National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Cholesteatoma


Other Names:
Primary acquired cholesteatoma (type); Secondary acquired cholesteatoma (type); Congenital cholesteatoma (type)
Categories:
Cholesteatoma is an abnormal growth of skin in the middle ear behind the eardrum. It can be congenital (present from birth), but it more commonly occurs as a complication of chronic ear infections.[1][2] Individuals with this condition usually experience a painless discharge from the ear.[3] Hearing loss, dizziness, and facial muscle paralysis are rare but can result from continued cholesteatoma growth.[3][2] Treatment usually involves surgery to remove the growth.[3]
Last updated: 4/28/2017
Early symptoms may include fluid drainage from the ear, sometimes with a foul odor.[1][4]. As the cholesteatoma enlarges, it can lead to:[1][3][4]
  • A full feeling or pressure in the ear
  • Hearing loss
  • Dizziness
  • Pain
  • Numbness or muscle weakness on one side of the face
Occasionally, individuals may experience complications of the central nervous system including:[3]
  • A blood clot in certain veins within the skull, including the sigmoid sinus
  • A collection of infected material between the outer covering of brain and skull (epidural abscess) 
  • Inflammation of the tissue that surrounds the brain and spinal cord (meningitis)
Last updated: 4/28/2017
A cholesteatoma usually occurs because of poor eustachian tube function in combination with infection in the middle ear.[1][4] When the eustachian tube is not working correctly, pressure within the middle ear can pull part of the eardrum the wrong way, creating a sac or cyst that fills with old skin cells. If the cyst gets bigger, some of the middle ear bones may break down, affecting hearing.[1] Rarely, a congenital form of cholesteatoma (one present at birth) can occur in the middle ear and elsewhere, such as in the nearby skull bones.[4]
Last updated: 4/28/2017
Initial treatment may involve careful cleaning of the ear, antibiotics, and eardrops. Therapy aims to stop drainage in the ear by controlling the infection.[4] Large or more complicated cholesteatomas may require surgery.[1][4] Cholesteatomas very often continue to grow if they are not removed. Surgery is usually successful.[4]
Last updated: 5/1/2017
Cholesteatomas usually continue to grow if not removed. Surgery is typically successful, but occasional ear cleaning by a healthcare provider may be necessary. Additional surgery may be needed if the cholesteatoma comes back.[1][3]

In rare cases, complications may arise. These include:[1][3]
  • A collection of pus and other material in the brain (brain abscess)
  • Hearing loss in one ear
  • Dizziness (vertigo)
  • A breakdown of the facial nerves leading to facial paralysis
  • Meningitis
  • Persistent ear drainage
  • Spread of the cyst into the brain
    • Last updated: 4/28/2017

    Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

    Clinical Research Resources

    • ClinicalTrials.gov lists trials that are related to Cholesteatoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

      Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
    • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

    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

    Social Networking Websites

    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

    • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
    • The Vestibular Disorders Association provides information about Cholesteatoma 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.
    • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
    • PubMed is a searchable database of medical literature and lists journal articles that discuss Cholesteatoma. Click on the link to view a sample search on this topic.

    Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

    • I have been diagnosed with cholesteatoma. I am looking for general information about this condition, including the chances of being affected. See answer


    1. Cholesteatoma. MedlinePlus. May 25, 2016; http://www.nlm.nih.gov/medlineplus/ency/article/001050.htm.
    2. Cholesteatoma. American Academy of Otolaryngology-Head And Neck Surgery. http://www.entnet.org/content/cholesteatoma. Accessed 4/28/2017.
    3. Roland PS. Cholesteatoma. Medscape. January 19, 2017; http://emedicine.medscape.com/article/860080.
    4. Cholesteatoma. Vestibular Disorders Association. https://vestibular.org/cholesteatoma. Accessed 4/28/2017.