Key points
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Management of children with hearing loss requires collaborative, interprofessional teams focused on patient-centered goals and shared problem solving.
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Referrals to otolaryngologists, audiologists, and speech-language pathologists with expertise working with children should be made for diagnosis, treatment, and management of hearing loss.
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The hearing of every child should receive ongoing monitoring throughout childhood to detect late-onset or progressive hearing loss.
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A variety of hearing technologies are appropriate and available for children with mild, moderate, and moderately severe sensorineural hearing losses.
Overview: nature of the problem
Childhood hearing loss impacts almost all areas of child development: speech and language, psychosocial, and psychoeducational development, among others. The earlier management is implemented, the better chance there is to ameliorate the negative impact on development. Although we currently screen the hearing of 97% of newborns in the United States, a significant percentage of infants who fail the screening do not return for follow-up testing. According to the Centers for Disease Control and Prevention, only 70% of infants with hearing loss receive appropriate diagnostic testing by 3 months of age and only 56% receive intervention before 6 months of age. Therefore, it is incumbent on pediatricians and otolaryngologists who treat these children to ensure that families understand the importance of follow-up and management to their children’s outcomes.
Effective management of childhood hearing loss requires the collaborative efforts of the child’s family and numerous professionals, including audiologists, pediatricians, otolaryngologists, educators, speech-language pathologists, and early interventionists, and others. Exposure to consistent, meaningful sounds during the first few years of life is essential for auditory neural pathways in the brain to develop, leading to spoken language and cognitive growth. Hearing technologies are now available for all types and degrees of hearing loss, and eligibility for these technologies has changed considerably over the past decade. With the rapidly evolving landscape of hearing technologies, communication among professionals becomes more important than ever before to ensure that families receive consistent and accurate counseling about their options.
Overview: nature of the problem
Childhood hearing loss impacts almost all areas of child development: speech and language, psychosocial, and psychoeducational development, among others. The earlier management is implemented, the better chance there is to ameliorate the negative impact on development. Although we currently screen the hearing of 97% of newborns in the United States, a significant percentage of infants who fail the screening do not return for follow-up testing. According to the Centers for Disease Control and Prevention, only 70% of infants with hearing loss receive appropriate diagnostic testing by 3 months of age and only 56% receive intervention before 6 months of age. Therefore, it is incumbent on pediatricians and otolaryngologists who treat these children to ensure that families understand the importance of follow-up and management to their children’s outcomes.
Effective management of childhood hearing loss requires the collaborative efforts of the child’s family and numerous professionals, including audiologists, pediatricians, otolaryngologists, educators, speech-language pathologists, and early interventionists, and others. Exposure to consistent, meaningful sounds during the first few years of life is essential for auditory neural pathways in the brain to develop, leading to spoken language and cognitive growth. Hearing technologies are now available for all types and degrees of hearing loss, and eligibility for these technologies has changed considerably over the past decade. With the rapidly evolving landscape of hearing technologies, communication among professionals becomes more important than ever before to ensure that families receive consistent and accurate counseling about their options.
Identification of childhood hearing loss
Before universal newborn hearing screening, a child born with congenital hearing loss was likely to be identified after the first 2 years of life. The evaluation and diagnosis of children was generally requested and often welcomed by the family, who suspected something was wrong based on their child’s lack of language development. For those families, the hearing loss of their child had an obvious impact on their lives, allowing them to accept the diagnosis and to see direct benefit from intervention. This might not be the case for many families of children with hearing loss today. When identified in the newborn period, parents have not yet observed any obvious indications of hearing loss. The “problem” of hearing loss is invisible to them. For these families, acceptance of a diagnosis of hearing loss might be especially difficult and will require a consistent message and reassurance from the family’s medical home. Furthermore, we know that infants born to teenage mothers and those born to mothers with less than a high school education are less likely to seek intervention services than older mothers and those with college degrees ; thus, these mothers will require additional counseling and encouragement for their infants to receive the hearing services they need.
The Joint Committee on Infant Hearing Guidelines (JCIH ) recommends newborn hearing screening that targets permanent, bilateral or unilateral hearing loss averaging 40 dB or greater. This includes those with moderate losses (41–55 dB HL), moderately severe (56–70 dB HL), severe (71–90 dB HL), and profound (91+ dB HL); however, those with minimal or mild loss will likely not be detected. There is now a large body of evidence that suggests children with mild degrees of bilateral and any degree of unilateral permanent hearing loss are at risk of psychoeducational and behavioral difficulties (eg, Refs ). Minimal or mild bilateral hearing loss can be defined as follows:
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Pure-tone average (500, 1000, 2000 Hz) between 20 and 40 dB HL, OR
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High-frequency hearing loss with pure-tone thresholds greater than 25 dB HL at 2 or more frequencies above 2000 Hz.
Fig. 1 illustrates that children with mild degrees of hearing loss are identified and fit with hearing aids later than children with greater degrees of loss. Additionally, because children with mild degrees of hearing loss can hear and respond to some sounds, there might be less parental concern for the child’s hearing behavior and reduced awareness of delayed auditory development, even if the child has been diagnosed with a mild hearing loss.
Management goals
Studies have consistently shown that early identification of and intervention for childhood hearing loss results in improved communication abilities, including language, speech perception, and speech production. Physicians and other health care providers play a significant role in this process. Although the screening and diagnosis might initially appear as the “main event” in the family’s life, the longer journey begins after the identification of hearing loss. Fig. 2 illustrates some of the steps involved in the management of children with hearing loss. Although the management team works together to provide services for the child and family, the frequency of the follow-up visits will be tailored to the individual child and family needs for each aspect of intervention. For example, a young child might receive weekly services from a speech-language pathologist during the first couple of years following identification, but those services will likely be reduced in frequency and eventually cease once the child has achieved age-appropriate speech and language skills. Alternatively, this child’s follow-up with an audiologist will continue through adulthood, likely on at least an annual basis.
Medical providers should consider the following management steps for children with newly diagnosed hearing loss:
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State Medicaid programs cover hearing screening, diagnosis, and intervention as part of the Early and Periodic Screening, Diagnostic and Treatment benefit. Children from birth to age 3 years who have been diagnosed with hearing loss receive free, early intervention services through the Individuals with Disabilities Education Act (IDEA) Part C. School-age children ages 3 to 21 years receive services through IDEA Part B. Ensure that the families of children with hearing loss are aware of their rights for free and appropriate services.
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Refer children to geneticists in cases of suspected genetic hearing loss.
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Incidence of vision impairments is higher in children with hearing loss than in the general population (22% and 14%, respectively ). Routine ophthalmologic examinations are recommended by the American Academy of Pediatrics periodicity schedule.
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Refer children to audiologists and speech-language pathologists with expertise in working with children who have hearing loss.
Communication Mode
One of the early decisions a family must make on receiving a diagnosis of hearing loss in their young child is that of communication mode. In 2005, 85% of parents chose listening and spoken language for their child with hearing loss. This is expected because approximately 97% of children with hearing loss are born to normal-hearing parents who use spoken language. However, parents might choose another option based on their experiences, family culture, and communication goals for their child. Other options, especially for those with severe hearing loss or those families who identify with deaf culture, might include some type of visual or manual communication (eg, American Sign Language, Signing Exact English), or a combination of oral and manual communication (eg, Total Communication).
Hearing Assistive Technology
Hearing aids
When a child’s family elects to pursue listening and spoken language as the primary mode of communication, the recommended course of treatment includes the fitting of hearing aids. The function of hearing aids is to provide the user with an audible broad frequency range of speech at various input levels (soft, average, and loud), and to ensure that loud inputs to the hearing aid are comfortable for the user. Fig. 3 shows a schematic of a behind-the-ear (BTE) hearing aid, the most common type of hearing aid used by children. An alternative to the BTE hearing aid is in-the-ear (ITE) technology. In this style of hearing aid, all electronics are housed in the plastic casing that fits in the canal or concha of the pinna. Because of the swallowing risk associated with the small size of ITEs, among other reasons, the American Academy of Audiology does not recommend their use for young children. For children with bilateral hearing loss, regardless of the symmetry of loss between ears, the American Academy of Audiology recommends bilateral hearing aids. Wearing 2 hearing aids provides binaural advantages over a single hearing aid for enhanced auditory localization, signal detection, and speech perception in noise.
The Pediatric Working Group recommends that audiologic appointments following hearing aid fittings be conducted every 3 months during the first 2 years of amplification use. In addition to monitoring auditory status and development, these frequent appointments are important for young children because earmolds must be replaced often because of rapid ear canal size growth during early life. Earmolds must fit snugly within the ear to avoid acoustic feedback, and a good fit is also needed for device retention. The timeline of follow-up visits for management will be adjusted based on individual factors such as whether the child has additional handicapping conditions, or is at risk for progressive hearing loss.
The management team should encourage families to aim for full-time use of hearing aids to maximize auditory experiences. Young children (birth to 3 years) and children with mild degrees of hearing loss are at risk for less device use than older children (4–7 years) and children with greater degrees of hearing loss. This might be because families see their child with mild hearing loss responding to sound when they are not wearing hearing aids and might feel inclined to discontinue use of the devices. Repeated counseling might be required to remind families that even mild hearing loss can have a negative psychosocial and psychoeducational impact on children.
Remote microphone technology
Children with hearing loss have greater difficulty understanding speech in suboptimal conditions than their normal-hearing counterparts; yet, like all children, children with hearing loss are frequently in noisy conditions, such as day care settings and classrooms. Although personal hearing aids improve audibility of speech for children with hearing loss, they also improve audibility of the interfering noise. To facilitate optimal learning and communication, the speech signal of interest should be at least 20 dB above the background noise (ie, 20-dB signal-to-noise ratio ). Remote microphone technology (eg, frequency-modulation systems) improves the signal-to-noise ratio for listeners. This technology requires a microphone to be placed close to the target speaker’s mouth (eg, teacher, therapist, parent), where the decibel level of the speech is well above that of the interfering noise. The high-quality signal from this microphone is then delivered to the listener via a wireless carrier frequency directly to a receiver (personal system) or to a loudspeaker positioned near the listener (audio distribution system). All children who wear hearing aids are candidates for remote microphone technologies. Although all young children with hearing loss can benefit from remote microphone technologies, the utility of this technology increases markedly as they enter the school environment.
Cochlear implants
Hearing aids provide significant benefit to those with mild to severe hearing loss who have good speech perception ability. However, there are some children with severe losses and poor speech perception who derive little if any benefit from hearing aids, but do not meet Food and Drug Administration candidacy criteria for cochlear implantation. Although in years past, only children with severe-to-profound hearing loss were eligible for cochlear implantation, some children with lesser degrees of hearing loss might now be eligible depending on their auditory progress with hearing aids alone. A recent review of 51 children who had more residual hearing than specified by current guidelines, found that all received speech perception benefit following implantation. These investigators called for consideration of cochlear implantation for all children with sensorineural hearing loss who use hearing aids on a full-time basis, and comply with therapy recommendations, but who are not making expected auditory and speech-language progress.
When being considered for implantation, children should maintain full-time hearing aid use, even after their child’s auditory progress has been deemed “inadequate” with hearing aids alone. Maintaining device use increases the child’s opportunity for continued auditory stimulation before implantation, which might reduce the effects of auditory deprivation. This continued hearing aid use might also help families maintain a routine of device placement and retention, facilitating a smooth transition to full-time use of a cochlear implant following activation.
Interprofessional Collaborative Approach
Advocacy by pediatricians and otolaryngologists is imperative for the success of early hearing detection and intervention programs as well as timely, ongoing monitoring and follow-up services. Pediatricians are typically the first health care providers to whom parents turn when they have concerns about their child’s hearing. By virtue of their regular contact with children throughout the first year of life, pediatricians play a central role in directing families through the diagnostic and intervention process. When caring for children with hearing loss and their families, teamwork is required to coordinate the numerous professionals involved, including audiologists, speech-language pathologists, early interventionists, pediatricians, otolaryngologists, and educators. Because a large percentage of children with hearing loss also have additional disabilities (30%–40%), the primary care physician should monitor developmental milestones and initiate referrals for specialty evaluations, as needed. Therefore, other professionals, such as geneticists, social workers, ophthalmologists, neurologists, psychologists, and developmental specialists, also could be part of the professional team. Staying focused on patient-centered goals and shared problem solving will create an effective team and result in optimum care of these families. The knowledge and skills required of providers for effective collaboration are provided in Box 1 .
