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Summary of Clinical Practice Guideline

Commission for the Early Detection of Hypoacusis (CODEPEH), Spain

Early Hearing Detection and Intervention: 2010 CODEPEH Recommendation
Trinidad-Ramos, G., de Aguilar, V. A., et al. (2010).
Acta Otorrinolaringologica Espanola, 61(1), 69-77.

AGREE Rating: Recommended with Provisos

Description:
This guideline provides recommendations for early hearing detection and intervention (EHDI) programs. The target audience of this guideline is audiologists and speech-language pathologists. Recommendations are provided in the areas of screening, audiological evaluation, intervention and surveillance.

Recommendations:

Permanent Childhood Hearing Loss

  • Screening/Assessment

    • Assessment

      • Audiologic

        • General Findings

          • “Diagnostic confirmation of children who have not passed the newborn hearing screening must be completed in the third month of age to enable early audiological diagnosis” (p. 71).

          • A diagnosis of hearing loss should not be delayed beyond 4-5 months of age.

          • Children diagnosed with a hearing loss should be referred for additional testing by a team of professionals (e.g., otolarygologist, pediatrician, pediatric neurologist, geneticist) to determine the etiology. Additionally, children with diagnosed hearing loss should be referred for a vision examination.

          • Diagnostic confirmation of hearing loss should be based on multiple tests and “should always include ABR, tympanometry, stapedial reflex (using 1000 Hz as a sound carrier), and TEOAE, repeated at least 2 times with a difference of one to 4 weeks” (p. 71).

          • The following are quality indicators of newborn hearing screening programs:

            • The objective of the program is to reveal all unilateral/bilateral hearing loss present at birth regardless of severity or etiology. “Screening procedures must ensure false negatives tend towards 0%” (p. 74).

            • The first hearing screening should take place within the first month of life on more than 95% of children. All children who did not pass the first screening should be re-screened.

            • The referral rate from screening should not exceed 4%. Confirmatory testing should be carried out during the third month of life on all children failing the re-screen.

            • Adequate early intervention should be established before 6 months of age with at least 90% of eligible children.

            • If amplification is required, “no more than one month should pass between the indication and adaptation [of the amplification] in 95% of the candidates” (p. 75).

            • For children with late-onset congenital or acquired hearing loss, “95% should have started treatment within 45 days after diagnosis” (p. 75).

        • Auditory Brainstem Response (ABR)

          • “To confirm a permanent hearing loss in children under 3 years old, it is necessary to have carried out at least one ABR test” (p. 72).

          • Diagnostic confirmation of hearing loss should be based on multiple tests and “should always include ABR, tympanometry, stapedial reflex (using 1000 Hz as a sound carrier), and TEOAE, repeated at least 2 times with a difference of one to 4 weeks” (p. 71).

        • Otoacoustic Emissions (OAE) and Tympanometry/Acoustic Reflex

          • Diagnostic confirmation of hearing loss should be based on multiple tests and “should always include ABR, tympanometry, stapedial reflex (using 1000 Hz as a sound carrier), and TEOAE, repeated at least 2 times with a difference of one to 4 weeks” (p. 71).

    • Screening

      • General Findings

        • If a re-screen is indicated, infants “should be evaluated bilaterally even if only one side did not pass the initial test” (p. 71).

        • The following are quality indicators of newborn hearing screening programs:

          • The objective of the program is to reveal all unilateral/bilateral hearing loss present at birth regardless of severity or etiology. “Screening procedures must ensure false negatives tend towards 0%” (p. 74).

          • The first hearing screening should take place within the first month of life on more than 95% of children. All children who did not pass the first screening should be re-screened.

          • The referral rate from screening should not exceed 4%. Confirmatory testing should be carried out during the third month of life on all children failing the re-screen.

          • Adequate early intervention should be established before 6 months of age with at least 90% of eligible children.

          • If amplification is required, “no more than one month should pass between the indication and adaptation [of the amplification] in 95% of the candidates” (p. 75).

          • For children with late-onset congenital or acquired hearing loss, “95% should have started treatment within 45 days after diagnosis” (p. 75).

        • To monitor the quality of newborn hearing screening programs key data should be collected such as: the number of newborns screened on hospital admission, number of newborns confirmed with hearing loss before 3 months of age, the number of infants enrolled in early intervention by 6 months, number of children with non-syndromic hearing loss who have adequate language and communication skills by school age, and the number of children referred for cochlear implants.

      • Risk Factors/Surveillance

        • Infants with NICU stays greater than five days should receive an ABR [auditory brainstem response] to avoid a diagnostic error of neural hearing losses. If the infant fails the ABR, the infant should be directly referred for re-assessment which should include ABR and TEOAE [transient evoked otoacoustic emissions], if these were not performed during the screening phase.

        • Infants re-admitted during the first month of life with auditory risk factors should have repeat hearing screening before discharge.

        • “Children with risk factors should be tracked individually according to the probability of late onset hearing loss. Even if they pass the neonatal test, they should be reevaluated at least once before age 24-30 months. In children with high risk, this should be done earlier and more frequently” (p. 71). Particular interest should be paid to children with a family history or parental suspicion of hearing loss, children with cytomegalo virus infection, children who have undergone chemotherapy or extracorporeal oxygenation, or those with syndromes, trauma, or neurodegenerative disorders associated with hearing loss.

        • In screening programs using TEOAE, “children who present risk factors for retrocochlear hearing loss must be subjected to a complementary test by AABR or by Auditory Brainstem Evoked Potentials (ABEP) for diagnosis even if they have passed the otoacoustic emissions, to avoid false negatives associated with the existence of auditory neuropathy” (p. 71).

        • For screening programs using the AABR, children who are at risk for retrocochlear hearing loss should receive a TEAOE if the AABR is not passed to document the possible existence of auditory neuropathy.

      • Physiologic

        • “Infants with no history or risk of retrocochlear hearing loss may be tested in the screening phase by either TEOAE [transient evoked otoacoustic emissions] or by AABR [automated auditory brainstem response]. If the AABR screening is not passed, then a second test would not be needed and they could be referred for diagnostic confirmation. However, if the TEOAE are used, especially if performed before the child is older than 72 hours, then the test should be repeated at least once before referral to the diagnostic phase” (p. 71).

        • Infants with NICU stays greater than five days should receive an ABR [auditory brainstem response] to avoid a diagnostic error of neural hearing losses. If the infant fails the ABR, the infant should be directly referred for re-assessment which should include ABR and TEOAE [transient evoked otoacoustic emissions], if these were not performed during the screening phase.

        • In screening programs using TEOAE, “children who present risk factors for retrocochlear hearing loss must be subjected to a complementary test by AABR or by Auditory Brainstem Evoked Potentials (ABEP) for diagnosis even if they have passed the otoacoustic emissions, to avoid false negatives associated with the existence of auditory neuropathy” (p. 71).

        • For screening programs using the AABR, children who are at risk for retrocochlear hearing loss should receive a TEAOE if the AABR is not passed to document the possible existence of auditory neuropathy.

  • Treatment

    • Communication

      • General Findings

        • Treatment of hearing loss including speech therapy and amplification should be initiated before 6 months of age.

        • “Early intervention services (speech therapy and hearing aids) must be provided by staff with appropriate qualifications and sufficient experience in infant hearing loss” (p. 73).

    • General Findings

      • The following are quality indicators of newborn hearing screening programs:

        • The objective of the program is to reveal all unilateral/bilateral hearing loss present at birth regardless of severity or etiology. “Screening procedures must ensure false negatives tend towards 0%” (p. 74).

        • The first hearing screening should take place within the first month of life on more than 95% of children. All children who did not pass the first screening should be re-screened.

        • The referral rate from screening should not exceed 4%. Confirmatory testing should be carried out during the third month of life on all children failing the re-screen.

        • Adequate early intervention should be established before 6 months of age with at least 90% of eligible children.

        • If amplification is required, “no more than one month should pass between the indication and adaptation [of the amplification] in 95% of the candidates” (p. 75).

        • For children with late-onset congenital or acquired hearing loss, “95% should have started treatment within 45 days after diagnosis” (p. 75).

    • Cochlear Implants

      • General Findings

        • Proper fitting and use of hearing aids allows the use of the child’s residual hearing. When hearing aids and early intervention “do not achieve the expected results between 3 and 6 months, then the inclusion of children in the cochlear implant programme must be considered” (p. 73).

    • Hearing Aids

      • General Findings

        • Treatment of hearing loss including speech therapy and amplification should be initiated before 6 months of age.

        • If an evaluation indicates that hearing aids are necessary, they should be provided within one month.

        • “Early intervention services (speech therapy and hearing aids) must be provided by staff with appropriate qualifications and sufficient experience in infant hearing loss” (p. 73).

        • Proper fitting and use of hearing aids allows the use of the child’s residual hearing. When hearing aids and early intervention “do not achieve the expected results between 3 and 6 months, then the inclusion of children in the cochlear implant programme must be considered” (p. 73).

Keywords:
Hearing Loss, Early Hearing Detection and Intervention

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