Malignant Jugulotympanic Paraganglioma C0347856
A rare tumor near the ear and skull base arising from paraganglion cells. Usually locally aggressive, treated with surgery and sometimes radiation.
Key facts at a glance
- ICD-10
- H93.9
- ICD-11
- 9B00.0
- Body system
- otolaryngologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Facial nerve weakness
Conductive hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Pulsatile tinnitus
(otolaryngologic)Vertigo
(otolaryngologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Surgical tumor resection
Complete surgical removal of malignant jugulotympanic paraganglioma when anatomically feasible, often requiring skull base approaches and potentially involving facial nerve reconstruction.
Stereotactic radiosurgery
Focused radiation therapy delivering precise high-dose radiation to tumor tissue while minimizing damage to surrounding structures, particularly useful for unresectable or residual tumors.
Additional treatment options
Preoperative tumor embolization
Catheter-based technique to block blood supply to the tumor before surgery, reducing operative bleeding risk and improving surgical visualization and safety.
procedureSerial imaging surveillance
Regular MRI and CT imaging follow-up to detect tumor recurrence or progression, with audiometric testing and cranial nerve function assessment at each visit.
monitoringSDHB genetic testing
Germline genetic testing for succinate dehydrogenase complex mutations that influence malignancy risk and guide intensity of surveillance protocols.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Malignant Jugulotympanic Paraganglioma C0347856?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy