Universal newborn hearing screening Flashcards

1
Q

What is the incidence of permanent hearing loss?

A

1-3 per 1000 live births

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2
Q

What is normal hearing?

A

0-20dB

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3
Q

What is mild hearing loss?

A

20-40dB

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4
Q

What is moderated hearing loss?

A

40-60dB

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5
Q

What is severe hearing loss?

A

60-80dB

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6
Q

What is profound hearing loss?

A

> 80dB

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7
Q

What is the most common type of congenital hearing loss?

A

Sensorineural

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8
Q

What are the causes of neonatal sensorineural hearing loss?

A
  1. Genetic 50%
    a) Syndromic
    b) Non-syndromic (70%) - cochlear hair dysfunction because of errors in gap junction protein connexin 26 production
  2. Congenital infections
  3. Hyperbilirubinemia
  4. Ototoxic medications
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9
Q

What are risk factors for neonatal sensorineural hearing loss?

A
  1. Family history of permanent hearing loss
  2. Craniofacial abnormalities including those involving the external ear
  3. Congenital infections including bacterial meningitis, cytomegalovirus, toxoplasmosis, rubella, herpes and syphilis
  4. Physical findings consistent with an underlying syndrome associated with hearing loss
  5. Neonatal intensive care unit stay >2 days OR with any of the following regardless of the duration of stay:
    • Extracorporeal membrane oxygenation
    • Assisted ventilation
    • Ototoxic drug use
    • Hyperbilirubinemia requiring exchange transfusion
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10
Q

What is the average age of diagnosis of hearing loss in unscreened children?

A

~24mo

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11
Q

What are the functional outcomes of delayed diagnosis and intervention?

A

Irreversible deficits in communication, psychosocial skills, cognition, and literacy

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12
Q

What tests are used to screen newborns for hearing loss?

A
  1. Otoacoustic emission (OAE)

2. Automated auditory brainstem response (AABR)

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13
Q

What are the characteristics of the OAE?

A
  1. Performed by a trained technician; 10–15 min; portable equipment brought to the bedside
  2. Screening best performed in infants older than 24 h, with a minimum 34 weeks’ corrected gestational age
  3. Results may be affected by the infant’s movements, environmental noise or dysfunction in the middle or external ear (eg, debris in the external ear canal)
  4. Noninvasive; ear probe placed in outer ear canal
  5. Ear-specific testing; both ears can be tested simultaneously
  6. Identifies conductive and cochlear hearing loss from the level of the external ear to the level of the outer hair cells in the cochlea
  7. Screening thresholds set to detect at least moderate hearing loss (30–40 dB)
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14
Q

What are the characteristics of the AABR?

A
  1. Performed by a trained technician; 15-20 min; portable equipment brought to the bedside
  2. Screening best performed in infants older than 24 h, with a minimum 34 weeks’ corrected gestational age
  3. Results may be affected by the infant’s movements, environmental noise or dysfunction in the middle or external ear (eg, debris in the external ear canal)
  4. Noninvasive; three electrodes taped to the head, earphones or ear probes placed on or in the infant’s ears
  5. Ear-specific testing; both ears can be tested simultaneously
  6. Identifies conductive, cochlear, and neural hearing loss from the level of the external ear to the level of the brainstem, including assessment of vestibular (8th) nerve function
  7. Screening thresholds set to detect at least moderate hearing loss (30–40 dB)
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15
Q

What form of hearing loss can only be detected by AABR?

A

auditory neuropathy

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16
Q

What is the recommended screening test?

A
  1. Two step screening:
    OAE 1st in newborns with no risk factors, then AABR if fail OAE
  2. AABR in infants with any risk factors or NICU
17
Q

What is the effectiveness of earlier intervention?

A

Infants who are diagnosed and receive intervention<6mo score 20-40 percentile points higher on school related measures then those diagnosed later

18
Q

Who should be involved in the management of children with hearing loss?

A
  1. Family MD
  2. Pediatricians
  3. Audiologists
  4. ENT
  5. SLP
  6. Educational specialists
19
Q

What are the intervention strategies for hearing loss?

A
  1. Medical/surgical: hearing aids, cochlear implants, bone-anchored hearing aids, brainstem-implanted auditory devices, surgical options for ear malformations, ossicular chain abnormalities, tumors, cholesteatomas
  2. Educational/habilitation strategies: oral and gestural communication
20
Q

What is a cochlear implant?

A

An electronic device surgically placed in the cochlea to provide stimulation to the auditory nerve

21
Q

What are the recommendations for cochlear implant in eligible children?

A

Bilateral implantation between 8-12mo coupled with auditory oral therapy

22
Q

What is the false positive rates for universal newborn hearing screening programs?

A

2-4% usually

0.5-1% in well-established programs

23
Q

Which populations with hearing loss will not be detected by universal newborn hearing screening?

A
  1. Less severe congenital hearing loss <30-40dB
  2. Progressive or late-onset hearing impairment e.g. CMV
  3. Auditory neuropathy if using OAE
24
Q

Is UNHS considered cost-effective?

A

Yes

25
Q

What should be assessed in children with confirmed hearing loss?

A
  1. Detailed family history
  2. Detailed history and physical examination for comorbidities and/or syndromic
  3. Consult peds ENT, ophtho, and genetics
  4. Vision screening (r/o Usher syndrome)
  5. Neuroimaging, genetic, renal and cardiac evaluation as indicated
26
Q

What does the CPS recommend regarding newborn hearing?

A

Hearing screening should be provided universally to all Canadian newborns via a comprehensive and linked system of screening, diagnosis and intervention.