Down Syndrome and Ear Problems
Contributed by Dr. Marcella Bothwell, MD
Children born with Down Syndrome may have significant problems in the region of the ear, nose, and throat. This article will focus on major ear problems and interventions for children with ear issues. The 3 major issues to be concerned about are the following and will be explained in more detail.
- External Ear Canal Stenosis or narrowing
- OME (Otitis Media with Effusion) or fluid in the middle ear
- Two types of hearing loss, Conductive Hearing Loss (CHL) and Sensorinerural (SNHL)
1) External Ear Canal Stenosis: affects up to 50% of newborns with Down Syndrome and can make it difficult to see your child’s ear drum (tympanic membrane). Visualization is required for the diagnosis of ear infections or chronic ear fluid. An ENT or Otolaryngologist / Head and neck surgeon may recommend cleaning with a microscopic exam up to every 3 months for ideal visualization. Ideally by 2-3 years, the ear canals may have grown enough not to need such frequent cleaning. Please do not use any Q-tips to clean ears because it actually makes the wax impaction (clogging of ear canal) worse.
2) Otitis Media with Effusion (OME): fluid in the middle ear may be chronic and not be acutely infected. Sometimes however it can also be a more typical ear infection with fever and pain. Reasons for having more ear problems are:
- Reduction of the immune system (both T and B cell function) which may increase upper respiratory infections or colds
- Eustachian tube (ET) dysfunction or abnormal function of the tube between the ear and nose. Causes are usually anatomical or structural such as: mid face hypoplasia or flattening; the shape of the Eustachian may have a smaller width; and / or the cartilage density may make it weaker causing collapse. Hypotonia or general muscle weakness may not just be in the core muscles but also in the muscle that opens the ear or ET i.e. the tensor veli palatini muscle dysfunction which opens and closes ET. Because of the above reasons, Eustachian Tube Dysfunction (ETD) persists much longer compared to other children without DS. However there are no studies that show the expected length of time for persistent OME.
3.) Hearing loss can either be conductive (CHL) or sensorineural (SNHL):
In a prospective study of children with Down Syndrome it was reported by Shott that 98% had normal hearing at one year enrollment but 83% of children required ear tubes due to chronic ear infections or OME. Balkany in 1979 reported 83% of hearing loss is conductive with 60% of the conductive loss due to chronic fluid or perforations. 40% was ossicular (hearing bones of the ear) either congenital or due to secondary erosion. However, Harado and Sandi in 1981 reported, 25% of patients with DS were evaluated at surgery where no obvious anatomic abnormalities were found. 4-20% SNHL or mixed hearing loss is also reported.
Hearing testing is done at birth by BAER (Brainstem Audio Evoked Potential)/ OAE (Otoacoustic Emission) testing and every 6 months following until ear specific information can be attained with Behavioral or pure tone audiogram (hearing testing)
At age 3, 12% of children were able to get ear specific information and by age 4, 41% were successful at behavioral hearing testing.
Remedies to hearing loss include: Ossicular reconstruction and Amplification (hearing aides). Remedy is important due to delayed expressive language skills compared to cognitive abilities. By helping children with Down syndrome express their needs, their disabilities may improve.