External Ear Canal Stenosis (Narrow Ear Canals)

A child with Down syndrome who has narrow ear canals should see an ENT specialist every three months to avoid undiagnosed and untreated ear infections. Cleaning of the ear canals by an ENT specialist is often necessary to ensure proper examination and diagnosis. The problem may resolve after age three.

Narrow Ear Canals

Narrow ear canals can occur in 40% to 50% of infants with Down Syndrome. Children with this condition should see an ENT specialist every three months to avoid serious problems.

Chronic Ear Infections

Children with Down syndrome have an increased incidence of upper repertory tract infections, which can lead to chronic ear infections. The facial anatomy of Down syndrome also increases the likelihood of chronic ear disease. Some children may need repeated placement of pressure equalization (PE) tubes to eliminate chronic infections.

Chronic Ear Infections

Increased incidence of upper respiratory tract infections and Down syndrome facial anatomy often lead to chronic ear disease. Monitoring and treatment is critical because there is a high rate of underdiagnosis and undertreatment of ear infections in children with Down syndrome.

Hearing Loss

Hearing loss will affect many people with Down syndrome at some point in their lives. This may be mild temporary hearing loss or a longer-term problem with hearing. Factors that may cause higher levels of hearing loss in people with Down syndrome include:

• Increased incidence of chronic ear diseases
• Differences in the structure of the ear
• Weaker immune systems.

Types of hearing loss can include one or both of the following:

• Conductive hearing loss
  • Typically a temporary problem where sounds cannot pass freely into the inner ear
  • In children, this may be caused by fluid in the space behind the eardrum (some times called glue ear)
  • If glue ear is identified, there should be a watchful period of three months before beginning active treatment to allow for spontaneous improvement
• Sensorineural hearing loss
  • A permanent hearing problem caused by damage in the inner ear

 

Hearing loss in children can affect educational, language-related, and emotional development. The American Academy of Pediatrics and the Down Syndrome Medical Interest Group recommend audiologic testing at birth and every six months up to age three, or until the child can cooperate for an audiogram that includes ear-specific testing. After age three, children with Down syndrome should have an annual hearing test. Adults with Down syndrome are also at high risk for conductive hearing loss. They can have frequent earwax impactions that may impair hearing. Routine ear examinations can assess wax impactions and periodic screening with an audiologist can formally assess hearing loss. Undiagnosed hearing loss is frequently mistaken for stubbornness, confusion, or disorientation in adults with Down syndrome and can be greatly improved with hearing aids, ear cleanings, and environmental adaptations.

Hearing Loss

Hearing loss in children can affect educational, language-related, and emotional development. Audiologic testing of children with Down syndrome at birth and every six months up to age three is recommended. Annual hearing tests are recommended after age three. Frequent earwax impactions may impair hearing and routine ear examinations should be performed.

Airway Obstruction and Sleep Apnea

Airway obstruction is common in children with Down syndrome. Loss of sleep due to apnea and poor quality sleep due to sleep-disordered breathing can result in sleepiness and disturbances in fine motor skills and can affect behavior and learning. Long-term complications of sleep apnea include systemic hypertension, pulmonary hypertension, heart failure, and even death. A comprehensive clinical exam, X-ray, and thorough sleep study should be conducted if sleep apnea is suspected. Medical and surgical treatments may be indicated, as well as a Continuous Positive Airway Pressure (CPAP) machine during sleep. Weight reduction may also help address sleep apnea.

Airway Obstruction and Sleep Apnea

Airway obstruction is common in children with Down syndrome. A comprehensive clinical exam, X-ray, and thorough sleep study should be conducted if sleep apnea is suspected.

Chronic Rhinitis and Sinusitis

The facial anatomy of Down syndrome along with the developing immunological system make children with Down syndrome prone to chronic rhinitis (inflammation of the mucus membranes of the nose and mucus discharge) and sinusitis (inflammation of the sinus membranes). Treatment includes saline drops or spray to keep the smaller nasal passages clear, antihistamine medications, and steroid nasal sprays. These issues may improve with age. In children whose sinusitis fails to resolve with medical management, surgical removal of the adenoids and/or endoscopic sinus surgery may be necessary.

Chronic Rhinitis and Sinusitis

Children with Down syndrome are prone to chronic rhinitis (inflammation of the mucus membranes of the nose and mucus discharge) and sinusitis (inflammation of the sinus membranes). Treatment includes saline drops or sprays, antihistamine medications, and steroid nasal sprays. If these treatments fail to solve the problem, surgery may be necessary.

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Sources
National Down Syndrome Society (NDSS)
Down Syndrome Education USA (DSE)
National Institute for Health and Care Excellence (NICE)