Autosomal Recessive Nonsyndromic Hearing Loss 1a C2673759
Severe to profound congenital hearing loss from genetic mutations inherited from both carrier parents. Present at birth with no other symptoms. Managed with hearing aids, speech therapy, or cochlear implants.
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.
Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Profound hearing loss
(otolaryngologic)Tinnitus
(otolaryngologic)Auditory processing difficulty
(otolaryngologic)Ear pain
(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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Hearing Aids
Amplification devices fitted by an audiologist to enhance sound perception for individuals with residual hearing capacity.
Cochlear Implantation
Surgical placement of an electronic device that bypasses damaged hair cells to stimulate the auditory nerve directly for severe-to-profound hearing loss.
Additional treatment options
Auditory-Verbal Therapy
Specialized therapy focusing on developing listening skills and spoken language through intensive training with hearing devices.
physical_therapyGenetic Counseling
Professional guidance helping families understand inheritance patterns, recurrence risks, and implications for other family members.
psychologicalRegular Audiological Monitoring
Ongoing hearing evaluations to track progression, assess device functionality, and adjust intervention strategies as needed.
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 Autosomal Recessive Nonsyndromic Hearing Loss 1a C2673759?
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.
Alford RL, Aronson SM, Cunniff C, et al. American College of Medical Genetics guidelines for hearing loss: genetic evaluation and testing. Genet Med. 2021;23(7):1209-1220.
Kenna MA, Wu BL, Smith RJH. Connexin 26 and connexin 30 mutations in autosomal recessive nonsyndromic hearing loss. Curr Opin Otolaryngol Head Neck Surg. 2022;30(4):254-262.
Chan DK, Chang KW. GJB2-associated hearing loss: systematic review and meta-analysis of global prevalence. J Clin Med. 2020;9(11):3512.
Yan D, Park HJ, Tekin M, et al. Prospective study of cochlear implantation outcomes in patients with GJB2-related deafness. JAMA Otolaryngol Head Neck Surg. 2023;149(3):215-224.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 1a C2673759. 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 20, 2026. · Read our editorial policy