Medical Evaluation and Medical Management of Hearing Loss in Children

Key Points

  • Proper medical diagnosis of hearing loss in children is predicated upon a thorough history, good physical examination and proper imaging studies
  • Most congenital hearing losses are hereditary
  • Constant vigilance by parents, pediatricians, and teachers is necessary because many hearing losses develop later in childhood
  • Sensorineural hearing loss (SNHL) is the most common birth disorder in the United States
  • Severe to profound SNHL occurs in one to two children per thousand live births and another two to three per thousand babies are born with partial hearing loss
  • The cumulative incidence of otitis media with effusion, associated with conductive hearing loss is 80% by the age of 4 years
  • The proper identification and medical management of these children with hearing loss is a priority if speech and language deficits are to be minimized and progressive otologic disease avoided
  • The most important aspect of managing a child with hearing loss is early identification
  • Universal newborn hearing screening allows for the early identification of most children with hearing loss
  • Constant vigilance by parents, pediatricians and teachers is necessary because many hearing losses develop later in childhood
  • If a child passes a newborn hearing screening but there is a family history of hearing loss, that child must be repeatedly tested over time
  • Neonatal hearing loss may be syndromic or non syndromic
    • syndromic hearing loss occurs in association with other clinical features, though they are not exclusively hereditary in nature
    • 70% of hereditary hearing loss is non syndromic
  • The most common cause of hereditary hearing loss is mutations in connexin 26, which accounts for up to 80% of autosomal recessive nonsyndromic hearing loss.
    • The hearing loss with connexin 26 mutations can be of any degree and these children are otherwise healthy and normal

The Medical Evaluation of the Newly Identified Child with SNHL


  • The history is the most important piece in identifying the etiology of the hearing loss
  • Birth History
    • perinatal factors?
      • prematurity, fetal distress, pregnancy related illness, drug treatments, low birth weight,
  • Family History
    • genetic suggestion?
  • Medical History
    • known history of cytomegalovirus (CMV), herpes or syphillis?
    • perinatal meningitis
  • Risk Factors
    • family history of hearing loss and genetic disorders
    • caregiver concern regarding hearing, speech, language or developmental delay
    • infants admitted to the neonatal intensive care unit
    • in-utero infections such as CMV, herpes, rubella, syphillis and toxoplasmosis
    • craniofacial anomalies
    • physical findings such as a white forelock or atresias
    • syndromes associated with hearing loss or late-onset or progressive hearing loss
    • neurodegenerative disorders
    • culture positive postnatal infections associated with sensorineural hearing loss
    • serious head trauma
    • chemotherapy or cancer
    • pediatricians should arrange for routine hearing evaluations of children who have these risk factors to ensure that no late-onset hearing loss is missed

Physical Examination