Key points

Demographics of pediatric hearing loss

Historically, children with hearing loss have fallen well behind their peers with normal hearing in the areas of speech and language development, thus limiting their abilities to participate fully in social, educational, and vocational opportunities. However, advances in both the early detection and the identification of hearing loss as well as the technology to improve speech processing through hearing aids, cochlear implants, and other hearing technology, have provided children with hearing loss unprecedented access to listening and spoken language. In turn, outcome measures for children with hearing loss in the areas of language and literacy have improved significantly in the past 2 decades.

According to the most recent Centers for Disease Control and Prevention (CDC) Early Hearing Detection and Intervention (EHDI) Hearing Screening and Follow-Up Survey, the prevalence of congenital hearing loss is 1.6 per 1000 newborns screened in the United States. Each year, 12,000 children in the United States are born with some degree of hearing loss, making it one of the most common birth defects. For some children, like the ones included in the CDC survey, hearing loss can be detected soon after birth; other children are older before hearing loss occurs or becomes evident. Hearing loss, regardless of its severity, can interfere with the development of speech and language as well as impede academic success if left undetected or untreated. However, when hearing loss is detected early and children are quickly enrolled in appropriate intervention services, most have the ability to progress at age-appropriate rates, to be mainstreamed into regular education classrooms, and to achieve significantly better outcomes than children who receive intervention later.

Demographics of pediatric hearing loss

Historically, children with hearing loss have fallen well behind their peers with normal hearing in the areas of speech and language development, thus limiting their abilities to participate fully in social, educational, and vocational opportunities. However, advances in both the early detection and the identification of hearing loss as well as the technology to improve speech processing through hearing aids, cochlear implants, and other hearing technology, have provided children with hearing loss unprecedented access to listening and spoken language. In turn, outcome measures for children with hearing loss in the areas of language and literacy have improved significantly in the past 2 decades.

According to the most recent Centers for Disease Control and Prevention (CDC) Early Hearing Detection and Intervention (EHDI) Hearing Screening and Follow-Up Survey, the prevalence of congenital hearing loss is 1.6 per 1000 newborns screened in the United States. Each year, 12,000 children in the United States are born with some degree of hearing loss, making it one of the most common birth defects. For some children, like the ones included in the CDC survey, hearing loss can be detected soon after birth; other children are older before hearing loss occurs or becomes evident. Hearing loss, regardless of its severity, can interfere with the development of speech and language as well as impede academic success if left undetected or untreated. However, when hearing loss is detected early and children are quickly enrolled in appropriate intervention services, most have the ability to progress at age-appropriate rates, to be mainstreamed into regular education classrooms, and to achieve significantly better outcomes than children who receive intervention later.

Early intervention

Early identification and early intervention are unconditionally critical to the development of listening and spoken language in children with hearing loss. Until the 1990s, the average age of identification for children with permanent hearing loss was 2 ½ to 3 years of age. In the past 20 years, newborn hearing screening has become a standard of care in hospitals and birthing centers nationwide. Today, 98% of newborns in the United States are screened for hearing loss before they leave the hospital. Because of the proliferation of newborn screening programs, the average age of hearing loss identification has decreased to 2 to 3 months of age.

Despite these advances, many children with hearing loss still do not receive the follow-up care they need. In 2007, the Joint Commission on Infant Hearing (JCIH) reported that only 54% of babies who are screened subsequently receive the recommended hearing evaluation. The other 46% are “lost to the system.” Furthermore, nearly 40% of children identified with hearing loss and their families do not receive a referral to their state’s early intervention system and therefore are not aware of the services and funding available to assist with the medical needs of their child. Congress established the Part C (early intervention) program through the Individuals with Disabilities Education Act (IDEA) in 1986 in recognition of “an urgent and substantial need” to

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  Enhance the development of infants and toddlers with disabilities;

  
  
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  Reduce educational costs by minimizing the need for special education through early intervention;

  
  
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  Minimize the likelihood of institutionalization, and maximize independent living; and

  
  
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  Enhance the capacity of families to meet their child’s needs.

The Part C program was last reauthorized in 2011 and is supported by a federal grant that assists states in operating a comprehensive statewide program of early intervention services for infants and toddlers with disabilities, birth through 3 years of age, and their families.

In 2013, the JCIH updated and expanded on their 2007 position statement to further delineate guiding principles for EHDI systems. In an effort to improve the efficiency of early intervention programs, the JCIH offered a timeline for universal newborn hearing screening, diagnosis, and intervention. As it has been come to be called, the 1:3:6 rule has been adopted by most states as a standard of care for universal newborn hearing screening programs. The 1:3:6 rule states:

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  1 = All infants are screened for hearing loss before discharge from birthing/neonatal facilities, or within 1 month of birth;

  
  
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  3 = All infants referred from the screening process complete diagnostic audiological evaluation by 3 months of age; and

  
  
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  6 = All infants with diagnosed hearing loss receive appropriate interventions by 6 months of age, including amplification selection and early intervention services.

Timeliness is vital. These steps provide the greatest opportunities for the child with hearing loss to attain age-appropriate listening and spoken communication skills.

When diagnosis of a hearing loss is confirmed, the audiologist is typically the first professional to inform the parents or caregivers. Receiving the diagnosis can induce a wide range of reactions and emotions in the family. The manner in which this information is imparted is critical to the emotional state of the parents as they begin the process of achieving eventual acceptance. Unlike most parents of children with normal hearing, parents of children with hearing loss must make important decisions about how they and other individuals in their child’s life will communicate with their child. Out of necessity, these difficult decisions must occur early in their child’s life and are often made during times of uncertainty and sadness. Families must be provided with information, resources, and the opportunity to work through a range of emotions. This process allows for information about hearing loss to be better understood and processed, and for informed decisions about hearing care management and intervention to be made in a timely manner.

The JCIH states that communication-based options need to be presented to parents in a nonbiased manner, and the choice of a communication option needs to be based on the specific needs of the family and the parents’ desired communication outcomes. Honest discussions regarding the dedicated effort required from families throughout their child’s treatment program must occur. All professionals who provide services to the child need to work in a consistent and collaborative manner in order to maximize the outcomes for each child. In partnership with the family, professionals need to follow the intervention plan for auditory, speech, and language development, monitor growth, and make changes and recommendations based on the progress of the child.

Communication options

Although there are several communication methodologies used with children with hearing loss, communication options generally range on a continuum from mostly visual to mostly auditory in their means of language transmission. Some of the communication options presented to parents and families of children who are deaf or hard of hearing typically include the following: American Sign Language (ASL), Bilingual-Bicultural (Bi-Bi), Total Communication, English-Based Sign Systems, Cued Speech, and Listening and Spoken Language (Auditory-Verbal Therapy [AVT] and Auditory-Verbal Education [AVEd]). Regardless of the communication methodology chosen, all families should receive:

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  Thorough information about all communication options;

  
  
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  Unbiased information about communication options;

  
  
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  Unconditional support and appropriate resources (local, state, national) and contacts for the methodology the family chooses; and

  
  
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  Contact information for a local support group or individual parents who have offered support to newly-identified families.

ASL is a visual language with its own unique grammatical structure, morphology, and phonology and is considered the language of Deaf Culture. The Bi-Bi approach emphasizes the use of ASL as the child’s first language and is used in academic settings with English taught as a second language through reading and writing. Traditionally, the use of hearing technology is discouraged in these approaches. Both ASL and Bi-Bi focus on integrating the child with hearing loss into the Deaf Culture through a common language.

Other approaches combine auditory- and visually-based communication. Total Communication is not a communication mode as such, but rather an educational philosophy often promoted for children with hearing loss. Its goal is to provide the most appropriate mode of communication for the child’s needs at any one time. Children, parents/caregivers, and teachers may use any form of communication, or any combination of communication strategies, to meet the child’s communicative and academic needs. English-based sign systems use signs with English grammar and morphologic markers. There are several English-based sign systems, including Signed Exact English, Signed English, Conceptually Accurate Signed English, and Pidgin Signed English. Cued Speech is an approach that uses the movement of hand shapes placed near the mouth to represent the sounds of speech for the purpose of speech-reading, but is not a stand-alone language system.

The Listening and Spoken Language Approaches, namely AVT and AVEd, focus on the development of spoken language through the use of hearing technologies and the engagement of parents as their child’s primary language facilitator in the acquisition of spoken communication. AVT, in particular, targets the parents as the main consumer of the approach and provides them with coaching and training to enhance their child’s development of spoken conversations primarily though listening.

In the past 25 years, there has been a movement toward listening and spoken language approaches for children with severe to profound hearing loss. Likely because approximately 95% of parents of children with hearing loss have normal hearing themselves, trends indicate that many parents are choosing spoken language as the primary mode of communication for their children more than 85% of the time ; this is especially true when they realize that spoken language is a viable outcome for their child. Furthermore, these parents typically select listening and spoken language approaches without initiating visual communication systems. Although some localities struggle with providing appropriate early intervention services to children with hearing loss and their families, important movements are emerging and a range of communication methodologies and early intervention services are becoming more widely available.

Numerous studies have shown that when children with hearing loss are identified early, use appropriately programmed hearing technology such as digital hearing aids, cochlear implants, and frequency modulation systems from the beginning in conjunction with early auditory-based intervention, they can achieve communication outcomes comparable to their same-age peers with typical hearing and develop positive speech, language, and social-emotional outcomes. More importantly, with early identification and appropriate intervention, these children have the opportunity to enter kindergarten or first grade with age-appropriate language and have been shown to achieve rates of literacy comparable to their peers who have normal hearing. Communication modes have been shown to have a high statistically significant association with speech and language outcomes of children with cochlear implants. Children exposed to spoken language have a greater probability of scoring higher on speech and language assessments than children exposed to some degree of either sign support or sign language. Children who receive auditory-based intervention score high on speech production and speech recognition measures, and these results improve as the emphasis on audition increases.

Improved hearing acuity, as provided by hearing technology, does not, by itself, guarantee the ability to discriminate between sounds nor the ability to develop spoken language. Children with hearing loss require intensive auditory, speech, and language training from professionals who have experience working with children with hearing loss and who possess the specialized training and knowledge to do so. In addition, parents and caregivers must be active participants in the child’s intervention/rehabilitation. Children’s first and most important teacher is their primary caregiver, most often their parents. Therefore, parent training is essential for the linguistic success of the child. Children cannot develop a functional communication system, if the only intervention they receive is in a therapy room. Specific speech, language, and auditory goals should be incorporated daily in the child’s home environment.

As discussed previously, children with communication or learning challenges and their families need access to appropriate family-centered early intervention services that are delivered by professionals who are well-trained and experienced in the use of current evidence-based practices; this is particularly true for children with hearing loss. Unfortunately, a lack of qualified practitioners, especially in remote and rural communities, in addition to limited funding, can affect the quality of services that some children receive. The program outlined in Part C of the IDEA of 1997 (PL 105-17) requires the implementation of family-centered intervention in a natural learning environment. Listening and Spoken Language Approaches have always embraced family-centered early intervention as a service delivery model. For children and their families who qualify under this legislation, early intervention services are designed to enhance the quality of their lives by facilitating the parent’s capacity to promote the development of skills in their infants and toddlers. That is, during family-centered intervention, the professional focuses on enhancing the parent’s ability, through coaching, to promote the growth and development of their infant or toddler during daily learning opportunities. When parents follow their children’s lead by supporting their interests and participation, there is a positive effect on the child’s development and learning. Families are viewed as having existing capabilities, the ability to make informed decisions, the power to act on their decisions to strengthen family competence and improve family functioning, and the capacity to become increasingly competent. These principles serve as the foundation for Auditory-Verbal Practice.

Intervention and assessment

Children with normal hearing thresholds appear to acquire language almost effortlessly by listening to the spoken language that surrounds them daily and by interacting with their environment. Borden and colleagues ^(p2) state: “They (children) are natural language learners, and they develop language by hearing the speech of others. Speech is audible. It can be described in terms of its loudness, its pitch, and its duration. It is meaningful sound strung out in time.” For children who have hearing loss, much of the acoustic aspects of speech remain unavailable without appropriate hearing aid amplification or cochlear implantation. These devices can improve access to this information, but are not singly responsible for speech and language development.

Numerous variables have been shown to influence the levels of performance outcomes observed in children with hearing loss. These variables include early diagnosis of the hearing loss, proper audiologic management, familial support, and appropriate aural (re)habilitation. The age of onset of deafness, age of implantation, duration of deafness, chosen communication mode, and duration of implant use also seem to be influential. Furthermore, the wear time of hearing technology, the cause of the child’s hearing loss, the child’s innate cognitive and linguistic abilities, parental motivation and dedication, and quality of therapeutic intervention also seem to influence the ultimate linguistic competence of these children. With appropriate medical, therapeutic, and educational management, many children who have hearing loss have the potential to develop language commensurate with their age-matched peers with normal hearing thresholds. It is clear that achievements of children are guided by both opportunity and ability. Although baseline assessments are completed when services are initiated and periodically thereafter, the professional’s mind-set should view each session as diagnostic in nature—an opportunity to observe the child’s performance and collect data on the child’s and family’s progress. With a steadfast commitment to meeting the child’s learning needs and assessing when he has the potential to master the content, professionals can ensure that each intervention session is designed to meet the child’s skill acquisition for listening, speech, language, cognition, and conversational competence. Similarly, the practitioner should closely monitor the parents’ or caregivers’ ability to facilitate language and communication and feel comfortable providing the necessary modeling and coaching to finely tune parent-child interactions to maximize learning.

Some children meet developmental milestones in a predictable and timely manner, while others may meet some milestones early but then slow down or stop making progress. Other children may make slow but steady gains, and some may make limited progress. Each child with hearing loss presents with individual needs and challenges and follows a unique trajectory in this process. Conservative estimates suggest that approximately 43% of these children present with additional challenges, such as low vision (4.4%), legal blindness (1.2%), developmental delay (8.5%), learning disability (12.4%), orthopedic impairment (4.3%), attention deficit disorder (7.2%), traumatic brain injury (0.3%), intellectual disability (7.7%), emotional disturbance (3.2%), autism (1.9%), Usher syndrome (0.1%), other health impairments (4.1%), and other conditions (8.0%).

By observing the child’s behavior during each session and documenting progress on session objectives, the clinician is able to judge if appropriate progress is being made. If expected progress is not occurring, the diagnostic nature of intervention requires a careful review of the factors that may be impeding the child’s progress. Some of these factors can be managed fairly easily, while others may require collaboration with other professionals. Once these issues are identified, steps should be taken to reduce their impact on the child’s progress.

Although outcomes will differ between children, all therapy should target auditory skills using normal sequences of development and should seek to attain skills at an expected rate. As such, it is necessary for the clinician to have knowledge of the hierarchy of typical development. Concerns about any aspect of the child’s development need to be addressed openly and honestly with the parents. Children with additional challenges may require therapy interventions to supplement weekly auditory-verbal sessions or a referral to another therapeutic approach. This process needs to be implemented as quickly as possible to take advantage of the child’s critical listening and language-learning years.

Audiologic management

Children need to have complete and consistent access to sound in order to develop listening, speech, and language skills at an acceptable rate. As mentioned previously, an essential precursor to speech and language development is adequate speech perception and the quality of early auditory input; this is especially important for children with significant hearing loss. The deficit in early access to the speech signal can be devastating to the language acquisition process.

Attendance at regular appointments, as prescribed by the child’s audiologist, is critical to evaluate the functioning of the child’s hearing technology and assess progression in speech perception abilities. These appointments are necessary for the optimal functioning of the devices. In addition, parents must maintain extra parts and batteries and become skilled at troubleshooting, in case the hearing aid or cochlear implant becomes unworkable at home.

The Alexander Graham Bell Association (AGBA) for the Deaf and Hard of Hearing has developed a Professional Practice Protocol (2012) for guidance in the audiologic management of children with hearing loss. In addition to the 1:3:6 rule that is explained earlier in this article, this protocol incorporates recommendations for follow-up well after the first 6 months. The following is included as part of the comprehensive protocol:

When hearing loss is diagnosed, routine evaluation should occur ideally at four-to six-week intervals until full audiograms are obtained, and at three-month intervals through age 3 years. Assessment at six-month intervals from age 4 years is appropriate if progress is satisfactory and if there are no concerns about changes in hearing. Immediate evaluation should be undertaken if parent or caretaker concern is expressed or if behavioral observation by parent, therapist or teacher suggests a change in hearing or device function. More frequent evaluation is appropriate when middle ear disease is chronic or recurrent, or when risk factors for progressive hearing loss are present.

One of the goals of thorough audiological management is to assess whether the child’s current hearing technology is providing adequate access to sound. If a child is in a listening and spoken language approach, this decision must be made expeditiously. Therefore, communication and collaboration between the child’s speech-language pathologist and audiologist are essential. Once it is determined that a child is not getting adequate sound access through his or her current hearing technology, the family should be counseled regarding the potential need for cochlear implantation. According to the US Food and Drug Administration (FDA), as of December 2012, approximately 324,200 people worldwide have received implants. In the United States, roughly 58,000 adults and 38,000 children have received the devices. Since 2000, cochlear implants have been FDA-approved for use in eligible children beginning at 12 months of age. For young children who are deaf or severely hard of hearing, implantation while young exposes them to sounds during an optimal period to develop speech and language skills. Growing evidence has shown that when these children receive a cochlear implant before 18 months of age and participate in an appropriate rehabilitation program, their listening skills, linguistic abilities, and clarity of speech surpass their peers who receive implants when they are older. Studies have also shown that children who receive a cochlear implant at a young age can develop communication skills at a rate comparable to children with normal hearing and can be successful in mainstream education settings. The decision to receive a cochlear implant, like all others, is one that the family of the child needs to make based on the most accurate information available and their goals for their child.