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Wildervanck syndrome

Other Names for this Disease
  • Cervico-oculo-acoustic dysplasia
  • Cervico-oculo-acoustic syndrome
  • Cervicooculoacoustic syndrome
  • COA Syndrome
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Your Question

I have Wildervanck syndrome.  I wonder if I will able to have children, a boy or a girl, or just a girl.  Is it true that it will affect a baby boy?

Our Answer

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

What is Wildervanck syndrome?

Wildervanck syndrome is a condition that occurs almost exclusively in females and affects the bones in the neck, the eyes, and the ears.  It is characterized by Klippel-Feil anomaly (in which the bones of the neck fuse together), Duane syndrome (an eye movement disorder that is present from birth), and hearing loss. [1]  The cause of Wildervanck syndrome is unknown.  In most cases, affected individuals have no family history of the condition. [2]
Last updated: 11/3/2010

What causes Wildervanck syndrome?

The exact cause of Wildervanck syndrome is not known.  It is suspected to be a polygenic condition, meaning that many genetic factors may be involved. [2] 
Last updated: 11/3/2010

How is Wildervanck syndrome inherited?

Wildervanck syndrome does not have a clear pattern of inheritance.  In most cases, only one person in a family is affected.  These cases are called isolated or sporadic because there is no family history of Wildervanck syndrome.  Because this syndrome occurs mostly in females, it is possible that this condition has X-linked dominant inheritance.  The lack of males with Wildervanck syndrome suggests that affected males have more severe features and do not survive to birth. [2]
Last updated: 11/2/2010

Can a person with Wildervanck syndrome have children?

It is not known if Wildervanck syndrome affects a person's ability to have a child.  Because the cause of Wildervanck syndrome is also uknown, it is difficult to determine the chance that a child could inherit the condition from an affected parent. [2]  At least one family has affected individuals in five generations. [3]  There is also a report of an affected mother having two sons and a daughter who were not affected. [4] 

To find out your specific risk of having a child with Wildervanck syndrome, we recommend that you consult with a genetics professional.
Last updated: 10/18/2013

How can I find a genetics professional in my area?

Genetics clinics are a source of information for individuals and families regarding genetic conditions, treatment, inheritance, and genetic risks to other family members. More information about genetic consultations is available from Genetics Home Reference. To find a genetics clinic, we recommend that you contact your primary healthcare provider for a referral.

The following online resources can help you find a genetics professional in your community:
Last updated: 10/18/2013

  • Gorlin, R; Cohen Jr., M; Hennekam, R. Syndromes of the Head and Neck. Oxford: Oxford University Press; 2001;
  • Wettke-Schafer and Kantner, G. X-linked Dominant Inherited Diseases With Lethality in Hemizygous Males. Human Genetics. 1983; 64:1-23.
  • Wildervanck syndrome. Orphanet. November 2009; Accessed 11/2/2010.
  • Shenoi, PM. Wildervanck's syndrome: Hereditary malformations of the ear in three generations. The Journal of Laryngology and Otology . 1972; 86:1121-1135.