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Summary of Clinical Practice Guideline

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

Clinical Practice Guideline: Report of the Recommendations. Hearing Loss, Assessment and Intervention for Young Children (Age 0-3 Years)
New York State Department of Health, Early Intervention Program. (2007).
Albany (NY): NYS Department of Health, Publication No. 4967, 354 pages.

AGREE Rating: Highly Recommended

Description:
This evidence-based and consensus-based guideline provides recommendations for the assessment and intervention of hearing loss for young children ages birth to three. 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. Each recommendation is provided with a strength of evidence rating defined as Level A (strong evidence), Level B (moderate evidence), Level C (limited evidence), Level D1 (consensus panel opinion based on topics where a systematic review has been conducted), and Level D2 (consensus panel opinion not based on findings from a systematic review).

Recommendations:

  • Screening/Assessment

    • Assessment

      • Audiologic

        • General Findings

          • “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).


        • Otoacoustic Emissions (OAE)

          • OAEs should be incorporated as part of the audiologic test battery. However some limitations are noted, specifically:

            • OAE results will not yield information regarding the degree and configuration of hearing loss.

            • Lack of emissions does not verify that permanent hearing loss is present.

            • OAE test results alone will not identify children with hearing loss due to auditory neuropathy.

            • Middle ear pathology, environmental noise and other factors may affect OAE results (Level C Evidence).

          • “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).

        • Auditory Brainstem Response (ABR)

          • “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).


        • Behavioral

          • 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).


        • Tympanometry/Acoustic Reflex

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

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

      • Communication

        • General Findings

          • Professionals conducting communication assessments with young children with hearing loss should have:


    • Screening

      • General Findings

        • “It is recommended that a newborn hearing screening program include the following components:

          • Infant hearing screening with an objective physiologic test before discharge from the birthing facility

          • Communication of screening results to parents

          • Provision of written educational materials

          • 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

          • Referral of infants who do not pass screening for appropriate evaluation and early intervention services

          • Documenting screening results and developing data systems to follow up on infants who do not pass newborn screening

          • Establishing 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).

        • 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).

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

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

      • Risk Factors/Surveillance

        • 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).

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

        • “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).

        • 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:

          • The child fails a physiologic screening, or

          • The child has been identified with a speech-language delay, or

          • There are multiple clinical clues, known risk factors, or parental/health care provider suspicions of hearing loss, or

          • The child has a history or recurrent and persistent otitis media with effusion (Level D2 Evidence).

      • Family Counseling

        • 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).

      • Physiologic

        • “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).

        • 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.

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

        • 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:

          • The child fails a physiologic screening, or

          • The child has been identified with a speech-language delay, or

          • There are multiple clinical clues, known risk factors, or parental/health care provider suspicions of hearing loss, or

          • The child has a history or recurrent and persistent otitis media with effusion (Level D2 Evidence).

      • Behavioral

        • 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).

  • Treatment

    • Communication

      • General Findings

        • 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).


      • Combination Approaches

        • 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).


      • Auditory/Oral Approaches

        • 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).


      • Visual Approaches (e.g., sign)

        • Visual communication approaches which utilize ASL as the primary language can either be considered bilingual or bilingual-bicultural. With these approaches:

        • English is considered the second language (Level D2 Evidence).

        • 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 (Level D2 Evidence).

        • Deaf children and adults should be included in the program to be role models and language models for the child (Level D2 Evidence).

        • “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).

        • 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).

    • General Findings

      • 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).


    • Hearing Aids

      • General Findings

        • 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).


      • Osseointegrated Implant (e.g., BAHA)

        • “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).

        • “…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).

      • Selection/Verification/Validation

        • “It is recommended that behavioral audiologic assessment for the selection of amplification include:


    • Hearing Assistive Technology Systems

      • 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 implant) may need to be considered (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).

      • 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 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).

      • 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).

      • 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).

    • Cochlear Implants


      • General Findings

        • “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).


      • Age/Duration of Implantation

        • 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 [and] there does not appear to be increased medical risk from early implantation compared with later implantation” (Level D1 Evidence) (p. 160).

        • “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).

      • Risks/Contraindications

        • 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 [and] there does not appear to be increased medical risk from early implantation compared with later implantation” (Level D1 Evidence) (p. 160).

        • “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).

        • 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).

Keywords:
Hearing Loss, Early Hearing Detection and Intervention

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