Clinical Practice Guideline: Report of the Recommendations. Hearing Loss Assessment and Intervention for Young Children (Age 0-3 Years)

NYS Department of Health. (2007).

Albany (NY): NYS Department of Health, (Publication No. 4967), 354.

This evidence- and consensus-based guideline provides recommendations for the assessment and intervention of hearing loss for young children ages birth to three years. The guideline targets parents and professionals. Recommendations of interest to audiologists and speech-language pathologists include screening, assessment, and management of hearing and assessment of communication.

Early Intervention Program, New York State Department of Health; U.S. Department of Education

Professionals working with children with hearing loss and their families should: <ul> <li>set achievable expectations (Level C Evidence);</li> <li>provide information and other resources;</li> <li>help families evaluate the accuracy and claims of effectiveness of resources;</li> <li>support parents in decision-making process by eliciting parental observations about the child, providing feedback on progress, and working collaboratively to develop an intervention program;</li> <li>ensure the multiple components are integrated and complementary;</li> <li>consider the potential impact on the family;</li> <li>communicate regularly about the child's progress;</li> <li>continually respond to questions and needs of parents as the child develops and changes; and</li> <li>provide education and support to help families to maximize their child's development (Level D2 Evidence).</li> </ul>

Using speech threshold testing alone is not sufficient since sloping or rising hearing loss configurations may be missed (Level D1 Evidence).

"It is recommended that children diagnosed with hearing loss of any type be referred to an otolaryngologist for a medical and otologic evaluation. It is important for this evaluation to include a thorough review of the child's medical and family history; a physical examination of the ears, head, and neck; and possibly a neurotological evaluation. Additional audiologic, radiologic, and serum laboratory tests and evaluation by a medical geneticist may be requested as indicated" (Level D2 Evidence; p. 167).

Children with severe to profound hearing loss that receive early implantation (i.e., less than 3 years old) "may have better outcomes (speech recognition and intelligibility) than children who receive implants at a later age; there does not appear to be increased medical risk from early implantation compared with later implantation" (Level D1 Evidence; p. 160).

"Children who cannot be fitted with conventional hearing aids or FM systems because of bilateral structural malformations of the outer ears or complete atresia (closure) of both ear canals may benefit from amplification through the use of bone-conduction hearing instruments" (Level D2 Evidence; p. 148).

"When using an auditory approach for a child with hearing loss, it is important to provide opportunities for the child to maximize auditory potential during daily activities when the child is expected to listen and to speak and participate in programs or activities (such as preschool or playgroups) in which they can interact with children with ... [typical] hearing and when spoken language is the only language used by the children at all times" (Level D2 Evidence; p. 124).

If an initial communication assessment is conducted prior to the identification of the child’s hearing loss, a comprehensive re-assessment should be completed after the results of the audiologic assessment are known. Communication skills should be monitored and assessed periodically especially if amplification is not initially provided (Level D2 Evidence).

"It is recommended that the steps for obtaining amplification devices for a child with hearing loss include: <ul> <li>examination by an otolaryngologist (frequently termed medical clearance for the fitting of amplification);</li> <li>taking impressions of the child's outer ear and ear canal for the fabrication of earmolds;</li> <li>selecting and fitting the electroacoustic characteristics of the hearing aids (electroacoustic characteristics means the sound levels produced by the hearing aid, as a function of frequency, as measured by specific test equipment);</li> <li>evaluating (verifying) the hearing aids using real ear measurements to ensure that the electroacoustic characteristics of the amplification are well matched to the hearing loss of each ear of the child;</li> <li>monitoring for the hearing aids proper functioning; and</li> <li>validating that the child is developing functional communication and listening skills through hearing aid use" (Level D2 Evidence; p. 147).</li> </ul>

If a child with persistent otitis media with effusion is treated with ventilating tubes, it is recommended that another hearing evaluation be performed after the placement as hearing loss may still be present due to unresolved effusion or sensorineural hearing loss (Level D2 Evidence).

Tympanometry and measurements of middle ear reflexes should be used in conjunction to assess middle ear function (Level D2 Evidence).

"Cochlear implants are not recommended for children with lesser degrees of hearing loss because it is expected that children with less than a severe loss will have equivalent, if not better, results from hearing aids [and] residual hearing could be lost in the implanted ear, which may affect the use of amplification technologies and future technical developments" (Level D2 Evidence; p. 161).

Loaner amplification devices may be provided at the beginning of the amplification acquisition process until a final recommendation is made to the family regarding specific hearing aids. The availability of these devices can facilitate the initiation of hearing aid use and provide opportunities for ongoing hearing aid evaluation or experimentation with various assistive listening device technologies (Level D2 Evidence).

"The Wave V latency of the ABR is increased with conductive hearing loss as compared to sensorineural hearing loss. Using both bone-conduction ABR as well as air-conduction ABR adds even more information in helping to differentiate between conductive and sensorineural hearing loss" (Level A Evidence; p. 71).

Intervention programs for any communication approach must provide ongoing parent education and encourage a high level of family participation (by all family members and caregivers) throughout the intervention. Given this, parents must consider the factors that affect both their child and family when selecting an approach (Level C Evidence).

A family assessment should also be conducted to determine the resources, concerns, and priorities of the family (Level D2 Evidence).

"If a young child receives a cochlear implant, it is essential that the communication approach for that child incorporate hearing and spoken language" (Level D2 Evidence; p. 160). Post cochlear-implantation, the child should receive "ongoing comprehensive intervention to develop listening skills (auditory training) and speech-language therapy to maximize the benefits of the device" (Level D1 Evidence; p. 162).

Automated screening equipment often has test parameters set by the manufacturer. Therefore, different equipment may yield different screening results (Level D1 Evidence).

The effectiveness of amplification should be subjected to on-going monitoring and validation both in the test booth as well as in the infant's typical listening environments. This should include aided assessment, documenting progress in auditory skill development, as well as the parent and professional perceptions of the child's ability to hear with the amplification device (Level D2 Evidence).

Children with mild or moderate hearing loss typically use an auditory based communication approach (Level D2 Evidence).   To facilitate the development of auditory skills, treatment should incorporate a listening skills program that targets the child's speech perception skills through hearing. Parents and professionals should have an understanding of the stages of auditory skill development and know the specific techniques that can be used at each stage to facilitate listening (Level D2 Evidence).

A listening check should be performed before each speech session to determine if the amplification device is working properly (Level D2 Evidence).

It is recommended that a comprehensive assessment of hearing for infants and young children (from birth to 3 years old) include the following as components of an audiometric test battery: <ul> <li>"hearing history;</li> <li>physiologic procedures; and</li> <li>behavioral audiometry testing (using a developmentally appropriate response procedure), including measures of speech perception" (Level D2 Evidence; p. 69).</li> </ul>

Intervention should be provided by qualified professionals with specialized expertise including: <ul> <li>"knowledge of amplification and assistive technology;</li> <li>knowledge of communication development in children with hearing loss;</li> <li>skills in facilitating auditory and speech development in children with hearing loss;</li> <li>fluency in the selected communication approach; and</li> <li>knowledge of family dynamics" (Level D2 Evidence; p. 111).</li> </ul>

"If a child with usable residual hearing is enrolled in an intervention program using ASL, it is important for the parents to be strong advocates for speech-language therapy and/or listening skills therapy when the child is learning English as a second language" (Level D2 Evidence; p. 129).

A hearing evaluation for children with persistent and recurrent otitis media with effusion should not be postponed. Delayed testing due to otitis media is a reason for late identification of sensorineural loss (Level C Evidence).

For the child with hearing aids, the communication assessment should be completed with appropriate amplification. It may also be important for assessments of functional listening skills to be completed both with and without amplification and in varying degrees of background noise (Level D2 Evidence).

Standardized assessments can assist in determining if a child's communication development is delayed. However, many of the assessments may need to be adapted especially if the child's communication either partially or totally depends on visual information (e.g., signs, cues) (Level D2 Evidence).

Hearing aids selected for infants and young children should have flexible electroacoustic characteristics. The specific characteristics selected for each ear should be established through a prescriptive amplification fitting procedure. "This is a method that specifies the amount that the sound input to the hearing aid will be amplified (gain) and the maximum limit (output) that sound will be amplified in the child's ear" (Level D2 Evidence; p. 151).

"In children with severe-to-profound sensorineural hearing loss, a cochlear implant in conjunction with other interventions can enhance speech perception, enhance speech production and speech intelligibility, enhance language acquisition, augment education, [and] increase visual attention" (Level C Evidence; p. 162).

If access to a hospital screening is not possible then the infant's hearing should be screened by one month of age (Level D1 Evidence).

The majority of children with hearing loss, regardless of age, degree of hearing loss, or communication approach, can benefit from training in auditory skills" (Level D1 Evidence; p. 132). Most children with hearing loss have some residual hearing. To maximize residual hearing, the child must receive specific training targeting auditory skill development (Level D2 Evidence).

Delayed initiation of canonical babbling (after 1 month of age) is a significant indicator for hearing loss (Level C Evidence). For children up to 6 months of age, the communication assessment should focus on the achievement of communication milestones and the various types of vocalizations (e.g., cries, babbles, laughs) the child demonstrates (Level D2 Evidence).

"It is recommended that children be considered for cochlear implantation if they demonstrate little benefit from hearing aids, lack progress in the development of auditory skills and speech, and are: age 12 months to 24 months with profound hearing loss, or age 24 months or older with severe to profound hearing loss" (Level D2 Evidence; p. 159).

Following the initial hearing aid fitting, amplification should be monitored at least every two to three months and include: <ul> <li>"assessment of hearing thresholds in each ear;</li> <li>a check of the adequacy of the earmold;</li> <li>electroacoustic and real-ear measures of the amplification devices;</li> <li>confirmation that the prescriptive gain and output targets are still appropriate and are being achieved; and</li> <li>validation of the amplification fitting by confirming the functional benefit of the hearing aids for developing communication and listening skills, and determining that progress in these areas is commensurate with the child's age and cognitive abilities" (Level D2 Evidence; p. 152).</li> </ul>

Preterm infants may show signs of hypersensitivity to stimuli and therefore not habituate to the behavioral audiometry procedures in the same way as full-term babies. These infants may exhibit unusual or incorrect responses to the stimuli. Those that do should be referred for a developmental assessment (Level D2 Evidence).

Children who are candidates for a cochlear implant should also "receive sufficient experience with well-fitting amplification and enroll in a program focused on the development of listening skills (auditory training) to determine whether or not the child will benefit from amplification or other assistive technology. The trial period with amplification may vary depending on a number of factors such as: age of identification, etiology of hearing loss, amount of residual hearing, progress or lack of progress, recurrent otitis media, the amount of time the child actually wears the hearing aid" (Level D1 Evidence; p. 161).

Before parents and professionals begin formal speech training, the child should be able to imitate and do so willingly (Level D2 Evidence).

Behavioral observation audiometry (using an unconditioned response procedure) should not be the sole method of hearing assessment in infants and young children (Level D1 Evidence). The selection of amplification can be based solely on physiologic measures (i.e., frequency-specific ABR using insert earphones) if the child cannot complete a behavioral audiologic assessment due to age or developmental level. ABR findings should be confirmed via a behavioral audiologic assessment as soon as possible to determine hearing thresholds across a wider range of speech frequencies (Level D2 Evidence).

When assessing any new hearing aid technology for a child, it is important to note if: <ul> <li>"evidence exists that the new technology is beneficial for use with children;</li> <li>the electroacoustic characteristics of the hearing aid can be measured;</li> <li>the hearing aid can be fit using a prescriptive fitting procedure; [and]</li> <li>the new technology provides listening options not available on the child's current hearing aid" (Level D2 Evidence; p. 154).</li> </ul>

When selecting a communication approach, intervention program factors (e.g., availability, intensity of services, setting, teacher experience with an approach, setting) should be considered (Level D2 Evidence). As communication skills and needs change over time, the family's choice of a communication approach may change as well (Level D2 Evidence). When choosing a communication approach, parents must take into consideration the benefits and limitations of each approach. Moreover, professionals assisting parents with this process should provide unbiased information; detail the parental role in each approach, and give parents time to consider all of the information (Level D2 Evidence).

When informing the family about the diagnosis of hearing loss, it is important to address the family's initial concerns, provide parents with information, respond to the needs of the family, and determine factors that may influence the family's reaction to the diagnosis (Level D2 Evidence).

Professionals conducting communication assessments with young children with hearing loss should have: <ul> <li>appropriate knowledge and expertise with this population including the implications of hearing loss for communication development;</li> <li>the results of the audiologic assessment; and</li> <li>proficiency in the home language of the family/child (including ASL) or use a trained interpreter for the evaluation (Level D2 Evidence).</li> </ul>

"It is recommended that an amplification device be considered for infants and children with unilateral hearing loss (hearing loss in one ear and normal hearing in one ear) unless there are audiologic or medical contraindications" (p. 148). Medical contraindications include structural malformations of the outer ear or unilateral atresia. Audiologic contraindications include severe or profound unilateral hearing loss. Unilateral hearing aid use with this degree of loss can "distort sounds relative to the same sounds heard in the normally hearing ear [or] amplify noise that could be distracting or hinder the child’s listening abilities" (Level D2 Evidence; p. 149).

If a total or simultaneous communication approach is selected, parents should understand that it will take time to learn how to communicate with sign and that they must have more than a cursory knowledge of sign to fluently convey spoken English. Additionally, parents must incorporate strategies to promote visual communication, some of which include: <ul> <li>focusing on the message and using every means possible to communicate;</li> <li>learning and using signs for daily activities, feelings, and values;</li> <li>providing visual information (e.g., family photos, sign books, sign videos, pictures of how signs are formed, and visual daily schedules);</li> <li>keeping eye contact with the child when signing;</li> <li>encouraging turn-taking, pausing, and waiting;</li> <li>encouraging other family members to sign during conversational times (e.g., at the dinner table);</li> <li>arranging seating so the child with hearing loss can see who is talking and who is signing; and</li> <li>learning how to interpret the conversation of others for the child (Level D2 Evidence; p. 126).</li> </ul>

"It is recommended that all hearing aids prescribed for infants and young children have safety features, for example: tamper-resistant battery doors (because the hearing aid battery is small and therefore a choking hazard, or could be harmful if swallowed) [and] volume control cover (to prevent the child from changing the volume)" (Level D2 Evidence; p. 151).

"It is recommended that a newborn hearing screening program include: <ul> <li>an infant hearing screening with an objective physiologic test before discharge from the birthing facility;</li> <li>communication of screening results to parents;</li> <li>provision of written educational materials;</li> <li>a follow-up for repeat infant hearing screening or provision of referrals to obtain follow-up screening on an outpatient basis for infants who do not pass inpatient hearing screening;</li> <li>referral of infants who do not pass screening for appropriate evaluation and early intervention services;</li> <li>documenting of screening results and developing data systems to follow up on infants who do not pass newborn screening; and</li> <li>establishing of quality assurance programs to evaluate the effectiveness of newborn screening and to ensure that all infants are screened for hearing loss" (Level D2 Evidence; p. 42).</li> </ul>

Hearing aid fitting can take place even when only limited audiologic information is available. This may include "auditory brainstem response (ABR) thresholds alone when behavioral measures of hearing sensitivity are precluded due to the child's chronological age or developmental level [or] behavioral responses within two frequency regions (e.g., 500 Hz and 2,000 Hz) indicating broadly the amount of low- and high-frequency hearing loss in each ear, [or] Some combination of limited electrophysiological and behavioral responses" (Level D2 Evidence; pp. 149-150).

"If a frequency transposition hearing aid is being considered, it is important to check the child's high-frequency residual hearing. If a behavioral audiogram indicates that sufficient high-frequency residual hearing is present, the transposition of speech may not be necessary" (Level D2 Evidence; p. 154).

"It is recommended that when hearing loss is present in both ears (bilateral hearing loss), a hearing aid be fitted to each ear (binaural amplification) unless there are audiologic or medical contraindications" (p. 148). These include structural malformations of the outer ear (e.g., atresia) or asymmetrical hearing loss (e.g., moderate loss in one ear and profound loss in the other). Benefits of binaural hearing aids are increased localization of sounds, better understanding of speech in background noise, and improved auditory perception (Level D2 Evidence).

Earmolds should be replaced as needed; "in infants under 6 months of age, it may be necessary to replace the earmolds monthly" (p. 149). Acoustic feedback during typical use is a common sign indicating earmold replacement (Level D2 Evidence).

Speech-language pathologists (SLP) providing services to children with hearing loss should have specialized knowledge and expertise since there are many techniques that can be used to maximize speech development in this population (Level D2 Evidence).

Targets for prescriptive amplification fitting should be determined across the key frequency range for speech perception (250-6,000 Hz). The targets should also consider and account for changes in the ear canal due to development and growth, especially in the first year of life (Level D2 Evidence).

"It is recommended that all newborns (both well baby and NICU babies) have their hearing screened using a physiologic test before being discharged from the hospital. Early identification of hearing loss is important because of the role of hearing and communication in the overall early development of the child" (Level B Evidence; p. 41).

Different techniques can be used to facilitate speech depending on the child's stage of speech development, age, and developmental maturity" (Level D1 Evidence; p. 134). While there are many ways to teach speech sounds to children with hearing loss, "it is recommended that a sound be presented in the following order: auditory, auditory/visual, tactile" (Level D1 Evidence; p. 134).

Some factors that may influence intervention outcomes include: <ul> <li>age of identification and initiation of intervention;</li> <li>amount of intervention;</li> <li>benefit gained from amplification;</li> <li>accessibility to intervention and support services;</li> <li>quality of language input;</li> <li>family participation (Level C Evidence);</li> <li>the characteristics of the hearing loss;</li> <li>environmental factors (e.g., socio-economic factors); and</li> <li>the child's additional disabilities or medical conditions (Level D2 Evidence).</li> </ul>

"If two attempts at behavioral audiometry by a pediatric audiologist are not successful in testing the hearing status of a child within a two-month period, it is recommended that the child be referred for ABR testing" (Level D2 Evidence; p. 73).

If a visual communication approach to support English is chosen (e.g., cued speech, simultaneous communication, or total communication), the following approaches are recommended: <ul> <li>The child should have auditory access to speech in many listening situations through the use of amplification or cochlear implants.</li> <li>The child and those communicating with the child should use and integrate visual information (e.g., facial expressions, signs, gestures) along with auditory information.</li> <li>Professionals should ensure the balanced presentation of visual information and spoken language.</li> <li>Family members should participate in treatment and become fluent users of the visual communication system.</li> <li>Parents should promote and use both spoken and visual language in the home and other natural settings.</li> <li>Children should be taught to monitor their voices as well as the voices of others in order to increase intelligibility of their speech (Level D2 Evidence).</li> </ul> If cued speech is the selected communication approach, it is important to note that cues can be learned quickly, however, practice is needed to become fluent (Level D2 Evidence).

"When evaluating the efficacy of any physiologic screening measure, it is important to recognize that the sensitivity and specificity of the measure are dependent on the criteria used for defining hearing loss, the criteria used for pass/refer, and the technical procedures involved in the test" (Level B Evidence; p. 57).

Certain strategies and environmental enhancements can promote communication, including: providing multiple communication partners (both peers and adults), ensuring adequate lighting for reading speech and signs, minimizing background noise so that residual hearing can be used more effectively, and focusing the child's visual attention to the speaker (Level D1 Evidence). "For children with hearing loss, it is important to remember that reading to the child from an early age is an important way to facilitate learning, just as it is for children with normal hearing" (Level D1 Evidence; p. 123). Speech-language therapy should include ongoing assessment of the child's progress. Strategies should be modified as needed, particularly when treatment objectives have been met or no progress/regression is noted (Level D2 Evidence).

To improve speech production, therapy should follow a developmental approach and emphasize aspects of speech that are less audible or visible (Level D1 Evidence). "It is important to use results from a speech evaluation to select the specific sounds to be included in the speech-language therapy goals" (Level D2 Evidence; p. 132). "For children with hearing loss, it is important to provide therapy for speech production in order to optimize the intelligibility of their speech" (Level D1 Evidence; p. 132).

Because conventional click evoked ABR typically does not detect low-frequency auditory sensitivity and the intensity of the clicks to elicit the ABR is limited; "children with no ABR may have residual hearing and may benefit from hearing aids or FM systems" (Level D2 Evidence; p. 150).

Children with hearing loss should receive a complete developmental assessment and be evaluated across various domains including cognition, social, motor, self-help/adaptive (Level D2 Evidence).

Factors to consider when determining whether or not to perform a cochlear implant should include: degree of residual hearing, benefit from a hearing aid trial, improvement expected with implant, parental interest, communication approach used in home, access to follow-up services, and financial considerations (Level D2 Evidence). "If the family goals do not include the use of spoken language with the child, a cochlear implant is not recommended" (Level D2 Evidence; p. 160).

Children diagnosed with a speech-language delay should undergo a full audiologic assessment instead of a screening (Level D2 Evidence).

Children with hearing loss require a language-rich environment to maximize the development of cognitive and language abilities (Level D2 Evidence).

Visual communication approaches which utilize ASL as the primary language can either be considered bilingual or bilingual-bicultural. For these approaches: <ul> <li>English is considered the second language.</li> <li>If ASL is considered the primary language, parents should become fluent in ASL (through instruction provided by an experienced or native user) to facilitate ASL vocabulary, grammar, and language acquisition.</li> <li>Deaf children and adults should be included in the program to be role models and language models for the child (Level D2 Evidence).</li> </ul>

"It is important to recognize that having an implant does not preclude the use of signing or cued speech. Some habilitation plans may continue use of signing or cueing" (Level D2 Evidence; p. 162).

"Physiologic tests that may require sedation (such as the ABR) are recommended for children whose hearing assessment results are unreliable or inconsistent and whose auditory status remains unknown. ABR is an appropriate test for children suspected of hearing loss who are developmentally delayed or are too young (under 5 months) for reliable conditioned behavioral testing procedures" (Level D2 Evidence; p. 70).

NICU infants should receive a hearing screening as part of their discharge plan since their hearing status may have changed since an earlier screening (Level D2 Evidence).

When considering treatment options, parents should be provided with information on: <ul> <li>different intervention approach options including what each approach entails, the different professionals involved, and what is known about the effectiveness of the interventions;</li> <li>hearing aids/assistive technologies and how to manage them;</li> <li>cochlear implants including the type and severity of hearing loss for which they are appropriate;</li> <li>costs;</li> <li>promoting language development in everyday routines;</li> <li>how to evaluate progress;</li> <li>treatment goals and objectives; and</li> <li>developmental expectations for their child (Level D2 Evidence).</li> </ul>

When interpreting the results of the ABR, it is important to remember that several factors may contribute to a false-negative (i.e., sensorineural hearing loss is present but the child passes the screening) screening result. These include late-onset or acquired loss, cutoff level set too high, and unusual hearing loss configuration. Other factors may lead to false-positive findings (i.e., child has typical hearing but abnormal screening results). These include neurological immaturity, incorrect normative values, conductive hearing loss or ear canal debris, and technical problems due to excessive noise (Level B Evidence).

Regardless of hearing loss type or severity (including mild), early identification and intervention can result in improved outcomes including age-appropriate linguistic milestones (Level C Evidence).

"It is recommended that direct interventions with the child be conducted by a professional who is fluent in the primary language of the child" (Level D2 Evidence; p. 109).

To reinforce visual communication, parents should be taught a variety of strategies including: <ul> <li>"matching the visual and auditory signals;</li> <li>gaining and sustaining attention of the child (such as making sure the child's attention is directed to sounds and other input in the environment);</li> <li>drawing attention to the source of the communication (hands and face); [and]</li> <li>modeling reciprocity (turn-taking and pausing in the communication process)" (Level D2 Evidence; p. 126).</li> </ul> To maximize language learning opportunities, conversations held near the child (even those that do not include the child) should incorporate the visual communication system (Level D2 Evidence).

A periodic comprehensive evaluation to assess individual progress compared to age-expected development should be performed at least annually (Level D2 Evidence).

Since babbling and early vocalizations are essential for speech development, parents should be encouraged to reinforce all of the child's spontaneous vocalizations instead of correcting them (Level D1 Evidence).

"It is recommended that the child's real-ear-to-coupler difference (RECD) be measured each time the child's earmold is changed" (Level D2 Evidence; p. 152).

Hearing screenings should be conducted in a quiet environment when the infant is asleep and not attached to any monitoring devices (Level D1 Evidence).

It is recommended that professionals provide key information and training to families of children with hearing loss who use amplification. This should include information on: <ul> <li>amplification and assistive technology options appropriate for the child;</li> <li>the process (including timeline and costs) to obtain hearing aids;</li> <li>the effectiveness of amplification and reasonable expectations for their child;</li> <li>how the amplification device will affect the child’s hearing (e.g., some sounds may be distorted, some frequencies may not be audible);</li> <li>the use of the amplification device, including a demonstration and explicit verbal and written instructions;</li> <li>the use and care of the amplification device (how to tell if the device is working, how to turn it on/off, how to insert and remove it, how to test/change the batteries, how to complete basic troubleshooting, and who to call if the device is not working);</li> <li>maintaining the hearing aid, which includes providing needed accessories (e.g., battery tester, listening stethoscope, dri-aid kit, air blower, and wax loop);</li> <li>techniques to prevent the child from removing/destroying the hearing aids; and</li> <li>the ongoing need for spare parts/earmolds (Level D2 Evidence).</li> </ul>

It is recommended that all children older than 6 months of age with hearing loss receive a complete age-appropriate assessment of their communicative competence, including, but not limited to: <ul> <li>functional listening skills (auditory skills and speech perception);</li> <li>standardized tests of receptive and expressive language;</li> <li>use of gestures and other nonverbal communication including, but not limited to, augmentative systems and sign language;</li> <li>an oral-motor/speech-motor assessment; and </li> <li>verbal and nonverbal language samples (Level D2 Evidence; p. 88).</li> </ul>

"It is recommended that the use of amplification be initiated as soon as possible after the hearing loss is confirmed.... For the majority of children with hearing loss, amplification devices can provide benefit and access to sound and speech. No child is too young to use some form of amplification device (it is possible to begin hearing aid use as young as 3 to 4 weeks of age). Consistent and early use of appropriate amplification is critical to the optimal development of spoken language" (Level C Evidence; p. 144).

Speech audiometry results are helpful for planning treatment, monitoring the child's ability to understand speech, and assessing the functional benefit of amplification or other devices (Level D2 Evidence). Given that there are speech audiometry procedures that have been developed for infants and young children, it is recommended that an audiologic evaluation include these measures. Examples include speech detection threshold, speech reception threshold for spondees, and speech recognition of common words/sentences within the child’s vocabulary (Level D2 Evidence).

To obtain optimal fitting of amplification devices for infants, monthly audiologic visits may be needed. Frequent audiologic visits are necessary because infants and young children lack the attention, motivation, and tolerance for sufficient audiologic information to be collected in one session. Additionally, behavioral responses are needed to gather information on residual hearing and optimize the fitting since frequency-specific information from the ABR alone is not adequate to fully individualize the hearing aid. Moreover, changes in hearing sensitivity (either permanent or temporary) may warrant a hearing aid adjustment (Level D2 Evidence).

Effective intervention programs have several key components including: <ul> <li>family education, participation, and support;</li> <li>a focus on language and auditory skill development;</li> <li>speech-language treatment;</li> <li>interaction with adults and children with hearing loss;</li> <li>qualified professionals; and</li> <li>ongoing monitoring and assessment of progress (Level D2 Evidence).</li> </ul>

"It is recommended that the decision to perform a cochlear implant not be based solely on the results of the behavioral audiogram or electrophysiologic studies" (Level D2 Evidence; p. 161).

Visual reinforcement audiometry (VRA) should be included as one component of an audiologic test battery for initial assessment or ongoing monitoring. Because VRA requires that the infant have the developmental ability to respond to conditioned procedures, sit, maintain head control, and turn his or her head, it should only be performed on infants that are at a developmental age of at least 6 months (Level D2 Evidence). Visual reinforcement audiometry may provide more reliable thresholds than conditioned orienting reflex (COR) audiometry because VRA thresholds are obtained using headphones (as opposed to loud speakers used with COR) and VRA does not require the child to correctly localize to the sound source for the behavioral response to be judged as accurate (Level D1 Evidence).

Prescriptive amplification fitting procedures should be used "to ensure that amplified speech is audible, comfortable, and safe for the child. One current prescriptive fitting procedure specifically designed for use with infants and children is the Desired Sensation Level (DSL) approach (Seewald 1995)" (Level D2 Evidence; p. 151).

"It is recommended that children with external ear canal malformations or atresia be evaluated by an otolaryngologist. Surgical correction of the ear canal can significantly improve the hearing. Bone-anchored hearing aids are also an option for consideration" (Level D2 Evidence; p. 168).

"It is recommended that custom earmolds be made for the child as soon as the decision has been made to initiate use of amplification. It is recommended that a professional with experience and knowledge take earmold impressions in infants and young children. The process can be more difficult and challenging with infants and young children than with older children and adults because of the size of the ear canals and the frequent and unexpected movements of young children" (p. 149). Professionals taking earmold impressions should use properly-sized tools (e.g., a pediatric syringe for easy dispersal of material into the child's ear canal). Moreover, earmolds should be made "from soft, nonallergenic materials to reduce the likelihood of any discomfort associated with wearing the earmolds" (Level D2 Evidence; p. 149).

Selection of treatment options should take into consideration the characteristics of the child (e.g., health conditions, other disabilities, age) and family characteristics (e.g., home language, stressors, priorities) (Level D2 Evidence).

"For infants who develop profound hearing loss due to meningitis, it is important to do implantation early after the meningitis episode since the cochlea can ossify (fill with bone) and may prevent optimal electrode insertion" (Level D2 Evidence; p. 161).

Risk factors can be useful as a predictor of hearing loss (Level B Evidence). Children with risk factors should receive periodic audiologic monitoring (Level D2 Evidence).

Infants treated with extracorporeal membrane oxygenation should receive audiologic follow up regardless of the results of their initial screening (Level B Evidence).

Children should receive a full audiologic assessment to confirm the presence of a hearing loss and determine the type, configuration, and degree of the loss if: <ul> <li>the child fails a physiologic screening;</li> <li>the child has been identified with a speech-language delay;</li> <li>there are multiple clinical clues, known risk factors, or parental/health care provider suspicions of hearing loss; or</li> <li>the child has a history or recurrent and persistent otitis media with effusion (Level D2 Evidence).</li> </ul>

If sedation is required for audiologic testing, the child should undergo testing at a facility with professionals who are experienced in handling adverse or paradoxical responses to sedation (Level D2 Evidence).

Noise in the testing environment can lead to less accurate screening results (Level C Evidence).

In selecting speech sounds to target in treatment, it is recommended that: <ul> <li>"the child's aided audiogram be used to select sounds that are audible to the child;</li> <li>a developmental speech methodology (such as the Ling methodology) be used as a frame of reference;</li> <li>vowel sounds be among the first sounds selected because a variety of clear vowel sounds constitutes a foundation for the development of natural voice for children with hearing loss;</li> <li>specific speech sounds be chosen as target sounds and playful ways be devised to say the target sound when interacting with the child; and</li> <li>a variety of sounds be used (such as short or long sounds with different pitch changes at different intensities)" (Level D1 Evidence; p. 134).</li> </ul>

Premature infants or those with a neurological injury may show improvement in physiologic screening tests with maturation (Level B Evidence).

Screening approaches using unconditioned behavioral responses (e.g., eye shift after sound presentation, responding to hand clapping) have low sensitivity and specificity and are not reliable (Level C Evidence).

Children not demonstrating development in communication or auditory skills should undergo a reassessment of the amplification device fitting. Alternative assistive devices (e.g., FM systems, cochlear implants) may need to be considered (Level D2 Evidence).

"It is recommended that behavioral audiologic assessment for the selection of amplification include: <ul> <li>assessment of hearing thresholds for both ears using insert earphones;</li> <li>measurement of hearing thresholds over the entire range of speech frequencies from 250-6,000 Hz;</li> <li>examination of the child's functional use of hearing without an amplification device (unaided abilities) using sound field testing at specific frequencies and for speech;</li> <li>assessment of speech perception abilities in each ear using insert earphones when possible;</li> <li>estimation of the child's loudness discomfort levels (LDL) for specific frequencies and speech sounds when possible; and</li> <li>documentation of current communication abilities to provide a performance baseline" (Level D2 Evidence; p. 75).</li> </ul>

Families should be aware that a traditional ASL classroom may be difficult to find since many preschool classrooms serve a variety of children and professionals may use a combination of ASL, English–based sign, and voice to communicate (Level D2 Evidence).

"It is recommended that children with hearing loss have ongoing otologic and audiologic monitoring because hearing loss can fluctuate or progress, and medical conditions can change or evolve over time" (Level D2 Evidence; p. 168).

Although rare (< 1%), there are risks involved with cochlear implantation which include infections, facial paralysis, meningitis (particularly for children whose implants have a positioner), cerebral spinal fluid (CSF) leak, migration of electrodes, and risk from anesthesia (Level D1 Evidence).

When identifying and assessing young children, it is important to consider the following: <ul> <li>Children may be identified through various means including direct screening or parental concern.</li> <li>The assessment is a process that should consider the whole child and include any factors that may affect the child's performance.</li> <li>The assessment is a joint process between parents and professionals.</li> <li>The cultural, linguistic, and family factors of the child should be taken into consideration (Level D2 Evidence).</li> </ul>

Because auditory approaches (i.e., auditory-verbal or auditory-oral) rely on hearing, families opting for this type of communication approach should ensure that the child with hearing loss has appropriate amplification devices or a cochlear implant so that auditory access to speech is available in a variety of listening situations. Even with amplification/cochlear implant, the child may not perceive sounds the same way that those with normal hearing do (Level D2 Evidence).

The combination of FM technology and a hearing aid can increase access to speech in different listening situations (particularly those with increased noise levels). "For some children with severe and profound hearing loss, a combination hearing aid with FM system may be recommended as the primary form of amplification" (Level D2 Evidence; p. 153).

"Responses obtained while the child is wearing the hearing aids (an aided audiogram) should not be used to set or change the electroacoustic characteristics of the hearing aids. This is important because the aided audiogram: <ul> <li>depends on obtaining reliable behavioral responses from the child;</li> <li>provides information about only the softest sounds a child can hear;</li> <li>provides no information about how the child hears speech;</li> <li>indicates responses at only a limited number of frequencies; and</li> <li>may be influenced by background noise in the test booth or internal noise of the hearing aid" (Level D2 Evidence; pp. 152-153).</li> </ul>

It is recommended that children with residual hearing (especially those using a communication approach that incorporates auditory information) use amplification devices/cochlear implants to maximize access to sounds within the speech range. A program for maintaining the device and helping the child adapt to and wear the device (on a consistent basis) should be established (Level D2 Evidence).

"When infants have a developmental age of 6 months, a conditioned behavioral response procedure such as visual reinforcement audiometry (VRA) can be used to obtain frequency-specific hearing thresholds to refine the hearing aid fitting and to measure the functional benefit of the amplification device" (Level D2 Evidence; p. 150).

Children with a flat tympanogram for over 3 months should receive a full audiologic evaluation (Level B Evidence).

If the infant fails the screening and debris from the canal is observed on the ear tip of the device, the infant should be re-screened after the tip is cleaned.

For children with a profound hearing loss, a tactile aid (an assistive device that converts sound to vibration on the skin) may be beneficial and should be used in conjunction with a hearing aid when possible. Tactile aid candidates include those with no cochleae, potential cochlear implant candidates during pre-implantation evaluation, and those who are not receiving a cochlear implant due to family choice or medically fragility (Level D2 Evidence).

"If the parent has a concern, it is important to follow up because there is a higher likelihood that the child may have a hearing problem" (p. 53). Parents can be particularly useful in identifying severe to profound hearing losses, however, mild to moderate losses are more difficult to detect through observation. Parent report alone is not sufficient to determine if a hearing loss exists (Level B Evidence).

Otoacoustic emissions (OAEs) should be incorporated as part of the audiologic test battery. However, some limitations should be noted: <ul> <li>OAE results will not yield information regarding the degree and configuration of hearing loss.</li> <li>Lack of emissions does not verify that permanent hearing loss is present.</li> <li>OAE test results alone will not identify children with hearing loss due to auditory neuropathy.</li> <li>Middle ear pathology, environmental noise, and other factors may affect OAE results (Level C Evidence).</li> </ul>

The speech-language pathologist providing intervention should understand "the results of the child's hearing aid fitting so that specific speech goals can be developed based on the audibility provided by the hearing aid or cochlear implant [and] the communication approach being used with the child in order to present speech and provide feedback about the child's speech in a way that is consistent with the child's intervention program" (Level D2 Evidence; p. 133).

Hearing aids for infants and young children should offer a range of coupling choices to make the hearing aid compatible with other technologies (e.g., FM systems, direct audio input capability for connection to television/microphone, and telecoil for connection to telephone) (Level D2 Evidence).

Speech and language interventions (both formal and informal) should be positive, target sounds/words that are meaningful, be at the child's skill level, and promote a feeling of success with vocal use (Level D2 Evidence).

There are a variety of approaches for children with more significant hearing loss. "There is no one approach that has been shown to be best for all children with hearing loss and their families. For achieving specific goals (such as speech production), there is evidence that some approaches are more effective than others" (Level C Evidence; p. 122). "Regardless of the communication approach, the most positive language outcomes generally result from providing intervention early" (Level C Evidence; p. 122).

American Speech-Language-Hearing Association

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