Rare Infectious Disease News

Disease Profile

Superior semicircular canal dehiscence syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

SCDS; Superior canal dehiscence syndrome

Summary

Superior semicircular canal dehiscence syndrome is a rare balance disorder characterized by auditory and/or vestibular symptoms. These might include dizziness and vertigo triggered by heavy lifting, straining, coughing or loud sounds that change the middle ear or intracranial pressure, fullness in the ear, autophony (an echo or reverberation in the ear when speaking, chewing or swallowing), hearing loss, nystagmus, or oscillopsia (the apparent motion of objects that are stationary). This condition is caused by an opening (dehiscence) in the bone that overlays the superior (uppermost) semicircular canal within the inner ear. While many patients with superior semicircular canal dehiscence syndrome are able to tolerate their symptoms and reduce or avoid triggering stimuli, others can benefit from surgical repair of the dehiscence.[1][2][3]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • The American Speech-Language-Hearing Association has a Clinical Topics page on Superior semicircular canal dehiscence syndrome on their Web site. Click on the link to view this information.
      • EURORDIS Rare Diseases Europe provides information about living with Superior semicircular canal dehiscence syndrome. Click on the link to view this information.
      • MayoClinic.com has an information page on Superior semicircular canal dehiscence syndrome.
      • The Ménière’s Society has an information page on Superior semicircular canal dehiscence syndrome. Click on the link to view this page.
      • The Vestibular Disorders Association provides information about Superior semicircular canal dehiscence syndrome on their Web site. Click on the link to access this information.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Superior semicircular canal dehiscence syndrome. Click on the link to view a sample search on this topic.

          References

          1. Minor LB, Carey JP. Superior Semicircular Canal Dehiscence (SSCD). Vestibular Disorders Association. https://vestibular.org/superior-canal-dehiscence-scd. Accessed 5/5/2015.
          2. Superior semicircular canal dehiscence syndrome. Ménière’s Society. https://www.menieres.org.uk/information-and-support/symptoms-and-conditions/superior-canal-dehiscence-syndrome. Accessed 5/5/2015.
          3. Superior Canal Dehiscence. American Speech-Language-Hearing Association. https://www.asha.org/PRPSpecificTopic.aspx?folderid=8589934662&section=Overview. Accessed 5/5/2015.