Universal Hearing Screen

Question 1

Who does the CPS recommend hearing screening for?

Answer 1

All newborns

Question 2

What is the incidence of congenital hearing loss?

Answer 2

1/3000 births

Question 3

How is hearing loss defined?

Answer 3

Degrees of loss of auditory sense of frequencies between 125 (low) to 8000 (high) Hz

Question 4

What kind of hearing loss is most common in neonates?

Answer 4

Sensorineural hearing loss

Question 5

What is the most common risk factor for sensorineural hearing loss in neonates?

Answer 5

Genetic causes and family history* seen in 50% of cases

Question 6

List 3 risk factors for sensorineural hearing loss

Answer 6

1. Family history or genetic cause** 50% of cases
2. Craniofacial abnormalities involving the outer ear (ie: Treacher Collins)
3. Congenital infection such as CMV and bacterial meningitis
4. Hyperbilirubinemia
5. NICU stay requiring ECMO, prolonged ventilation, exchange transfusion
6. Ototoxic medications
7. Physical exam consistent with underlying syndrome associated with hearing loss

Question 7

What is the abnormality in genetic sensorineural hearing loss?

Answer 7

Cochlear hair cell dysfunction due to errors in production of gap junction connexin 26

Question 8

When does hearing loss get detected with and without a universal hearing screening?

Answer 8

• WITHOUT - when language delay is present
• Usually will see expressive language delay by 1 year
• Age of diagnosis WITHOUT = 2 years
• Age of diagnosis WITH = 3 months

Question 9

Why do we need universal screening if we can just screen for risk factors?

Answer 9

• Because many children with SNHL do NOT have risk factors and therefore are missed
  Early identification = early intervention
  Improvement in timing of diagnosis from 17 months to 10 weeks

Question 10

List three consequences of delayed diagnosis and intervention to hearing loss?

Answer 10

HEARING IS CRITICAL to DEVELOPMENT!

1. Hearing, speech, reading and writing are closely correlated
2. May result in irreversible deficits in communication, psychosocial skills, cognition, and literacy
3. Lower reading level- high school graduates with hearing loss often have reading level at 9-10 years
4. Low academic achievement and lower literacy results in underemployment, maladaption, psychological distress

Question 11

What are two tests that can be done to screen for hearing loss?

Answer 11

OAE- otoacoustic emissions

AABR- automated auditory brainstem response

Question 12

How does the OAE test work?

Answer 12

Both ears at the same time; sound sent to auditory system and returning emission from outer hair cells detected by probe which is placed in the outer ear canal

Assessment of external ear to outer hair cells of cochlea

Question 13

What are the criteria to eligible for OAE?

Answer 13

> 24 h of age

>34 GA

Question 14

How does AABR test work?

Answer 14

Measures brainstem electrical activity in response to sound
Place 3 electrodes on/in infants ears to identify conductive, cochlear, and neural hearing loss

Assessment of external ear to brainstem

Question 15

What is the normal sequence of testing?

Answer 15

• All babies get OAE –> if abnormal then AABR

- Direct to AABR if any risk factors are present

Question 16

What are the criteria to eligible for AABR?

Answer 16

> 24 h

>34 GA

Question 17

What threshold of hearing loss will OAE and AABR detect?

Answer 17

At least moderate hearing loss

30-40 dB

Question 18

What is the impact of early intervention?

Answer 18

Improved expressive and receptive language scores

If identified prior to 6 months, 20-40 percentiles higher on school related measures

Question 19

What are adverse effects of screening?

Answer 19

Parental anxiety

False positives 0.5-4%

Question 20

Who is hearing screening recommended for?

Answer 20

ALL children with developmental or learning problems

Question 21

List three limitations of screening

Answer 21

Less effective at detecting SEVERE hearing loss <30-40 dB
Progressive or late onset hearing impairment not detected by NBS
Hearing assessment recommended for all children with development or learning problems

Question 22

Who should be on the medical team for child with suspected hearing loss?

Answer 22

Family physician
Pediatrician 
Audiologist
ENT 
SLP
Education specialist

Question 23

What is the role of the physician in these cases?

Answer 23

Advocate for timely followup and parental compliance if positive or equivocal results
Medical evaluation for comorbidities/syndromes
Early referral to ENT, optho*, or genetics if concerns are present
Awareness of increased OM and meningitis in children with hearing loss

*visual assessment is important to maximize sensory input and determine if there is an underlying genetic syndrome

Question 24

What are some audiologic interventions for hearing loss?

Answer 24

Hearing aids

FM and wireless devices

Question 25

What are some medical/surgical interventions for hearing loss?

Answer 25

Cochlear implant*
Bone anchored hearing device
Brainstem auditory devices

Question 26

What are some rehabilitation interventions for hearing loss?

Answer 26

Auditory oral therapy (oral language habilitation)
Auditory-verbal therapists
Teachers of the deaf
Speech therapy

Question 27

What are some educational interventions for hearing loss?

Answer 27

Parent/caregiver involvement

Daily exposure to language

Question 28

What is the primary main intervention for hearing loss?

Answer 28

Cochlear implants by 8-12 months and oral language therapy