Summary
Hearing loss in children can be present at birth or acquired and can lead to abnormal pediatric development. Symptoms include delayed language acquisition, inappropriate response to sounds, and inattention. Due to the impact of hearing loss on development, screening for hearing loss is recommended at birth and at regular intervals throughout childhood. Children with abnormal screening or concerning features (e.g., caregiver concerns, risk factors for pediatric hearing loss) should be referred to audiology for diagnostic audiometry. Early recognition and intervention are essential for development and decreasing the risk for language deprivation. Management includes addressing modifiable causes of hearing loss, coordinating care with a multidisciplinary team, and ensuring access to amplication technologies.
Epidemiology
-
Incidence in newborns [1]
- ∼ 1 case per 1,000 newborns with severe or profound hearing loss
- 1–2 cases per 1,000 newborns with unilateral hearing loss or mild to moderate bilateral hearing loss
- Prevalence in children: ∼ 20% [1]
Epidemiological data refers to the US, unless otherwise specified.
Classification
Hearing loss in children may be classified according to:
-
Onset[1]
- Congenital hearing loss: a type of hearing loss that is present at birth and is caused by a genetic disorder, congenital infection, or structural anomaly
- Delayed-onset hearing loss: a type of hearing loss that is detected after the neonatal period and caused by progressive congenital conditions (e.g., Usher syndrome)
- Acquired hearing loss: a type of hearing loss that occurs after the neonatal period and is due to causes not present at birth (e.g., trauma, ototoxic substances)
- Type: See "Overview of hearing loss types."
- Severity: See "Classification of severity of hearing loss."
Etiology
Risk factors for pediatric hearing loss [2]
Congenital and delayed-onset hearing loss [1][3]
-
Genetic
- Family history of hearing loss
- Gene mutations
- Genetic syndromes (may be associated with craniofacial malformations), e.g.:
- Congenital
- Birth factors (e.g., premature birth, increased birth weight) [1]
-
Perinatal complications, e.g.:
- Asphyxia
- Hypoxic-ischemic encephalopathy
- Hyperbilirubinemia that requires exchange transfusion
- Neonatal meningitis
- Need for > 5 days of intensive care [2]
- Extracorporeal membrane oxygenation
- Administration of ototoxic drugs for > 5 days [2]
- Retained amniotic fluid in the middle ear space
Over 90% of children who are deaf or hard-of-hearing have parents with normal hearing. [3]
Acquired hearing loss [1][3]
- Trauma (e.g., basal skull or temporal bone fracture, noise-induced hearing loss)
- Infection, e.g.:
- Measles, mumps
- Bacterial or viral meningitis (most commonly caused by Streptococcus pneumoniae)
- Lyme disease
- Chronic otitis media with effusion (OME) [3][5]
- Use of ototoxic substances (e.g., aminoglycosides, chemotherapy)
- Autoimmune disorders (e.g., Cogan syndrome)
Clinical features
- Abnormal pediatric development (e.g., delayed language and communication milestones) [2][3]
- Inappropriate response to sounds [2][3]
- Inattention [2][3]
- Symptoms of underlying causes, e.g.: [5]
- Symptoms of OME
- Symptoms of congenital hypothyroidism
-
Features suggestive of genetic hearing loss [2][3]
- Facial asymmetry
- Malformations of the auricle or ear canal (e.g., microtia)
- Preauricular pits, cysts, or tags
- Pigmentary abnormalities (e.g., heterochromia iridis, white forelock)
- Cleft lip and/or palate
- Microcephaly
- Enlarged thyroid gland
- Visual changes
Abnormal pediatric development (e.g., in literacy, cognitive function, mood regulation) can be caused by a delay in language development or a coexisting developmental or learning disorder. [3]
Screening
General principles [2][3]
- Screen for risk factors for pediatric hearing loss and caregiver concern for hearing loss at every well-child visit.
- Risk factors or caregiver concern present: Obtain diagnostics for pediatric hearing loss. [6]
- All other children: Follow pediatric hearing screening.
- Perform screening in a quiet area and limit distractions. [2][3]
- Ensure both ear canals are free of obstruction before performing the assessment. [3]
Newborn hearing screening [3][6]
- Timing of screen: before hospital discharge [2][6]
- Method [2][3]
-
Next steps
- Screen positive
- Repeat screening a minimum of several hours later. [2]
- Second screening positive: Promptly refer for pediatric diagnostic audiometry before 3 months of age. [2]
- Screen negative: Continue age-appropriate screening. [2][3][6]
- Screen positive
Newborns with features suggestive of genetic hearing loss should have pediatric diagnostic audiometry performed by an audiologist before hospital discharge. [2]
Pediatric hearing screening [3][6]
-
Screening interval [6]
- Annually between 4–6 years of age
- At 8 years and 10 years of age
- Once between 11–14 years, 15–17 years, and 18–21 years
- Method: pure tone audiometry (preferred) or otoacoustic emissions [2][3]
-
Next steps
- Screen positive
- Risk factors for pediatric hearing loss, caregiver concern, and/or previous screening positive: Refer for pediatric diagnostic audiometry.
- All other children: Consider repeating the screening once.
- Screen negative: Continue age-appropriate screening. [6]
- Screen positive
Repeat screening may be considered for children without risk factors or concern for hearing loss. Referral to audiology is recommended if two consecutive screening results are positive. [3]
Diagnosis
Approach
- Perform a hearing test. [2][3]
- ≤ 6 months of age: automated auditory brainstem response (gold standard) and/or otoacoustic emissions
- 7 months–3 years of age: otoacoustic emissions
- ≥ 4 years: pure tone audiometry or otoacoustic emissions
- Perform an ear examination, including tympanometry and pneumatic otoscopy. [2]
- Assess for features consistent with genetic hearing loss using:
- Consider additional diagnostics for pediatric hearing loss on an individual basis.
- Refer children with positive screening, ongoing caregiver concern, and/or risk factors for pediatric hearing loss for diagnostic audiometry. [2][3]
Objective audiometry (e.g., auditory brainstem response, otoacoustic emissions) is used to evaluate children who, due to age or development issues, cannot follow directions or respond to sound stimuli during subjective audiometry (e.g., pure tone audiometry). [2][3]
Diagnostic audiometry for children [2][3][7]
A combination of the following tests is usually performed by audiologists for a complete evaluation.
- A gold standard test for hearing loss
- Pure tone audiometry: children who can actively participate in testing (usually ≥ 4 years of age)
- Automated auditory brainstem response: children who cannot actively participate in testing
- Otoacoustic emissions: to assess cochlear function and locate the pathology
- Middle ear assessment (e.g., via tympanometry)
- Acoustic reflexes to assess the middle ear and auditory brainstem pathways
- Behavioral assessment of hearing to determine hearing thresholds
- Younger children: visual or conditioned play response to sounds
- Older children: pure tone audiometry
Additional diagnostics for pediatric hearing loss [2]
-
Laboratory studies: Selection depends on risk factors for pediatric hearing loss, e.g.:
- CBC with differential
- Thyroid function tests
- Diagnostics for congenital TORCH infections
-
Electrocardiography
- Perform for all children with profound hearing loss.
- Used to assess for long QT syndrome caused by Jervell and Lange-Nielsen syndrome
-
Imaging: Consider in consultation with a specialist. [2][8]
- Indications: further evaluation for an underlying cause and/or for surgical planning
- Options: CT temporal bone, MRI head and internal auditory canal
- Genetic testing: Genetic counseling and testing are recommended for all infants who are deaf or hard-of-hearing and their families. [2]
Management
Approach [2][3]
- Treat modifiable causes of hearing loss (e.g., cerumen impaction, OME); surgical referral may be required.
- Promptly coordinate care with a multidisciplinary team, e.g.: [3]
- Otolaryngology
- Visual language resources, e.g., American Sign Language (ASL)
- Speech therapy
- Early intervention program or school-aged intervention program
- Ophthalmology to assess for visual impairment and conditions that cause dual sensory impairment
- Explore families' communication preferences.
- Caregivers can use multiple communication modalities with children. [3]
- Parents who identify as culturally Deaf may place a strong emphasis on ASL and decline interventions to correct hearing loss in their children. [9]
- Refer as needed for investigation of underlying conditions, e.g.: [2][3]
- Neurodevelopmental disorders
- Visual impairment
- Monitor for the following at well-child visits: [3]
- Age-appropriate developmental milestones [2]
- Visual changes [2][3]
Ensuring timely diagnosis and treatment of hearing loss improves critical language acquisition. Early access to spoken and/or visual language is necessary for development and to decrease the risk of language deprivation, especially for children ≤ 5 years. [2][3]
Delaying referral to an early intervention program until after diagnostic audiometry and treatment is not recommended. [2]
Hearing loss in children ≤ 4 years of age is a reportable condition to state health authorities; check local laws for guidance. [3]
Amplification technologies [2][3]
A combination of technologies may be used (e.g., hearing aids plus hearing assistive technologies). See also "Management of hearing loss in adults."
Hearing aids [2][3]
- Recommended for most children who are deaf or hard-of-hearing, as early as possible (no later than 4 months of age) [2]
- Options include:
- Air-conduction hearing aids: deliver amplified sounds into the ear canal
- Bone-conduction hearing aids: transmit sound via vibration of the skull direct to the cochlea; used if there are problems with the outer or middle ear [2]
- Children need regular reassessment from audiology as hearing loss may be progressive or impacted by middle ear effusions. [2]
Cochlear implants
- Prosthetic devices that are surgically implanted and function by electrical stimulation of the vestibulocochlear nerve (CN VIII)
- Indications
- Intact cochlea and eighth cranial nerve [2][3]
- Sensorineural hearing loss, especially if there is minimal improvement with hearing aids
- Currently approved for children:
- ≥ 9 months of age who are bilaterally deaf [3]
- ≥ 24 months of age with bilateral severe hearing loss (> 70 dB threshold) [3]
- ≥ 5 years of age with unilateral deafness or discrepant audiometric thresholds alongside reduced speech discrimination [3]
- Earlier age at implementation (younger than 1–2 years of age) is associated with better spoken language acquisition. [2]
- Children with cochlear implants are at increased risk for bacterial meningitis; ensure vaccinations are up to date. [2]
Surgical intervention
-
Interventions to restore hearing
- Myringotomy (with or without tube placement) to treat middle ear effusions [3]
- Surgical reconstruction for outer and/or middle ear malformations [2]
-
Interventions to augment hearing
- Cochlear implants
- Bone-anchored hearing aids for children ≥ 5 years of age who cannot use air-conduction hearing aids [2]
Prevention
- Approximately 60% of childhood hearing loss is preventable. [10]
- During preconception counseling: Encourage vaccination (e.g., against measles, mumps, and rubella).
- During pregnancy [10]
- Recommend preventive measures against CMV infection.
- Address risk factors for birth-related complications (e.g., prematurity, birth asphyxia).
- Avoid ototoxic substances.
- For caregivers of children, recommend:
- Vaccination (e.g., against measles, mumps, rubella, and causes of meningitis) [10]
- Noise control: Minimize loud sounds from personal audio devices and environmental noise. [10]
- Middle and outer ear care [10]
- Advise against inserting objects into the ear.
- Seek prompt treatment for otitis media.
When possible, avoid the use of ototoxic substances in pregnant individuals and children. [10]