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Summary of Clinical Practice Guideline

Joint Committee on Infant Hearing

Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs
Joint Committee on Infant Hearing (2007).
Pediatrics, 120(4), 898-921.

AGREE Rating: Highly Recommended

Description:
This consensus-based guideline provides recommendations for screening infants for hearing loss, assessing infants identified as at-risk for hearing impairment, and providing appropriate early intervention for infants with hearing impairment. The intended audience of these guidelines is not specified, however they are relevant to all professionals involved in the screening, assessment or treatment of hearing in infants.

Recommendations:

  • Screening/Assessment

    • Assessment

      • Communication

        • General Findings

          • Because they are at risk for speech and language delay, children with unilateral or minimal to mild bilateral hearing loss should undergo developmental monitoring at regular 6-month intervals.

          • If there are significant family or physician concerns or if the child fails the speech-language section of a global screening, the child should be referred for additional evaluation by an audiologist and speech-language pathologist.

      • Audiologic

        • General Findings

          • Audiologic assessments and selecting and fitting of amplification devices should be provided by audiologists with expertise and knowledge of evaluating newborns and infants with hearing loss.

          • “If the family chooses personal amplification for their infant, hearing aid selection and fitting should occur within one month of initial confirmation of hearing loss even when additional audiologic assessment is ongoing” (p. 21).

          • Infants who fail the newborn screening should receive a comprehensive audiologic evaluation which includes a test battery used to assess the integrity of the auditory system, determine hearing sensitivity and type of loss, establish baseline for ongoing monitoring, and provide information for amplification fitting.

          • Children with confirmed hearing loss or middle ear dysfunction should be receive and otologic or other medical evaluation.

          • For infants birth through a developmental age of 6 months, the test battery should include:

            • Child and family history including an assessment of risk factors and parental reports of the infant’s response to sounds

            • Frequency-specific ABR with air-conduced bursts and bone-conducted bursts as needed

            • Click-evoked ABR

            • OAEs (transient evoked or distortion product)

            • 1000 Hz tympanometry

            • Clinical observations of the infant’s auditory behavior.

          • For children with a developmental age between 6 and 36 months, the test battery should include:

            • Child and family history including information on the child’s attainment of communication milestones and parental report of the child’s auditory and visual behaviors

            • Behavioral audiometry (visual reinforcement or conditioned–play audiometry) consisting of pure tones across the frequency range as well as speech detection and speech recognition measures

            • OAE

            • Tympanometry and acoustic reflex thresholds

            • If reliable responses cannot be obtained through behavioral audiometry, the child should receive an ABR.

               

          • If there are significant family or physician concerns or if the child fails the speech-language section of a global screening, the child should be referred for additional evaluation by an audiologist and speech-language pathologist.


           

        • Auditory Brainstem Response (ABR)

          • Infants who do not pas an ABR screening in the NICU should receive a direct referral to an audiologist for re-screening and, a comprehensive assessment including ABR when indicated.

          • “At least one ABR test is recommended as part of a complete audiology diagnostic evaluation for children younger than 3 years for confirmation of permanent hearing loss” (p. 2). For infants birth through a developmental age of 6 months, the test battery should include:

            • Child and family history including an assessment of risk factors and parental reports of the infant’s response to sounds

            • Frequency-specific ABR with air-conduced bursts and bone-conducted bursts as needed

            • Click-evoked ABR

            • OAEs (transient evoked or distortion product)

            • 1000 Hz tympanometry

            • Clinical observations of the infant’s auditory behavior.

          • For children with a developmental age between 6 and 36 months, the test battery should include:

            • Child and family history including information on the child’s attainment of communication milestones and parental report of the child’s auditory and visual behaviors

            • Behavioral audiometry (visual reinforcement or conditioned –play audiometry) consisting of pure tones across the frequency range as well as speech detection and speech recognition measures

            • OAE

            • Tympanometry and acoustic reflex thresholds

            • If reliable responses cannot be obtained through behavioral audiometry, the child should receive an ABR.

           

        • Auditory Steady State Response (ASSR)

          • “There is insufficient evidence for the use of auditory steady state response as the sole measure of auditory status in newborn and infant populations” (p. 14).

        • Otoacoustic Emissions (OAE)

          • For infants birth through a developmental age of 6 months, the test battery should include:

            • Child and family history including an assessment of risk factors and parental reports of the infant’s response to sounds

            • Frequency-specific ABR with air-conduced bursts and bone-conducted bursts as needed

            • Click-evoked ABR

            • OAEs (transient evoked or distortion product)

            • 1000 Hz tympanometry

            • Clinical observations of the infant’s auditory behavior.

          • For children with a developmental age between 6 and 36 months, the test battery should include:

            • Child and family history including information on the child’s attainment of communication milestones and parental report of the child’s auditory and visual behaviors

            • Behavioral audiometry (visual reinforcement or conditioned –play audiometry) consisting of pure tones across the frequency range as well as speech detection and speech recognition measures

            • OAE

            • Tympanometry and acoustic reflex thresholds

            • If reliable responses cannot be obtained through behavioral audiometry, the child should receive an ABR.

          • Behavioral

            • For infants birth through a developmental age of 6 months, the test battery should include:

              • Child and family history including an assessment of risk factors and parental reports of the infant’s response to sounds

              • Frequency-specific ABR with air-conduced bursts and bone-conducted bursts as needed

              • Click-evoked ABR

              • OAEs (transient evoked or distortion product)

              • 1000 Hz tympanometry

              • Clinical observations of the infant’s auditory behavior.

            • For children with a developmental age between 6 and 36 months, the test battery should include:

              • Child and family history including information on the child’s attainment of communication milestones and parental report of the child’s auditory and visual behaviors

              • Behavioral audiometry (visual reinforcement or conditioned –play audiometry) consisting of pure tones across the frequency range as well as speech detection and speech recognition measures

              • OAE

              • Tympanometry and acoustic reflex thresholds

              • If reliable responses cannot be obtained through behavioral audiometry, the child should receive an ABR.

          • Tympanometry/Acoustic Reflex

            • “There are insufficient data for routine use of acoustic middle ear muscle reflexes in the initial diagnostic assessment of infants younger than 4 months” (p. 14).

            • For infants birth through a developmental age of 6 months, the test battery should include:

              • Child and family history including an assessment of risk factors and parental reports of the infant’s response to sounds

              • Frequency-specific ABR with air-conduced bursts and bone-conducted bursts as needed

              • Click-evoked ABR

              • OAEs (transient evoked or distortion product)

              • 1000 Hz tympanometry

              • Clinical observations of the infant’s auditory behavior.

            • For children with a developmental age between 6 and 36 months, the test battery should include:

              • Child and family history including information on the child’s attainment of communication milestones and parental report of the child’s auditory and visual behaviors

              • Behavioral audiometry (visual reinforcement or conditioned –play audiometry) consisting of pure tones across the frequency range as well as speech detection and speech recognition measures

              • OAE

              • Tympanometry and acoustic reflex thresholds

              • If reliable responses cannot be obtained through behavioral audiometry, the child should receive an ABR.

      • Screening

        • General Findings

          • Even if only one ear failed the initial screening, both ears should be re-screened.

          • Infants who are re-admitted to the hospital within the first month of life with conditions that are associated with hearing loss should receive a repeat hearing screening before hospital discharge.

          • If needed, outpatient re-screening should occur within one month and testing should include both ears even if only one ear failed the initial screening.

        • Risk Factors/Surveillance

          • Infants who do not pas an auditory brainstem response (ABR) screening in the NICU should receive a direct referral to an audiologist for re-screening and, a comprehensive assessment including ABR when indicated.

          • “Infants who pass the neonatal screening but have a risk factor should have at least one diagnostic audiology assessment by 24 to 30 months of age. Early and more frequent assessment may be indicated for children with cytomegalovirus (CMV) infection, syndromes associated with progressive hearing loss, neurodegenerative disorders, trauma, or culture-positive postnatal infections associated with sensorineural hearing loss; for children who have received ECMO or chemotherapy; and when there is caregiver concern or a family history of hearing loss” (p. 2).

          • ABR is the only appropriate screening technology for the NICU. Infants in the NICU who do not pass the automated ABR screening should be referred directly to an audiologist for re-screening or a comprehensive evaluation (when indicated).

        • Family Counseling

          • Screening results should be conveyed to families as soon as possible. If follow-up is needed, information regarding the importance of prompt follow-up should be shared and an appointment for follow-up testing should be offered before hospital discharge.

        • Physiologic

          • Physiologic measures such as OAE and automated ABR “must be used to screen infants and newborns for hearing loss” (p. 9).

          • Hearing loss due to neural conduction disorders or auditory neuropathy will not be identified through OAE testing.

          • Either OAEs or ABR will detect 40dB or greater peripheral hearing loss.

          • Infants who fail OAE screening but pass an AABR screen can be considered as passing the screening. However, “infants in the well-baby nursery who fail automated ABR should not be rescreened by OAE and “passed,” because such infants are presumed to be at risk of having a subsequent diagnosis of auditory neuropathy/dyssynchrony” (p. 11).

          • Screening technologies that integrate automated response detection offer several advantages including reducing screener error or operator bias, eliminating individual test interpretation, and increasing consistency across screeners, infants, and test conditions. However when technologies that employ statistical probability to make pass/fail decisions (i.e., OAE, automated ABR) are used, there is an increased probability that a result of “pass” will be obtained by chance alone when the screening is performed repeatedly. Organizational re-screening policies should consider this.

          • ABR is the only appropriate screening technology for the NICU. Infants in the NICU who do not pass the automated ABR screening should be referred directly to an audiologist for re-screening or a comprehensive evaluation (when indicated).

    • Treatment

      • General Findings

        • “The initiation of early intervention services should begin as soon as possible after the diagnosis of hearing loss, but no later than 6 months of age” (p. 17).

        • Through early intervention, children with hearing loss should receive regular developmental assessments in speech and language, cognitive skills, auditory skills, vocabulary, and social-emotional development every 6 months until the child turns three years of age.

      • Hearing Aids

        • Selection/Fitting/Validation

          • Audiologic assessments and selecting and fitting of amplification devices should be provided by audiologists with expertise and knowledge of evaluating newborns and infants with hearing loss.

          • “If the family chooses personal amplification for their infant, hearing aid selection and fitting should occur within one month of initial confirmation of hearing loss even when additional audiologic assessment is ongoing” (p. 21).

    Keywords:
    Hearing Loss, Early Hearing Detection and Intervention

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