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,
- perinatal factors?
- 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