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Characteristics of Congenital Hearing Loss

Explore outcomes of Universal Newborn Hearing Screening (UNHS) in a metropolitan nursery, identifying types and degrees of hearing loss in newborns and tracking the progression of these losses over the initial months/years. This study presents data from the UNHS program at Massachusetts General Hospital, highlighting the screening protocols used and statistical analysis of hearing loss cases identified. The flow of UNHS in Massachusetts and detailed statistics from the program are provided, shedding light on the incidence and classification of hearing loss in newborns. The study delves into the degree of hearing loss, the distribution of conductive and sensorineural hearing losses, and the outcomes of follow-up evaluations, offering insight into the management and resolution of different types of hearing loss in infants.

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Characteristics of Congenital Hearing Loss

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  1. Characteristics of Congenital Hearing Loss Barbara S. Herrmann, Ph.D. CCC-A Audiology Department Massachusetts Eye and Ear Infirmary Harvard Medical School

  2. Examine outcomes of UNHS For one metropolitan nursery: • What types/degrees of hearing loss are being identified in newborns referred by UNHS • What happens to those hearing losses over the first few months/years

  3. My UNHS Background:

  4. Data presented: • From UNHS program at Massachusetts General Hospital • Initiated NICU screening in 1981, UNHS in 1997 • Follow-up evaluation at Massachusetts Eye and Ear Infirmary Audiology Department scheduled within 1 to 3 weeks of hospital discharge • If hearing loss, usually next evaluation at 4 months of age

  5. UNHS Flow in Massachusetts Discharge Notify Parent Pediatrician Pass Hearing Screen Audiologist Supervise Notify Parent Pediatrician Make F/U Appt Keep F/U Appt Refer EBC Phone Call to Parent Pass DPH DPH Parent Liason Refer

  6. 5 Critical Elements for Success 1 Discharge Notify Parent Pediatrician Pass Hearing Screen Audiologist Supervise 2 Notify Parent Pediatrician Make F/U Appt Keep F/U Appt Refer EBC 3 Phone Call to Parent Pass 4 DPH DPH Parent Liason Refer

  7. Screening Protocol at MGH • ALGO Infant Hearing Screeners (ALGO I to ALGO 3i) • Audiologists or Technicians • 35 dB HL shaped-click at 37/sec over 25 msec analysis time. • Custom ear couplers. • Electrode montage; Vertex (+), Nape (-), Forehead (ground). • Single Screen

  8. All MGH Nursery: 2000 to 2005N=21,613 babies

  9. MGH Statistics by Nursery Level MGH Level 1 N=19,252 MGH Level 3 N=1,371

  10. Follow-up between 1 and 4 weeks after discharge

  11. AER Estimated Audiogram Bone-Conduction Left Ear Threshold Right Ear Threshold

  12. First follow-up test N=393 of 455 refers (12% transferred or lost to follow-up) Normal hearing = AER thresholds of 20 dB HL or better

  13. First follow-up by refer type Bilateral Refers Unilateral Refers

  14. Type of Neonatal Hearing Loss Percent of Babies with Hearing Loss ( N=135) 60% bilateral loss; 40% unilateral loss Incidence of Hearing Loss overall - 0.6% Incidence of sensorineural loss – 0.15%

  15. Degree of Hearing loss: • Classified by threshold at 2000 Hz toneburst • Classification rules: • Normal: 20 dB HL or better at 2000 and 4000 Hz • Borderline: 25 db HL • Mild: 30 to 40 dB HL • Moderate: 45 dB to 65 dB HL • Severe: 75 to 85 dB HL • Profound: 90 dB HL or greater

  16. No response at 102 dB HL Range of thresholds for each degree Borderline Mild Profound Moderate Severe

  17. Degree of Hearing Loss: all loss ears 23% 28% 32% 5% 12% N=215 ears 51% of refers have 2000 Hz thresholds between 25 and 40 dB HL

  18. Degree of Hearing Loss: by type 20% 20% 21% 2% 12% 2% 10% 7% 3% 3% 65% conductive/unknown type 35% known sensorineural component N=215 ears

  19. Further details: • Unknown or type not determined • Conductive hearing losses • Sensorineural / mixed hearing loss • Insight into current issues

  20. Unknown/ Type not determined

  21. Degree of loss for Unknown/ Type not determined 56% 20% 18% 3% 3% N=66 ears

  22. Right ear Left ear Primarily symmetric

  23. Conductive hearing loss Right masked bone

  24. Conductive hearing loss Conductive hearing loss 10% 46% 41% 3% N=71 ears

  25. Again, primarily symmetric Right ear Left ear

  26. Conductive and Unknown ears similar, so have grouped them 20% 20% 21% 2% 12% 2% 10% 7% 3% 3% 65% of all loss are conductive or unknown origin: 41% of those are mild or better

  27. What have we learned • About 60% of ears with conductive or type not determined hearing loss identified within 1 month of newborn screen have mild or borderline degree of loss. • Most of the babies have similar degrees of loss in each ear

  28. Sensorineural / Mixed Losses

  29. 20% 36% 8% 9% 28% N=76 ears Sensorineural / Mixed Loss Ears

  30. Again, mostly symmetric

  31. What have we learned • 66% of ears with sensorineural hearing loss identified within 1 month of newborn screen have moderate hearing losses or better. • 30% of sensorineural hearing loss identified by UNHS have hearing thresholds better than 40 dB HL

  32. What happens over time…. • Infants scheduled for another AER evaluation three months after initial test • After that usually seen at 3 month intervals using behavioral audiometry • Many with borderline hearing do not keep appointment • Have re-evaluation data for 65 of 135 infants

  33. Sensorineural / Mixed Losses 46% 82% 26% 22% 7% 9% 9% N=65 babies Most conductive losses resolve to normal within 3 months Most sensorineural loss do not change

  34. Questions that have arisen in UNHS • Are we identifying mild losses with ABR? • What about unilateral refers with bilateral loss? • Will any screening technique catch all losses? • Should we follow unilateral refers?

  35. 20% 36% 8% 9% 28% N=76 ears Are we identifying mild losses with ABR?

  36. Yes Bilateral Refer Bilateral Refer First follow-up at 2 days, now 18 months hearing stable First follow-up at 3 days, now 7 years hearing stable

  37. What about unilateral refers with bilateral loss? 3 cases of sensorineural/mixed loss on first follow-up in this cohort

  38. No response left ear at 85 dB HL Case 1 and Case 2 Identified at 3 weeks, now 3 years old Hearing stable Identified at 6 weeks, now 12 months Hearing stable

  39. Case 3 Follow-up AER First AER Which was correct – the screen or first follow-up bone?

  40. Will any screening technique catch all losses? ABR bilateral refers; present OAEs

  41. First follow-up by refer type Should we follow unilateral refers? Bilateral Refers Unilateral Refers

  42. Yes Unilateral Refer Age 6 months First Behavioral Age 1 year

  43. YES Age 2.6 years Age 3.5 years

  44. Things to keep in mind….

  45. Fluctuating conductive always an issue in follow-up studies 3 weeks of age 5 months of age

  46. Fluctuating conductive always an issue in follow-up studies 5 months of age 8 months of age

  47. Fluctuating conductive always an issue in follow-up studies

  48. Lots of confusion over earphones….. Voss and Herrmann 2005 ALGO ear coupler has the least variation from baby to baby than any earphone

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