10. What caused my child's hearing loss?
This question represents one of the most pressing concerns expressed by parents of children newly diagnosed with hearing loss. Fortunately, advances in technology have allowed us to determine the etiology of most types of congenital hearing loss.
Almost 60 percent of congenital hearing loss cases have a primary genetic component, and a comprehensive genetic evaluation can identify the specific cause of hearing loss for about 90 percent of these patients (bit.ly/HL-congenital http://bit.ly/HL-congenital). Additionally, as many as 25 percent of congenital hearing loss cases may be attributed to congenital cytomegalovirus (CMV; N Engl J Med 2006;354:2151-2164 http://www.nejm.org/doi/full/10.1056/NEJMra050700).
For many parents, understanding the cause helps them deal with the emotional consequences of hearing loss and support their child. In our experience, it's common for parents to harbor feelings of guilt because they erroneously assumed that their own actions while the child was in utero caused the hearing loss. After learning that her baby's hearing loss resulted from a connexin 26 mutation, a mother of a child we serve admitted that she had feared her own excessive mobile phone use during pregnancy was responsible.
Furthermore, many families find it helpful to know the cause of their child's hearing loss so that they can understand the potential for the condition in future children.
Understanding the etiology of the hearing loss can be important for medical reasons as well. Certain etiologies, such as CMV and enlarged vestibular aqueduct, can be associated with progressive hearing loss. When a child is diagnosed with one of these conditions, it alerts the team to be vigilant, consistently monitoring the child for potential shifts in hearing loss.
In addition, parents can be given information that may prevent or reduce the likelihood of progressive hearing loss. For example, minor head trauma can lead to hearing loss progression in children with enlarged vestibular aqueduct. Avoidance of contact sports may reduce this risk.
Also, some forms of genetic hearing loss can be related to other medical complications that require intervention. For instance, Pendred syndrome is associated with hearing loss secondary to enlarged vestibular aqueduct, as well as the potential for goiter in adolescence. An endocrinologist can provide pharmacological treatment to prevent adolescent-onset goiter related to thyroid dysfunction.
Finally, knowing the cause of a child's hearing loss can assist families in determining the prognosis associated with different interventions. For example, children with profound hearing loss secondary to connexin 26 mutation typically do very well with cochlear implants (CIs). This diagnosis can help a family decide to pursue the technology.
9. How many hours per day should my child wear hearing aids?
This question is easy. The quick answer is that every child needs to wear her aids during all waking hours. At Hearts for Hearing, we like to say, “Eyes open; ears on.”
During the first three years of life, children typically sleep between 13 and 16 hours a day. With that in mind, hearing aid wear time should probably range from seven to eight hours a day for newborns, to nine to 11 hours a day for children 6 months to 3 years of age. Hearing healthcare providers must be proactive in helping parents establish these wear-time goals and equipping families with strategies to achieve them.
Data-logging is an excellent tool to evaluate our progress on this front. The audiologist may use data-logging to guide discussions about wear time and strategies for facilitating hearing technology use during all waking hours. This topic will be further discussed in a future Tot 10 column.
8. What can my child hear?
Based on our experience, parents ask this question most frequently. During the initial interactions with families, hearing healthcare providers should strive to explain what the child is able to hear both with and without hearing technology. Equipping families with this understanding not only satisfies their curiosity, but, more importantly, it also establishes the importance of full-time hearing technology use.
Many hearing aid analyzers allow clinicians to demonstrate the effects of hearing loss. Hearing loss simulations are often quite useful, and they are widely available on the Internet. Also, the industrious clinician may use additional programs, such as Adobe Audition, to filter speech in order to simulate the child's specific type of hearing loss.
Other great tools for demonstrating hearing loss are audiologic measures. The well-known familiar sounds audiogram may be used to show what can be heard with and without amplification, presenting a perfect opportunity to introduce the goal of obtaining aided thresholds of 25 dB HL or better so that speech is readily available to the child.
7. Does my child need other technology in addition to a hearing aid?
This question also is easy to answer. The response is a resounding “yes,” regardless of whether the child is using hearing aids or cochlear implants.
Imran Mulla, PhD, demonstrated that infants and young children routinely encounter situations where the signal-to-noise ratio is -5 dB or worse (bit.ly/Wolfe-CI http://bit.ly/Wolfe-CI). News flash here! In these situations, children do not have access to intelligible speech without the use of remote microphone technology.
Another study from the University of Western Ontario indicated that children must try to interpret speech in the presence of competing noise for at least 70 percent of their day (J Educ Audiol 2011;17:23-35 http://www.edaud.org/journal/2011/2-article-11.pdf). Again, optimal speech understanding in these situations will only occur with the use of remote microphone technology.
Remember, we know that children should be exposed to about 46 million words by the time they are 4 years old (Hart B, Risley TR: Meaningful Differences in the Everyday Experience of Young American Children; Baltimore: Paul H. Brookes Publishing, 1995). The only way children who use hearing aids or cochlear implants can reach this goal is by using remote microphone technology.
Hearing healthcare professionals should strive to make digital, adaptive remote microphone technology available to all children using hearing aids and cochlear implants. In fact, the use of remote microphone technology should be considered as essential as the child's personal hearing technology.
6. How much should my child understand when using hearing aids or cochlear implant sound processors?
It is challenging to provide an evidence-based, simple answer to this question. With improved technology, it is tempting to say that children who have hearing loss should understand 100 percent of monosyllabic words while using their hearing aids or cochlear implants.
However, limitations of the impaired peripheral auditory system and in our ability to stimulate that system effectively do not allow every patient to understand 100 percent of target words on a linguistically appropriate monosyllabic word recognition test.
At Hearts for Hearing, we strive to provide our patients with access that enables scores of at least 80 percent of target stimuli on such a test. When technology is optimized for both ears, most of our patients can achieve this goal.
If a patient can understand 80 percent of monosyllabic words without visual cues, then we can expect that person to communicate adequately when using optimal technology, contextual cues, and speechreading in most real-world situations.
5. Is my child's speech, language, and auditory skill development normal?
This may be the most important question family members can ask their child's hearing healthcare provider. Providers should verify that every child periodically receives formal, standardized assessments of speech and language, with the goal of assuring that the child is making at least one year of progress in speech and language development for every one year of chronological age.
Also, given today's technology, it is critically important to compare children who have hearing loss with children who have typical hearing, and we must strive for speech and language scores similar to those achieved by hearing children of the same age.
Likewise, hearing healthcare providers need to administer formal, standardized questionnaires to evaluate the child's auditory skill development in day-to-day situations. Marlene Bagatto, AuD, PhD, developed an excellent protocol suited to this purpose, the University of Western Ontario Pediatric Audiological Monitoring Protocol (UWO PedAMP; Trends Amplif 2011;15[1-2]:57-76 http://tia.sagepub.com/content/15/1/57.long).
Of course, a child's nonverbal IQ, neurological/cognitive abilities, and motor development should also be considered as variables that affect spoken language development.
Because 40 percent of children with hearing loss have a secondary diagnosis (Gallaudet Research Institute; bit.ly/GRI-secondary http://bit.ly/GRI-secondary), it is critically important to consider all factors when counseling families and optimizing the opportunities available for every child. From the start, hearing healthcare providers should establish these ambitious, yet realistic, goals with their families and provide a schedule for pursuing the desired outcome.
4. Does my child need a CI?
Once again, this is a question that does not always have a simple answer. Without a doubt, most children with average hearing thresholds of 80 dB HL or poorer will achieve better spoken language development with the use of a cochlear implant for at least one year than they would with bilateral hearing aids.
The question becomes more complex for children who have average thresholds at the better end of the severe hearing loss range. Recent findings from the Australian Longitudinal Outcomes for Children with Hearing Impairment (LOCHI) study showed that children with cochlear implants achieve the same speech, language, and auditory outcomes as children with hearing aids and a four-frequency pure-tone average of 66 dB HL. However, that finding does not imply that every child with hearing loss greater than 66 dB HL should receive a cochlear implant.
In reality, a child's candidacy for cochlear implantation does not hinge on audiometric criteria alone. As previously mentioned, children with hearing loss should achieve one year of speech and language growth within one calendar year. If this progress is not achieved, then the pediatric hearing healthcare team should evaluate the effectiveness of the child's hearing aid technology.
If the team determines that the child is using the best hearing technology available, the technology is optimally set for the child, the child has access to a robust model for speech and language development, and additional disabilities are not the primary reason for the delay, then a cochlear implant should be considered.
Furthermore, if probe microphone testing indicates elevated aided thresholds, unsatisfactory word recognition, and insufficient audibility even with the hearing aids programmed to optimal settings, then a cochlear implant should be considered.
3. What can I do to facilitate spoken language development?
At Hearts for Hearing, the Listening and Spoken Language Specialist (LSLS) who is certified as an Auditory–Verbal Therapist is typically the expert who answers this question. However, it is critically important for all members of the pediatric hearing healthcare team to be equipped with strategies they can recommend to facilitate spoken language development in children with hearing loss.
We often recommend that our parents become the sportscaster Bob Costas of their child's life, providing a play-by-play of every moment throughout the day.
For example, when preparing cereal for an infant, the parent might say, “Oh, you are so hungry. I think it's time to eat breakfast. Today, we are going to have rice cereal and bananas. The cereal is in the pantry. Uh-oh, Daddy moved the box. It's on the bottom shelf today. Now we need a bowl from the cabinet. Let's open the cabinet door. There it is! The bowl has a frog on it.”
Although the preceding example may seem elementary, such a play-by-play description results in a tidal wave of intelligible words, serving as a rich model for spoken language development in a child.
In addition, we encourage our parents to sing to their children as much as possible. Singing or talking in melodic tones actively engages both sides of the brain, resulting in rich stimulation of the auditory centers of the nervous system.
Further, we recommend that our parents read as many books throughout the day as possible. The simple act of reading age-appropriate books provides a robust model for speech and language development.
2. How long should my child's hearing aids last?
This is yet another tough question to answer. In many children, hearing loss is progressive, necessitating a change to more powerful hearing aids or cochlear implant technology.
From the beginning, we tell families that they should expect hearing technology to last no longer than four to five years. We also prepare them for the fact that a variety of situations may necessitate a change to different technology prior to that time.
We then encourage families to consider the cost of hearing technology when choosing an insurance plan. Hearing aid benefits vary widely from one plan to the next. Also, we encourage families to put aside money each month to cover technology that they should expect to purchase every three to five years. We attempt to give them an approximate cost of the technology so that they are able to construct their budget accordingly.
1. How do I take care of my child's hearing aids?
Before a need arises, families should be equipped with hearing technology care packs stocked with tools for keeping the technology in good working order. A listening stethoscope is absolutely imperative so that the family can conduct daily checks to ensure that the technology is working appropriately. Also, a dehumidifier is considered an essential resting spot for hearing technology when the child is sleeping.
Microphone filters, battery testers, and a variety of other accessories should also be provided to the family the day hearing technology is fitted. It is critically important that caregivers understand appropriate care, use, and maintenance of hearing technology in order to ensure the child always has access to appropriately functioning devices.
Additionally, effective strategies for hearing technology retention are at the top of the list of topics for hearing healthcare professionals to address from the very first fitting. It is safe to assume that hearing aids and cochlear implant sound processors will not securely remain on a child's ears throughout an active day, whether an infant is removing her hearing technology to use as a teething device or a toddler is turning somersaults down the hall.
All hearing healthcare providers should be equipped with effective strategies to facilitate full-time hearing technology use during all waking hours, such as the utilization of headbands, sports bands, pilot caps, and toupee tape to keep devices in place.
Also, in our experience, families have developed some very creative ways to keep technology in place. Those ideas are often shared in our support groups and networks. Encouraging the formation of such local networks is highly recommended. As the saying goes, it takes a village.Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.