A resident was seeing children in his continuity clinic. He had noticed that several of his patients had failed newborn hearing screenings at birth, but were normal when repeated a couple weeks later. He asked how good the testing was. His attending said she didn’t know the exact numbers but that universal screening had been occurring for several years and “seemed to have pretty good results.” She also remarked that vision was to also be screened but again she couldn’t give any specific numbers. The attending told the resident that in addition to observing the child for any vision or hearing problems, and specific screening, she always tried to ask the family if they or anyone else had any concerns about hearing or vision and always would refer if anyone had any concerns. She said, “I know that it is not the best test, but a positive answer means the child needs further testing.”
Sensory problems in children like any problem should try to be identified as early as possible so effective treatment plans can be carried out.
About 1-4 children/1000 newborn infants have hearing loss or about 8-16,000 infants/year in the US. Before universal hearing screening the average age for detection was 2 1/2 -3 years of age.
In 2010, an evaluation of the universal newborn hearing screening programs found that about 92% of infants were screened before discharge with 4% failing the before discharge screening. Unfortanately only 2% were referred for a diagnostic evaluation. The authors cite multiple barriers to universal screening and followup. Types of hearing testing for screening includes evolked otoacoustic emission testing, auditory brainstem response or both.
Amblyopia is a “functional reduction in visual acuity characterized by abnormal processing of visual images, which is established by the brain during a critical period of vision development.” Strabismus is the most common cause of amblyopia and is an ocular misalignment. Asigmatism or blurred vision at any distance, hyperopia or farsightedness, and anisometropia or an asymmetric refractive error between the eyes, are also risk factors for amblyopia. 2-5% of preschool children have amblyopia. Highly effective treatment includes eyeglasses, patching and cycloplegic medications. Common standard tests are the HOTV visual acuity test, Random Dot E steroacuity test, cover-uncover visual alignment test, and fundus red reflex testing. Photoscreening, which detects ocular alignment and refractive blurring, and autorefraction techniques which automatically screens for refractive error are also commonly used.
In one study of 300 infants, a sensitivity of 100% and specificity of 99.7% was achieved with an overall referral rate of 2.0% was found hearing screening. The states of Colorado, Mississippi, Rhode Island, and Texas have reported referral rates of 3-10% and a false positive rates of 3.6%. These are well around the NIH guidelines for hearing screening programs of failure rates of 5-7%.
The US Preventive Services Task Force (USPSTF) found that of the studies they evaluated “… none of the tests was associated consistently with both high sensitivity and high specificity (ie, > 90%) for specific amblyogenic risk factors.” The testing included visual acuity, steroacuity, ocular alignment, photoscreening and autorefractors. The USPSTF recommends screening at 3-5 years but says there is insufficient evidence before age 3 for universal screening . In the USPSTF statement the tests evaluated included HOTV, Random Dot E, cover-uncover, photoscreening, and autorefraction techniques.
The Children’s Eye Foundation cites photoscreening and autorefraction studies with generally high rates of being able to do the screening (97-99% screenability) with sensitivities in the 45-70% range with a specificity of ~98%. Referral rates were 5-10% for further studies.
Some authors critical of the omission of the USPSTF screening recommendations for screening in children < 3 years of age, point out that fundus red reflex examination is a standard screening test that can help to detect congenital cataracts, other causes of deprivation amblyopia, and retinoblastoma. They go on to point out some newer studies demonstrate good positive predictive values and urge use of photoscreening and autorefraction in < 3 year olds. One study they cite showed photoscreening to have higher positive predictive values in 3-4 year old children than testing with HOTV monoular distance acuity testing and Random Dot E testing for steopsis.
Questions for Further Discussion
1. What type of hearing screening are performed at your local institutions?
2. What is your hearing screening failure rate?
3. What visual screening tests do you perform and at what ages?
4. What is your referral rate for failed visual screening?
- Disease: Health Screening | Vision Impairment and Blindness | Hearing Problems in Children | Newborn Screening
- Symptom/Presentation: Abnormal Laboratory Test | Health Maintenance and Disease Prevention | Hearing Problem
- Specialty: Ophthalmology | Otolaryngology
- Age: Preschooler
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Health Screening, Vision Impairment and Blindness and Hearing Problems in Children.
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
Schmidt P, Baumritter A, Ciner E, Cyert L, Dobson V, Haas B, Kulp MT, Maguire M, Moore B, Orel-Bixler D, Quinn G, Redford M, Schultz J, Ying GS. Predictive value of photoscreening and traditional screening of preschool children. J AAPOS. 2006 Aug;10(4):377-8; author reply 378-9.
Shulman S, Besculides M, Saltzman A, Ireys H, White KR, Forsman I. Evaluation of the universal newborn hearing screening and intervention program. Pediatrics. 2010 Aug;126 Suppl 1:S19-27.
Delaney, AM, Meyers AD. Newborn Hearing Screening. Medscape.
Available from the Internet at http://emedicine.medscape.com/article/836646-overview#a1 (rev. 06/15/2010, cited 9/26/2011).
US Preventive Services Task Force. Vision screening for children 1 to 5 years of age: US Preventive Services Task Force Recommendation statement. Pediatrics. 2011 Feb;127(2):340-6.
Donahue SP, Ruben JB; American Academy of Ophthalmology; American Academy of Pediatrics, Ophthalmology Section; American Association for Pediatric Ophthalmology and Strabismus; Children’s Eye Foundation; American Association of Certified Orthoptists. Pediatrics. 2011 Mar;127(3):569-70.
Children’s Eye Foundation. Types of Vision Screening Devices.
Available from the Internet at http://www.childrenseyefoundation.org/index.php/Programs/types-of-vision-screening-devices and http://www.childrenseyefoundation.org/index.php/Programs/types-of-vision-screening-devices-continued (rev. 2011, cited 9/26/2011).
ACGME Competencies Highlighted by Case
12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
16. Learning of students and other health care professionals is facilitated.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital