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Winter 2002 Table of Contents
Versión Español de este artículo (Spanish Version)
By Kate Moss, Family Specialist, TSBVI, Texas Deafblind Outreach
Goldenhar Syndrome is one of the many syndromes that can result in a child having vision loss, hearing loss, or deafblindness. Like many other syndromes, there is typically a range of specific conditions. One child with Goldenhar may have only a few of the associated conditions while another child may have many of these conditions. It is important that those of us responsible for addressing issues of vision loss, hearing loss or deafblindness in educational settings are aware of what conditions might be present with each child who has this syndrome. Then, we can appropriately assess and program for his or her needs. Parents and family members need to connect with the medical and informational resources that may help them support their child.
Goldenhar (sometimes called Oculo-Auriculo-Vertebral or OAV Spectrum) is one of a group of conditions known as craniofacial conditions. That means that the impact is primarily to the head and face. Individuals with Goldenhar typically have faces that may appear smaller on one side, or the sides appear very different from each other. The cheekbones, jaws, and bones forming parts of the skull may be underdeveloped or malformed. There is sometimes incomplete development of the facial muscles, cleft lip and/or palate, teeth abnormalities, and what appears to be an abnormally wide mouth due to cleft-like extensions of the corners of the mouth. With Goldenhar, there is also the possibility of vertebral malformations, where the vertebrae may be underdeveloped, fused, or missing. Some children may also have heart, lung, kidney and/or gastrointestinal problems and genital malformation. A small percentage also have mental retardation (N.O.R.D. 2001).
Both the National Organization for Rare Disorders (N.O.R.D.) and Pediatric Database give information about the various conditions associated with Goldenhar. The Children's Craniofacial Association is another resource. Someone familiar with all aspects of this disorder will be better able to plan for the child and support the family's needs.
P.O. Box 8923
New Fairfield, CT 06812-8923
Children's Craniofacial Association
12200 Park Central Drive, Suite 180
Dallas, TX 75251
Phone: (972) 566-5980, (800)535-3643
World Craniofacial Foundation
7777 Forest Lane, Suite C-621
P.O. Box 515838
Dallas, TX 75251-5838
Phone: (972)566-6669 and (800) 533-3315
The Children's Craniofacial Association hosts the Annual Cher's Family Retreat weekend for immediate family members of individuals with a craniofacial condition. The retreat is named for the singer, Cher, who portrayed a mother of a child with a craniofacial disorder in the movie, Mask. She lends her support to the organization and to this very special event. This year's event is scheduled for June 27th - 30th in St. Louis, Missouri.
Vision conditions associated with Goldenhar Syndrome include:
When addressing vision programming for these children, teachers must be aware that the child may be photophobic or have problems adjusting to bright light and glare due to the coloboma or microphthalmia. There may be decreased acuity due to the microphthalmia or retinal anomalies. The child may also have a field loss related to many of these eye conditions. Additionally there may be problems with depth perception if strabismus is present. A thorough ophthalmological examination and functional vision evaluation should guide the team in addressing modification and accommodations for the child's specific vision issues. If the child is also hearing impaired, these vision issues may impact his/her ability to read sign language or speechread in certain situations.
Hearing conditions associated with Goldenhar Syndrome include:
Appropriate programming for hearing loss begins with a thorough otological and audiological exam. Depending on the different malformations, a conductive, sensorineural or mixed hearing loss may occur. Because malformation of the outer ear and ear canal prohibit the use of certain types of hearing aids, amplification may be an issue. Bone conduction hearing aids may be needed. However, malformation of the mastoid bones might reduce the benefits of this type of amplification. Depending on the type and degree of hearing loss, you might see a child with this condition having problems localizing sounds, listening in noisy environments, missing critical elements of spoken words or being unable to hear certain speech and/or environmental sounds. If the child is deafblind, this may greatly impact learning orientation and mobility skills. Malformation of facial muscles and the jaw may cause problems in forming certain speech sounds.
Children with Goldenhar should be thoroughly assessed in both vision and hearing. Finding and fitting assistive devices such as glasses and hearing aids may be a challenge. Appropriate modifications made for programming and materials should also consider associated health issues. Children with Goldenhar may be subjected to numerous surgeries to address jaw abnormalities and dental issues. They may have ongoing health issues that impact school attendance and reduced stamina. Problems with teeth and jaws, cleft palate and lip, and general gastrointestinal problems may cause nutritional concerns. All these factors must be taken into consideration when developing an educational program for this child.
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Last Revision: July 30, 2002