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).
- 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).
Evidence-Based Systematic Reviews Monitoring for Postnatal Hearing Loss Using Risk Factors: A Systematic Literature Review
Beswick, R., Driscoll, C., et al. (2012).
Ear & Hearing, 33(6), 745-756.
This review meets the criteria for a high-quality evidence-based systematic review.Added: May 2013
This is a systematic review of the peer- and non-peer-reviewed published literature investigating the effectiveness of using a risk-factor registry for targeted surveillance to identify hearing loss after the newborn hearing screen.
- Among risk-factor registries, the most commonly used risk factors included: gestational age (GA), low birthweight (LBW), craniofacial anomalies, respirator support, aminoglycoside use, and TORCH infections (Toxoplasmosis, other infections, Rubella, Cytomegalovirus [CMV], and Herpes simplex virus).
- Risk factors strongly associated with hearing loss were: CMV, extracorporeal membrane oxygenation (ECMO), congenital diaphragmatic hernia, and persistent pulmonary hypertension of the newborn (PPHN).
- Risk factors with limited or no association with hearing loss included LBW, preauricular skin tags and ear pits(PSEP), neonatal toxoplasmosis.
- No studies comparing risk-factor registries to alternative protocols for detecting postnatal hearing loss were found.
A Systematic Review of the Incidence of Sensorineural Hearing Loss in Neonates Exposed to Herpes Simplex Virus (HSV)
Westerberg, B. D., Atashband, S., et al. (2008).
Int J Pediatr Otorhinolaryngol, 72(7), 931-937.
Added: July 2012
This is a systematic review of longitudinal studies of children infected with Herpes simplex virus (HSV) prior to 6 weeks of age to identify the incidence of sensorineural hearing loss (SNHL).
- “Audiometric screening of all infants in well baby nurseries and neonatal intensive care units is recommended prior to discharge. There is no recommendation for audiometric rescreening if children with exposure to HSV either in utero or at the time of delivery if there are no clinical signs of HSV infection, unless the child fails the first screen” (p. 936).
- “Neonates with clinical and/or serological evidence of infection with HSV should undergo hearing screening prior to discharge from hospital. The incidence of development of delayed-onset of SNHL in this group of patients is unknown. It may be prudent for the child to have at least one diagnostic audiometric assessment prior to 30 months of age pending availability of further data.” (p. 936).
- “There is limited evidence to support the assumption that HSV infection is a cause for SNHL in children…However, clinical infection with HSV is associated with significant other confounding factors (i.e., prolonged stay in NICU) that have been linked with development of SNHL in neonates” (p. 936).
A Systematic Review of Neonatal Toxoplasmosis Exposure and Sensorineural Hearing Loss
Brown, E. D., Chau, J. K., et al. (2009).
Int J Pediatr Otorhinolaryngol, 73(5), 707-11.Added: July 2012
This is a systematic review of cohort studies investigating the prevalence of delayed and immediate onset toxoplasmosis in individuals with sensorineural hearing loss (SNHL).
- "Children with congenital toxoplasmosis should have hearing screening performed at birth" (p. 710).
- Children who have received and complied with a 12-month course of antiparasitical therapy initiated prior to 2.5 months of age should have repeat audiometric evaluation at 24-30 months of age.
- "Children with congenital toxoplasmosis that had no treatment, partial treatment, delayed onset of treatment, or compliance issues should undergo annual audiologic monitoring until able to reliably self-report hearing loss” (p. 710).
A Systematic Review of Pediatric Sensorineural Hearing Loss in Congenital Syphilis
Chau, J., Atashband, S., et al. (2009).
Int J Pediatr Otorhinolaryngol, 73(6), 787-792.Added: July 2012
This is a systematic review of prospective longitudinal studies investigating the prevalence, characteristics, severity, and progression of sensorineural hearing loss (SNHL) in children with congenital syphilis.
- “All infants born with congenital syphilis should have hearing screening performed at birth if not performed under a neonatal hearing screening program” (p. 791). There are no data to indicate that a different screening protocol be used with this population than other well babies.
- “All children with confirmed syphilis serology at birth who have received appropriate neonatal antibiotic treatment should have a repeat hearing screening performed at least once further by 24-30 months of age” (p. 791). Further data confirming no delayed–onset SNHL in this patient group may make this second screening unnecessary.
- "All children with confirmed syphilis on serological testing who did not receive appropriate antibiotic treatment should have on going audiological monitoring at least on an annual basis. The influence of partial and/or late antibiotic treatment on the subsequent development of SNHL is unknown” (p. 791).
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.
- Infants who do not pass 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).
Continuing Care of NICU Graduates
Verma, R. P., Sridhar, S., et al. (2003).
Clin Pediatr, 42(4), 299-315.Added: July 2012
This guideline provides recommendations for the on-going care of infants who have been discharged from the neonatal intensive care unit (NICU). The guideline was developed primarily for pediatricians but gives key recommendations regarding feeding, swallowing, and hearing with this population.
- All neonatal intensive care unit (NICU) infants should receive a hearing screening before discharge. “Infants who fail an initial evaluation should be rescreened within a month and, if necessary, referred to an audiologist” (p. 15).
- “Infants diagnosed with persistent pulmonary hypertension of the newborn infant have a 53% chance of developing severe sensorineural hearing loss at 5-6 months of age. Some of these infants may have passed their initial screening before discharge. If they fail the second screening, they should be referred to an audiologist” (p. 15).
Newborn Hearing Screening Programme (UK)
Newborn Hearing Screening and Assessment: Guidelines for Surveillance and Audiological Monitoring of Infants and Children following the Newborn Hearing Screen
Wood, S., Foley, R., et al. (2010).
In G. Sutton (Ed.), England: National Health Service Antenatal and Newborn Screening Programmes, 15 pages.Added: July 2012
This guideline provides recommendations on the early identification, surveillance, and monitoring of children with permanent hearing loss in the neonatal period.
- Children with microtia/atresia (no patent ear canal in one or both ears) or neonatal Meningitis (confirmed or strongly suspected bacterial meningitis or septicaemia) should not receive a newborn screen. “Screening is inappropriate for these babies because those with microtia/atresia will always have a degree of loss, and the risk of SNHL following bacterial meningitis is very high…These children should be immediately referred by the Paediatrician to Audiology and given an early hearing assessment (usually ABR) within 4 weeks of discharge from hospital” (p. 7).
- The following groups require targeted follow-up and should be seen for behavioral testing at 8 months of age:
- Those that declined or did not complete the newborn screen or follow-up.
- Children with conditions associated with hearing loss including syndromes (e.g., Down’s, Turner’s), cleft palate, or other cranio-facial abnormalities.
- Children with high-risk factors for late-onset or progressive deafness specifically family history, severe jaundice, mechanical ventilation or ECMO, prolonged NICU stay with clear ABR response but no OAE response, or neuro-degenerative/developmental disorders.
- Children with suspected or confirmed A155G mitochondrial mutation who have received ototoxic drugs or those who have received ototoxic drugs outside of the therapeutic range.
American Academy of Pediatrics
Hearing Assessment in Infants and Children: Recommendations Beyond Neonatal Screening
Harlor, A., Bower, C., et al. (2009).
Pediatrics, 124(4), 1252-1263.Added: July 2012
This guideline provides recommendations for the early identification of hearing loss in young children. The target audience of this guideline is pediatricians but many key recommendations are applicable for audiologists serving this population.
Children with one or more identified risk factors should have “ongoing developmentally appropriate hearing screening and at least one diagnostic audiology assessment by 24-30 months of age” (p. 1252).
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.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 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.
Dutch Cochlear Implant Group (CI-ON, Cochlear Implant Overleg Nederland)
Dutch Cochlear Implant Group (CI-ON) Consensus Protocol on Postmeningitis Hearing Evaluation and Treatment
Merkus, P., Free, R. H., et al. (2010).
Otol Neurotol, 31(8), 1281-1286.Added: July 2012
This guideline provides recommendations for the assessment, monitoring, and management of hearing loss secondary to meningitis. The target audience of this guideline is physicians and audiologists.
Post meningitis treatment, children should receive an otoacoustic emissions (OAE) examination. Auditory brainstem response (ABR) should be performed within 2 weeks after initial OAE. Follow-up should continue with OAE screening (at a minimum) at 1, 2, 6, and 12 months post first hearing test. In case of sensorineural hearing loss progression, further treatment options, including cochlear implants, should be considered.
No information was found pertaining to client/patient/caregiver perspectives.
Use the images below to navigate to other sections of the Permanent Childhood Hearing Loss evidence map.