Hearing Loss in Children

Question 1

JCIH recommendations for middle ear conditions

Answer 1

The following abnormalities can cause persistent, congenital, delayed onset or progressive HL in children

• complete head and neck exam for carniofacial anomalies (ex: abnormal pinna, EAC, ear tags & pits, and temporal bone)
• Eye exam –> signal stigmata of a syndrome that includes HL (ex: irses of diff colors, abnormal positioning)
• surveillance of ME status at all check ups
• – using pneumatic otoscopy and tympanometery
• refer children w/ persistent ME effusion lasting 3+ months for otologic evaluation

Question 2

Congenital Aural atresia and microtia: associated abnormalities

Answer 2

• low set ears rotated posterially
• mildest and common= pits and tags–> branchial clefts
• atresia or stenosis may happen in microtia, but also because of persistent CHL
• abnormal middle ear structures or fusion of the malleus and incus
• – stapes deforminty
• –absent oval and round windows
• –facial nerve absence

Question 3

Congenital Aural atresia: abnormalities

Answer 3

-absent ear canal and/or pinna malformations

Question 4

Congenital Aural atresia and microtia: treatment

Answer 4

Microtia:

Aural Atresia:
ABone conduction HA
CROS
Osseointergated devices
Atresiaplasty to create an ear canal/middle ear
Congnenital Atresia
-surgical aural atresia repair, but must consider the poorer hearing following surgery and the presence of the facial nerve

Question 5

Otitis Media and High Risk Populations

Answer 5

• indigenous populations
• countries w/ high rates of poverty
• bottle feeding and poverty related indicies (low SES, malnutrition, crowding)
• –> overcrowding in home, smoking, or cooking smoke
• -> lack of protective factors: breastfeeding, vaccines, access to preventative care, high quality day care, clean water, and hygiene practices

Question 6

Low Risk OME and AOM children criteria

Answer 6

Watchful waiting for 3 mos is recommended for low-risk child with OME because of likelihood of resolution in most children
Child is low-risk unless:
Persistent HL
Suspected or dx speech-language delay
Autism spectrum disorder
Other developmental disorder
Down Syndrome
Craniofacial anomalies associated with cognitive or speech-language delay
Visual impairment
Cleft palate
Developmental Delay

Question 7

AHRQ recommendations for treatment of OM

Answer 7

-advocate hearing testing for children
w/ OME for 3 months or longer
Are at high risk
Have language delays or speech problems
-Surgically placed tympanostomy (pressure-equalization) tubes are recommended unless findings indicate that additional procedures are needed
-Discourages population-based screening programs among low-risk children (b/c will resolve on its own) & routine treatment with antihistamines, decongestants, antibiotics, and corticosteroids
-Recommends reducing risk factors

Question 8

Medical Aspects of ANSD

Answer 8

ANSD is most common in the newborn population
-Particularly premature babies and those who have medical illness
Major Risk Factors
-Hyperbilirubinemia
-Hypoxia and accompanying -metabolic acidosis
-Exposure to antibiotics that are toxic to hair cells
Genetic causes of ANSD
-Mutations of Connexin 26 and of otoferlin
Congenital disorders such as atresia of the auditory nerve

Genetic disorders affecting the auditory nerve as part of more general peripheral and cranial nerve involvement
–E.g., Friedreich’s Ataxia
–Deficits in auditory function may present before symptoms in other sensorimotor systems (Starr et al., 1996)
Immune disorders
–E.g., Guillain-Barre syndrome–affects the proximal nerve roots and proximal portions of the auditory nerve

Question 9

Screening for ANSD

Answer 9

–Most diagnoses are made following NBHS
Programs using only OAEs will miss a high proportion of ANSD cases
Adult and early childhood-onset forms can be associated with progressive sensorimotor neuropathies

JCIH (2007) recommends that ABR be used as the screening measure, at least for babies housed in the NICU
Berlin et al. (2005) suggested that the acoustic reflex screening could be used to ID children in need of ABR assessment for screening programs using OAEs.

Question 10

ANSD Management at Diagnosis

Answer 10

1. Medical/Developmental workup (JCIH, 2007)
   - -Pediatric and developmental evaluation and hx
   - -Otologic eval with imaging (CT/MRI) of cochlear and auditory nerves and vestibular assessment
   - -Medical genetics eval
   - -Ophthalmologic assessment
   - -Communication development assessment and ongoing monitoring
   - —-Additionally, referral for neurological evaluation to assess peripheral and cranial nerve function is warranted in children with the ANSD result pattern

Question 11

ANSD: Audiological follow up

Answer 11

2. Audiologic Follow-up
   –Inability of ABR and ASSR to predict behavioral thresholds is a challenge of early management
   –Behavioral hearing thresholds can vary from normal to profound levels
   –Sound audibility doesn’t predict degree of functional disability. However, access to sound is still the basis of early intervention strategies (e.g., hearing aid fitting)
   –Visual Reinforcement Audiometry
   6 months of age onward
   —Younger or delayed children
   Behavioral observation audiometry
   —Parent observation including formal assessment tools (questionnaires)

In most cases
improvement involved children with hyperbilirubinemia or very low birth weight
occurred within the 1st year of life
Highlights the need for repeat ABR assessment and careful audiometric monitoring of infants with ANSD, particularly NICU graduates

Question 12

ANSD: Audiological Clinical Profile

Answer 12

• Sound detection thresholds
• ME muscle reflex
• Cortical AEP
• Basic Auditory Processing
• Functional Hearing

**See slides for details