Deafness Digenic Gjb2 Gjb6 C2673760
An inherited form of hearing loss caused by mutations in two different genes (GJB2 and GJB6) that work together. Affected individuals are typically born with significant hearing impairment. Hearing aids or cochlear implants can help improve communication.
Key facts at a glance
- ICD-10
- Q17.9
- ICD-11
- LA00.0
- Body system
- otolaryngologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Bilateral prelingual hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Mild high-frequency hearing loss
(otolaryngologic)Speech articulation difficulties
(otolaryngologic)Recurrent otitis media
(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
Cochlear Implantation
Surgical placement of a cochlear implant device that bypasses damaged hair cells and directly stimulates the auditory nerve. Requires extensive pre-surgical evaluation including imaging and audiologic testing.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Hearing Aid Fitting
Custom-fitted hearing amplification devices programmed based on audiometric findings. Regular adjustments and follow-up are essential for optimal performance.
Additional treatment options
Auditory-Verbal Therapy
Intensive speech and hearing therapy focusing on developing listening skills and spoken language through specialized training sessions with certified therapists.
psychologicalAssistive Listening Devices
Personal and classroom amplification systems including FM devices, loop systems, and alerting devices that enhance communication in various environments.
lifestyleAudiologic Monitoring Program
Regular hearing assessments and device programming adjustments to track hearing levels and optimize amplification as hearing status may change over time.
monitoringWhen 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 Deafness Digenic Gjb2 Gjb6 C2673760?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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.
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 20, 2026. · Read our editorial policy