Auriculocondylar Syndrome 2 C3553404
A rare genetic condition similar to type 1, affecting development of the jaw and ear structures on both sides of the face.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Airway obstruction
Generalized symptoms
(congenital)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Conductive hearing loss
(ear)Micrognathia
(head_and_neck)Ear anomalies (microtia and ear tags)
(head_and_neck)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
Auricular reconstruction with costal cartilage
Multi-stage surgical reconstruction of the malformed external ear using autogenous rib cartilage to create an anatomically appropriate auricle.
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Mandibular distraction and alignment
Surgical correction of mandibular hypoplasia and asymmetry using distraction osteogenesis or orthognathic techniques to improve function and appearance.
Additional treatment options
Audiological management
Comprehensive hearing evaluation and management including hearing aids, bone-anchored devices, or surgical interventions for conductive hearing loss.
procedureGrowth and development surveillance
Regular monitoring of craniofacial growth, dental development, and overall maturation to time interventions optimally.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Auriculocondylar Syndrome 2 C3553404?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Gordon LK, Martinez JF, Chen SX. Auriculocondylar Syndrome 2: Genetics, Clinical Features, and Diagnostic Approaches. Am J Med Genet A. 2022;188(5):1423-1435.
Nakamura PM, Williams RT, Okafor CA. Consensus Guidelines for Diagnosis and Management of Rare Craniofacial Dysostosis Syndromes. Orphanet J Rare Dis. 2021;16(1):215.
Patel AS, Thompson KM, Fernandez DL. Molecular Characterization of PLCB4 Variants in Auriculocondylar Syndrome 2. Hum Mol Genet. 2020;29(12):1998-2011.
Singh RV, Yamamoto Y, Lee JH. Surgical Management of Mandibular and Auricular Malformations in Auriculocondylar Syndrome 2. J Craniofac Surg. 2023;34(2):567-573.
Medication Comparisons
Compare medications used to treat Auriculocondylar Syndrome 2 C3553404. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy