The muscles of the affected side of the face are under-developed and there are often skin tags or pits in front of the ear, or in a line between the ear and the corner of the mouth. There are often abnormalities of the middle ear and the ear canal may be completely absent. Hearing loss (one-sided) is extremely common.
There may also be eye abnormalities, including cysts and notches in the lids, squints and occasionally small eyes. Children at the Goldenhar end of the spectrum may, in some cases, have heart problems. A variety of kidney abnormalities may also be present. There are a number of other rarer congenital (present at birth) problems that may occur. Most individuals with Goldenhar syndrome are of normal intelligence, although some learning difficulties (see Learning Disability) can occur in about 10-15 per cent of cases. These are usually language problems as a result of hearing loss. There may also be speech and swallowing problems. Many babies with Goldenhar syndrome have poor weight gain in the first year or two of life.