for pediatric patients that follow the principles put forth by the Alexander Graham Bell Association for the Deaf and Hard of Hearing (AGBell), which is the certifying body for Listening and Spoken Language Specialists (LSLS).
Principles of LSLS Auditory-Verbal Therapy (LSLS Cert. AVT™)
(The following list of principles was taken directly from the AGBell website)
Promote early diagnosis of hearing loss in newborns, infants, toddlers, and young children, followed by immediate audiologic management and Auditory-Verbal therapy.
Recommend immediate assessment and use of appropriate, state-of-the-art hearing technology to obtain maximum benefits of auditory stimulation.
Guide and coach parents to help their child use hearing as the primary sensory modality in developing listening and spoken language.
Guide and coach parents to become the primary facilitators of their child’s listening and spoken language development through active consistent participation in individualized Auditory-Verbal therapy.
Guide and coach parents to create environments that support listening for the acquisition of spoken language throughout the child’s daily activities.
Guide and coach parents to help their child integrate listening and spoken language into all aspects of the child’s life.
Guide and coach parents to use natural developmental patterns of audition, speech, language, cognition, and communication.
Guide and coach parents to help their child self-monitor spoken language through listening.
Administer ongoing formal and informal diagnostic assessments to develop individualized Auditory-Verbal treatment plans, to monitor progress and to evaluate the effectiveness of the plans for the child and family.
Promote education in regular schools with peers who have typical hearing and with appropriate services from early childhood onwards.
*An Auditory-Verbal Practice requires all 10 principles.
The term “parents” also includes grandparents, relatives, guardians, and any caregivers who interact with the child.
Resources:
[1] Information from a print resource book the Center For Family Involvement Provides