Permanent Childhood Hearing Loss
External Scientific Evidence
Evidence-Based Practice Guidelines 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.Added: July 2012
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).
- “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).
Evidence-Based Systematic Reviews Universal Neonatal Hearing Screening
Merlin, T., Hedayati, H., et al. (2007).
Adelaide: Adelaide Health Technology Assessment (AHTA), MSAC reference 17 Assessment Report, 266 pages.
This review meets the criteria for a high-quality evidence-based systematic review.Added: May 2013
This is a review of the literature examining the prevalence of permanent childhood hearing loss (PCHL), the diagnostic accuracy of hearing screening tests used for universal newborn hearing screening (UNHS), and the safety, effectiveness and cost-effectiveness of UNHS.
- The accuracy of transient evoked otoacoustic emissions (TEOAE) testing is affected by ambient noise. In quiet environments, TEOAE has been noted to have sensitivity up to 100% and specificity of 92%. However, the positive predicative value (1.5%) of an initial TEOAE test to correctly diagnose PCHL is very low. This is likely due to "the frequency of transient hearing losses in newborns due to ear occlusion and the low prevalence [of PCHL]" (p. ix).
- Early model automated auditory brainstem response (AABR) tests have a reported sensitivity of 80% and specificity of 92%. The positive predictive value of the test (2.2%) was considered low however experts note that later model AABR tests may have improved diagnostic accuracy.
- False positive rates of either test (i.e., TEOAE or AABR) may be minimized with the introduction of a second or third stage screen prior to diagnostic testing. "This may, however, result in unnecessary caregiver anxiety and added costs and delays in rehabilitation" (p. ix).
Early Detection of Hearing Impairment in Newborns and Infants
Ptok, M. (2011).
Deutsches Aerzteblatt International, 108(25), 426-431.
Added: July 2012
This is a systematic review of the literature examining the early detection and treatment of hearing loss in in newborns and infants.
“Two-stage (or combined) screening yields better results than measurement of otoacoustic emissions alone; however, children exhibiting specific risk factors should immediately undergo AABR [automated auditory brainstem response] screening or (confirmatory/excluding) diagnostic evaluation” (p. 431).
Universal Newborn Hearing Screening, a Revolutionary Diagnosis of Deafness: Real Benefits and Limitations
Papacharalampous, G. X., Nikolopoulos, T. P., et al. (2011).
Eur Arch Otorhinolaryngol, 268(10), 1399-1406.Added: July 2012
This is a systematic review of the literature investigating the efficacy, results, and outcomes of universal newborn hearing screening.
- To improve the efficacy of Universal Hearing Screening Protocols, a primary goal should be to decrease the initial referral rate. Higher pass rates are typically obtained from the second day of life forward. “However, a two-stage screening (more than one attempt during the same day) appears to improve the pass rate significantly, even on the first day of life. The advantages of OAE-based [otoacoustic emissions]screenings in comparison to a-ABR [automated auditory brainstem response] protocols are speediness and expensive consumables becoming unnecessary. However, the use of a-ABR provides a significant decrease in referral rates compared with TEOAE [transient evoked otoacoustic emissions] plus AABR and TEOAEs alone. Combined TEOAE and AABR protocols also result in low referral rates” (p. 1404).
- “False positive rates remain considerably high when newborns are screened with TEOAEs and cases of auditory neuropathy are missed as well. The combination of TEOAEs and AABR provides a significantly reduced referral rate, although such an alteration slightly increases the cost of screening” (p. 1405).
Hearing Screening in Newborns. Systematic Review of Accuracy, Effectiveness, and Effects of Interventions after Screening
Wolff, R., Hommerich, J., et al. (2009).
Archives of Disease in Childhood, [Epub ahead of print].This review meets the criteria for a high-quality evidence-based systematic review.Added: July 2012
This is a systematic review of randomized and non-randomized controlled trials investigating the accuracy and effectiveness of hearing screening in newborns and investigating the effects of early intervention.
“No overall reliable evaluation is possible for the diagnostic accuracy of otoacoustic emissions (OAE) and auditory brain stem response (ABR) as initial screening tests, as there has been no evaluation in an adequately large group of children without risk factors” (p. 11).
Clinical Expertise/Expert Opinion
Consensus Guidelines 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.Added: July 2012
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.
- 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).
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.Added: July 2012
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.
- “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.
Newborn Hearing Screening Programme (UK)
Neonatal Hearing Screening and Assessment: Automated Auditory Brainstem Response – Information and Guidelines for Screening Hearing in Babies
Elliott, C., Lightfoot, G., et al. (2002).
In S. Mason (Ed.), England: Newborn Hearing Screening Programme, 5 pages.Added: July 2012
This guideline provides recommendations for screening and testing the hearing of babies in the first few months of life with automated auditory brainstem response (AABR).
The following guidelines should be considered when screening for hearing loss with AABR:
- For AABR screening provided outside of the designated clinic area, “levels of acoustical and electrical interference must be sufficiently low so as not to influence the results of the test. Careful selection of the local test area or room may be necessary in order to achieve satisfactory environmental conditions” (p. 2).
- “If re-usable electrodes are employed then appropriate precautions must be taken to avoid the risk of cross infection. Since testing is often performed on very young babies extreme care must be adopted regarding preparation of the skin for placement of the electrodes. The use of harsh skin preparation materials should be avoided” (p. 2).
- “Any comparison on the performance of different AABR systems must take into consideration the baseline calibration of the click stimulus (ppeSPL). The value of ppeSPL on which the dBnHL is based may be different for different AABR systems. The recommended value in the air conduction click protocol is 33 dBpeSPL” (p. 3).
- Certain test parameters for data collection are a fixed part of protocol and must not be changed as this may invalidate the scoring algorithm. These include: gain or sensitivity of the amplifier, level of amplitude artifact rejection, filter bandwidth, acquisition window, and number of averaging sweeps.
- The machine based scoring algorithm must be clinically proven in terms of its sensitivity and specificity and “provide a separate screening result for each ear so that referral of babies for further testing can be initiated using either unilateral or bilateral referral criteria” (p. 4).
- The interpretation of the screening results should under no circumstances be performed by inexperienced personnel.
No information was found pertaining to client/patient/caregiver perspectives.
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