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Genetic and Rare Diseases Information Center (GARD)

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Duane syndrome

Other Names for this Disease
  • DRS
  • Duane anomaly
  • Duane retraction syndrome
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Your Question

Duane syndrome affects my left eye. I have constant ringing in my left ear and neck pain when I look over my left shoulder. Are these issues related to Duane syndrome? My doctors did not know the answer.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What are the signs and symptoms of Duane syndrome?

Duane syndrome (DS) is present at birth and is characterized by limitation of horizontal eye movement (a limited ability to move the eye inward toward the nose (adduction), outward toward the ear (abduction), or in both directions). In addition, during adduction, the eyeball pulls in and the eye opening (palpebral fissure) narrows. In some cases, when the eye attempts to look inward, it moves upward or downward. Duane syndrome has 3 sub-types which vary depending on which type of eye movement is most restricted.[1]

In Duane syndrome type 1, abduction is limited, but adduction is normal or close to normal. The eye opening narrows and the eyeball retracts into the orbit during adduction, and the reverse occurs during abduction. In Duane syndrome type 2, adduction is limited, and abduction is normal or only slightly limited. In Duane syndrome type 3, both adduction and abduction are limited. The eyeball retracts during adduction in types 2 and 3. Each of these three types has been further classified into three subgroups designated A, B, and C to describe the eyes when looking straight.

Duane syndrome is an isolated finding in approximately 70 percent of cases but may be associated with other abnormalities. Major anomalies that may be associated with DS may affect the skeletal system, ears, eyes, nervous system, and/or the kidneys and urinary tract.[1]
Last updated: 3/26/2012

Are neck pain and ringing in the ears associated with Duane syndrome?

We were unable to find information in the available medical literature about neck pain and/or ringing in the ears being present in individuals with Duane syndrome (DS). It is known that in about 30% of cases, other conditions and syndromes have been found in association with DS. One syndrome associated with DS that may involve the neck and/or ears is called Wildervanck syndrome, which is characterized by fusion of bones in the neck, Duane syndrome, and hearing loss.[2] It has also been reported that individuals with DS often maintain a specific head posture or head turn to keep the eyes straight, and poor neck or head posture in any individual may theoretically lead to neck pain.[3]
Last updated: 3/26/2012