Pediatric Hearing Loss 1

Question 1

Mild (or worse) unilateral or bilateral hearing loss threshold sensitivity

Answer 1

> 25dB

Question 2

Moderate (or worse) bilateral hearing loss threshold sensitivity

Answer 2

> 40dB

Question 3

Conductive hearing loss

Answer 3

Refers to impairment of the outer and/or middle ear conductive mechanism only

Question 4

Sensory hearing loss

Answer 4

Refers to damage to the cochlea (outer hair cells or outer and inner hair cells)

Question 5

Mixed hearing loss

Answer 5

Refers to the presence of both conductive and sensory impairment
*Otitis media, cleft lip

Question 6

Neural hearing loss (2)

Answer 6

1. Refers to damage to the auditory neurons (spiral ganglia) and/or the auditory branch of the 8th nerve
2. Auditory neuropathy and dysynchrony are examples of neural hearing loss
   * High frequency but difficulty distinguishing words

Question 7

Central hearing loss

Answer 7

Refers to damage to auditory structures in the brainstem, thalamo-cortex and/or cortex
*Intracranial bleeds and brain tumors

Question 8

Later onset hearing loss

Answer 8

A hearing loss that is not present at birth and the newborn hearing screening would result in ‘pass’

Question 9

Auditory neuropathy hearing loss (2)

Answer 9

1. Children with auditory neuropathy have evidence of normal cochlear function, but show impairment in the function of the auditory nerve. Functional hearing can often be quite impaired and diagnosis and treatment can be confusing and complicated
2. Will need accommodations to be in the front of the room of a class

Question 10

Risk Indicators for Hearing Loss (11)

Answer 10

1. Caregiver concern* regarding hearing, speech, language, or developmental delay.
2. Family history* of permanent childhood hearing loss.
3. Neonatal intensive care of more than 5 days or any of the following regardless of length of stay: ECMO*, assisted ventilation, exposure to ototoxic medications (gentamycin and tobramycin) or loop diuretics (furosemide), and hyperbilirubinemia with exchange transfusion.
4. In utero infections, such as CMV*, herpes, rubella, syphilis, and Toxo
5. Craniofacial anomalies, including those that involve the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies.
6. Physical findings, such as white forelock, that are associated with a syndrome known to include a sensorineural or permanent conductive hearing loss.
   * White forelock – waardenburg syndrome
7. Syndromes associated with hearing loss or progressive or late-onset hearing loss, such as neurofibromatosis, osteopetrosis, and Usher syndrome, other frequently identified syndromes include Waardenburg, Alport, Pendred, and Jervell and Lange- Nielson.
   * Alport syndrome → presents with hematuria
   * Jervell and Lange-Nielson syndrome both present with long QT syndrome
8. Neurodegenerative disorders* such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich ataxia and Charcot-Marie Tooth syndrome.
   * Hunter syndrome → coarse features, large tongue, toxic metabolites, hearing loss
   * Due to mucopolysaccharides (the toxic metabolites)
9. Culture-positive postnatal infections associated with sensorineural hearing loss*, including confirmed bacterial and viral (especially herpes viruses and varicella) meningitis. 1
10. Head trauma, especially basal skull/temporal bone fracture§ that requires hospitalization.
11. Chemotherapy

Question 11

Newborn screening before 1 month: Automated Otoacoustic Emissions (O-OAE) (4)

Answer 11

1. This test measures a response produced by the cochlea (outer hair cells) when a sound is presented to the ear.
2. To conduct the test, a tiny probe is placed just inside the baby’s ear canal and a soft click is presented, a tiny microphone measures the response produced by the baby’s ear.
3. The test is quick (about 5 to 10 minutes), painless, and may be done while the baby is sleeping or lying still.
4. Thus, OAEs reflect the status of the peripheral auditory system extending to the cochlear outer hair cells.

Question 12

Newborn screening before 1 month: Automated Auditory Brainstem Response (A-ABR) (3)

Answer 12

1. This screening test measures how the hearing nerve responds to sound
2. Clicks are presented to the ear through a probe or soft earphones, and the neural response is measured through three electrodes placed on the baby’s head.
3. Automated ABR measurements reflect the status of the peripheral auditory system, the eighth nerve, and the brainstem auditory pathway

Question 13

ABR Screening characteristics (4)

Answer 13

1. Lower fall rate
2. More sensitivite to neural loss
3. Requires electrodes
4. Less sensitive to ear canal and middle ear conditions

Question 14

OAE Screening characteristics (3)

Answer 14

1. Frequency specific
2. Less invasive
3. Faster and less expensive

Question 15

Several common reasons why a baby with normal hearing would fail the newborn hearing-screening test (3)

Answer 15

1. Vernix in the ear canal
2. Fluid in the middle ear
3. Movement and/or crying during the test

Question 16

Newborn screening (7)

Answer 16

1. If a newborn does not pass their in-hospital newborn hearing screen, the PNP should assure that the baby is rescreened before one month of age.
2. This may be done in the hospital, with an audiologist who has experience with infants and in some circumstances at the Primary Care facility.
3. Parents have found that it is helpful to be given guidance to obtain timely follow-up.
4. There is never a reason not to retest an infant who does not pass their newborn hearing screen.
5. Providing written information to parents and assisting in making the appointment can be helpful.
6. Phone follow-up is important.
7. The results of this rescreen should be confirmed with the state EHDI program

Question 17

Follow up after Failing Newborn Screen (9)

Answer 17

1. Administering a battery of audiologic tests is critical: all measures are important to the provision of appropriate audiologic follow-up.
2. Test results should be in accord; findings that lack agreement need to be further explored before the audiological diagnosis can be completed.
3. Audiologic assessment of infants and young children is an ongoing process; frequent follow- up visits are needed to accurately determine auditory status and monitor the development of auditory skills.
4. Delays between visits and in the provision of intervention should be avoided.
5. Amplification fitting should proceed as soon as the hearing loss is confirmed even when the audiological evaluation is ongoing
6. Ear canal acoustics (developmental changes in the size and impedance characteristics of the external ear) affect the acoustic characteristics of all stimuli delivered to the closed ear canal, as is the case with insert ear phones and ear mold coupling.2
7. Otitis media with effusion (OME) should be managed aggressively in infants and children with permanent hearing loss3; audiological evaluations should not be postponed however, due to the presence of OME.4
8. The Auditory Brainstem Response (ABR) Test can be done on young infants without sedation.
9. The goal is to get amplification and to get hearing aids early on for the child presenting with out hearing.
   * A lot of policies do not cover the entire payment of hearing aids, which are very expensive.

Question 18

Rescreening

Answer 18

Readmissions in the first month of life for all infants (NICU or well-baby) when there are conditions associated with potential hearing loss

• Hyperbilirubinemia requiring exchange transfusion
• Culture positive sepsis

Question 19

Current National Recommendations regarding Evaluation (3)

Answer 19

1. Provide universal screening by 1 month of age
2. Follow-up audiologic diagnostic assessment by 3 months of age
3. Initiation of or referral for appropriate early intervention by 6 months

Question 20

Comprehensive ABR, ASSR, Evaluation (5)

Answer 20

1. For pediatric audiologic screening and assessment, the most useful attribute of the ABR is the relation of the wave V threshold to hearing sensitivity. Therefore, the ABR is used widely to estimate the overall degree of hearing loss.
2. ASSR is very similar to ABR.
3. In ASSR, frequency modulated tones are delivered to the infants’ ears. Collection algorithm looks for phase locking of the neuronal potential to the modulated tones.
4. This phase locking is indicative of a true response to a stimulus. From the phase locking pattern, hearing thresholds are estimated.
5. The advantages of ASSR:
   a. Stimuli are frequency specific.
   b. Louder stimulus intensity can be delivered. Therefore, ASSR can delineate between a severe versus profound hearing loss more accurately.

Question 21

What to do at 3 months of age to confirm hearing loss (4)

Answer 21

1. If the infant does not pass the outpatient rescreen, they need to be referred to an audiologist who has experience with infants.
2. In some cases, referral to Early Intervention can facilitate further evaluations and early treatment.
3. If hearing loss is confirmed, medical and ENT evaluation should be done and HEARING AIDS should be fitted if desired.
4. Information should be confirmed with the state EHDI program and referral to Early Intervention is essential.

Question 22

Acoustic reflex test (9)

Answer 22

1. Do at 3 months
2. The acoustic reflex is an involuntary contraction of the middle ear muscles (primarily the stapedius muscle) in response to intense sound stimulation.
3. The test is completed during the measurement of middle ear function (tympanometry).
4. Acoustic reflex thresholds can be recorded in children with hearing threshold levels of 50 dB HL or better.
5. With worsening hearing sensitivity, the ART can be elevated or absent.
6. When conductive hearing loss is present, the ART may also be absent.
7. ART testing can be used in infants and children as a passive test of middle ear function and the associated neural pathways.
8. Thus, it is a useful component in the test battery for differentiating among conductive, mixed, sensory and neural forms of hearing loss.
9. Does not provide hearing sensitivity

Question 23

Identification of Pediatric Audiology facilities that can take care of Pediatric Patients

Answer 23

Early Hearing Detection and Intervention EHDI-PALS (pediatric audiology level of services) is a web-based directory and search engine developed by the CDC and NCHAM. Facilities listed in EHDI-PALS must have the appropriate equipment and be able to provide audiology services to evaluate and treat children who are younger than 5 years of age.

Question 24

Why is hearing loss a developmental emergency? (5)

Answer 24

1. Adept Language Learners
   a. Research documents that 6- to 8-month-olds learning English can successfully discriminate contrasts in a language never heard (e.g.. Hindi).
   b. By 10 to 12 months, they can no longer do so, and are sensitive only to English contrasts.
2. Newborn Hearing Screening
3. Untreated Hearing Loss
4. Quality of Life
5. Delayed in Onset of Canonical Babble

Question 25

Quality of Life (6)

Answer 25

1. The terms “mild” or “moderate” hearing loss are unfortunate, because they often convey limited impact or concern.
2. With infants, any degree of hearing loss should be a concern because of the potential impact on language learning.
3. Adults with mild hearing loss may find the condition annoying, yet only occasionally disruptive to communication.
4. However, adults with an acquired mild hearing loss have already learned language.
5. When a sound like “s” is missed, the adult can easily fill in the details and comprehend the message.
6. Eg, “We need to go to the _tore to get _ome new hoe”

Question 26

If a child has a risk factor for hearing loss…Refer children with risk factors for hearing loss for follow-up hearing assessment by offering (3)

Answer 26

1. At least 1 diagnostic audiologic assessment by 24 to 30 months of age
2. Earlier and more frequent assessment
3. Customized and individualized services based on the relative likelihood of a subsequent delayed-onset hearing loss

Question 27

If a child has a risk factor for hearing loss…Monitoring communication development

Answer 27

Monitor all infants and children, with or without risk indicators for hearing loss, for communication development

• At each well-child assessment
• Any time a caregiver expresses concern about hearing or speech

Question 28

If a child has a risk factor for hearing loss…Monitoring Developmental Outcomes: (4)

Answer 28

1. Monitor developmental outcomes of early intervention participation to ensure that services provided are appropriate for
2. Child developmental communication and learning skills
3. Family preference
4. The degree and type of hearing loss

Question 29

If a child has a risk factor for hearing loss…evaluation and referral (4)

Answer 29

1. Provide medical evaluation for all children who have confirmed permanent hearing loss
2. Provide referral to an otolaryngologist with knowledge of pediatric hearing loss and an ophthalmologist experienced in evaluating infants to assess for comorbid ophthalmic conditions and visual acuity
3. Offer a genetics consultation to families
4. Counsel and assist families at transitional points to assure educational, communication and family support services are appropriate and continuous (IDEA parts C [0 to 3 years] and B [> 3 years]).

Question 30

Auditory Neuropathy (3)

Answer 30

1. Unique set of hearing test results, including present OAE’s (suggesting normal outer hair cell function) but absent ABR findings (suggesting neural conduction dysfunction).
2. Typically, these children have poor speech perception abilities and hearing loss may be mild to profound.
3. These children vary considerably in performance and in ability to benefit from hearing aids

Question 31

Types of hearing loss (5)

Answer 31

1. Bilateral or unilateral
2. Mild, Moderate, Severe, Profound
3. Conductive
4. Sensorineural
5. Neural
   * Premature infants may have neural hearing loss (auditory neuropathy or auditory dyssynchrony) as a result of conditions of and interventions for prematurity

Question 32

Category of Hearing Decibel Level on Audiogram (5) – know these!

Answer 32

1. Normal hearing or minimal loss = 10 to 24 dB
2. Mild Loss = 25 to 40 dB
3. Moderate Loss = 40 to 70 dB
4. Severe loss = 71 to 90 dB
5. Profound loss = +91 dB

Question 33

Types of Configurations of Hearing Loss (4)

Answer 33

1. Flat, indicating equal hearing loss at all frequencies
   a. Could be damage to CN8 or auditory cortex
   b. Will be flat on otiology
2. Sloping, indicating better hearing in low frequencies, decreasing to greater hearing loss in the high frequencies
   a. High frequency loss; found in sensorineural
3. Reverse slope, or rising, indicating poorer hearing in the low frequencies, with improving thresholds in the high frequencies
   a. Conductive loss
4. U-shaped, or cookie-bite or trough-shaped, indicating better low and high frequencies with poorest thresholds in the mid-frequency range
   a. Mid frequency range hearing loss

Question 34

What is the role of the PNP in addressing a potential hearing loss in a young infant? (4)

Answer 34

1. To wait and see at well-baby checks if the infant continues to show evidence of hearing loss before referring to the audiologist, because many infants will pass the diagnostic testing following initial screening.
2. To be aware of community resources, including skilled pediatric audiologists and early interventionists with expertise in infant hearing loss so that prompt referrals and family support can be given
3. To avoid upsetting the family unnecessarily during early stages of infancy when the focus is on bonding, so waiting to discuss the possible hearing loss
4. To advise the family or parents to seek a cochlear implant for their infant as soon as the infant is 12 months old

Question 35

Important things to Remember about Later Onset Hearing Loss (7)

Answer 35

1. An infant may pass newborn hearing screening, yet still be at risk for late onset hearing loss.
2. If there is a positive history for hearing loss in a relative’s child. This risk factor should prompt careful surveillance of hearing and language.
3. A change in language status may signal hearing loss. Parental concern should prompt referral for audiological evaluation.
4. Consider the child’s language development.
5. A referral for pediatric audiology and for speech or language
6. The hearing evaluation should be completed first, because those results will influence the interpretation of subsequent speech/language evaluation.
7. Use your resources. When a child has a diagnosis of late-onset permanent hearing loss, ENT, ophthalmology, medical-genetics and early intervention services should be utilized.

Question 36

Medical Work-up (6)

Answer 36

1. An otolaryngologist (ENT) is almost always involved in the diagnostic evaluation and therapeutic management of children with a suspected or confirmed hearing loss.
2. The age of the child and the nature of the hearing loss will determine the type and extent of the medical work-up needed for a particular child.
3. The primary care provider is crucial to management of each child’s case, even after referral to the otolaryngologist.
4. A coordinated approach involving the child’s pediatrician, parent, and other health service providers should be used.
5. For instance, an infant referred from a newborn hearing screening and presenting with a confirmed bilateral severe-to-profound sensorineural hearing loss (SNHL) by auditory brainstem response testing would undergo an extensive battery of tests depending on the maternal and neonatal history and physical findings.
6. A 12-year-old with a late onset permanent conductive hearing loss would undergo a markedly different evaluation.