Hearing Loss in Children Flashcards
What are the most common/treatable reasons for CHL?
- Impacted cerumen
- Otitis media with effusion
What are the Joint Committee on Infant Hearing’s recommendations for middle ear conditions?
- Complete head/neck exam for craniofacial anomalies
- Pneumatic otoscopy
- Tympanometry
- Referral of children with persistent OME lasting >3 months for otologic evaluation
Describe craniofacial anomalies that could be indicative of pediatric HL.
-Need to inspect pinna for size, shape, landmarks, and position on the head
- Ear tags/pits
- Low set ears
- Classification of microtia severity
Describe Grade 1 of microtia severity?
-Pinna is smaller than normal but EAC is patent, all normal ear structures are present
Which is more common: atresia/EAC stenosis or microtia?
-Atresia/EAC stenosis
What other abnormalities of the ME structures also frequently occur with microtia?
- Stapes deformity
- Absence of oval and round windows
- Facial nerve absence
- Anomalous development
- Poor pneumatization of the middle ear cells & space
- Fusion of the malleus and incus
Describe treatment options for congenital aural atresia.
- Bone conduction HAs
- CROS
- Osseointegrated devices
- Atresiaplasty to create an ear canal/middle ear
What populations are high risk for OM/OME?
- Down syndrome
- Cleft lip and palate
- Indigenous populations (i.e. Alaskan inuit, Australian aboriginals)
- Countries with high poverty rates
- Bottle feeding
What ENT problems are often associated with Down syndrome?
- Stenotic ear canal
- Eustachian tube dysfunction
What Eustachian tube dysfunctions are associated with cleft lip/palate?
- ET obstruction
- Narrow opening
- Abnormal control
What should families be encouraged to do to reduce risk of chronic OM?
- Breastfeed as long as possible
- Place baby supine to sleep
- Advise parents not to smoke
- Avoid large group child care
- Wash their hands and children’s hands before eating
What are the Agency for Healthcare Research and Quality (AHRQ) recommendations for the treatment of OME & AOM?
- Watchful waiting for 3 months is recommended for low-risk child with OME because of likelihood of resolution in most children
- Reduce risk factor
- Medication if still not resolved
- Surgically-placed tympanostomy (PE) tubes if medication is unsuccessful
What would make a child at-risk for OME & AOM?
- ASD
- Cleft palate
- Craniofacial anomalies
- Developmental disorder/delay
- Down syndrome
- Persistent HL
- Speech-language delay
- Visual impairment
Describe the medical aspects of ANSD.
- Hyperbillirubinemia
- Hypoxia and accompanying metabolic acidosis
- Genetic mutations of Connexin 26 and otoferlin
- Congenital disorders, i.e. atresia of the auditory nerve
- Immune disorders (i.e. Guillain-Barre syndrome)
Describe the screening process for ANSD.
- JCIH recommends using ABR, at least for babies in the NICU
- Programs using only OAEs will miss a high proportion of ANSD
- Suggested that ARTs could be used to ID children in need of ABR assessment for screening programs using OAEs
Describe ANSD management at diagnosis.
MEDICAL/DEVELOPMENTAL WORK-UP
- Otologic eval with imaging (CT/MRI) of cochlea and auditory nerves and vestibular assessment - Medical Genetic eval - Ophthamlogic assessment - Communication development assessment and ongoing monitoring - Referral for neurological evaluation to assess peripheral and cranial nerve function
Describe ANSD audiologic follow-up.
- Required when ABR and ASSR are unable to predict behavioral thresholds (can vary from normal to profound levels)
- VRA (6 months+)
- Behavioral audiometry (up to 6 months)
- Parent observation including formal assessment (questionnaires)
Describe the ANSD audiological clinical profile: sound detection thresholds.
- Some show audios within normal range
- Most (>90%) show impaired sound detection (typically symmetrical)
- Unilateral ANSD most commonly associated with auditory nerve hypoplasia (cochlear nerve deficiency)
- Reverse slope configuration is common
- Fluctuations are common
Describe the ANSD audiological clinical profile: middle ear muscle reflex.
- Modulated by the IHCs, CN VIII, and auditory brainstem pathways
- Consistently abnormal in cases of ANSD regardless of behavioral hearing levels
Describe the ANSD audiological clinical profile: CAEPs.
- Despite absence or abnormality of ABR,
- May reflect the fact that they are less dependent on synchronous neural firing than ABR
Describe the ANSD audiological clinical profile: basic auditory processing.
- Distortion of neural firing patterns results in impaired temporal resolution
- Frequency resolution and intensity discrimination are usually unaffected because they depend on cochlear processing
Describe the ANSD audiological clinical profile: functional hearing.
- Speech perception difficulty
- Higher degrees of everyday listening and communication difficulty than matched controls
Describe Grade 2 of microtia severity?
-External ear is malformed, EAC is present but stenotic, ME space is small, ossicles are malformed/fused
Describe Grade 3 of microtia severity?
-Severe malformation of the pinna, absence/complete stenosis of EAC, absence/near absence of ME space and ossicles
Describe Grade 4 of microtia severity?
-Anotia (total absence of ear canal)
What are some factors of at-risk populations for OM/OME?
- Overcrowding in the home
- Increased smoking rates, cooking smoke in the home
- Pacifier use
- Large daycare settings
- Infants sleeping face-down
- Lack of protective factors: breastfeeding, vaccines, access to preventative care, high-quality daycare, clean water, hygiene practices
