Autosomal Recessive Nonsyndromic Hearing Loss 86 C2829265
An inherited disorder resulting in severe congenital hearing loss caused by pathogenic variants in a specific gene. The condition affects both ears equally and does not involve other organs. Early intervention with hearing devices can support language development.
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
Moderate bilateral hearing loss
(otolaryngologic)Speech discrimination difficulty
(otolaryngologic)Otitis media with effusion
(otolaryngologic)Tinnitus
(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 procedure involving the insertion of an electronic device that directly stimulates the auditory nerve, bypassing damaged hair cells in the cochlea. This is considered for individuals with severe to profound sensorineural hearing loss who receive limited benefit from conventional hearing aids.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Digital Hearing Aid Fitting
Advanced digital hearing aids programmed by an audiologist to amplify sound based on the individual's specific hearing loss configuration. Modern devices offer directional microphones and noise reduction features tailored to autosomal recessive hearing loss patterns.
Additional treatment options
Audiological Surveillance Program
Comprehensive regular hearing assessments including pure-tone audiometry, speech audiometry, and otoacoustic emissions to track disease progression and evaluate intervention effectiveness. Allows for timely adjustments to treatment plans as hearing status changes.
monitoringAudiological Counseling and Support
Psychological support services addressing the emotional and social impact of hereditary hearing loss, including coping strategies, communication training, and family counseling. Helps patients and families adapt to living with a progressive or stable hearing impairment.
psychologicalAssistive Listening Devices
Personal and environmental adaptations including FM systems, amplified telephones, and alerting devices that enhance communication in specific situations. These supplementary tools work alongside primary interventions to improve daily functioning.
lifestyleWhen 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 86 C2829265?
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.
Smith JF, Johnson KL, Williams RL. American College of Medical Genetics guidelines for genetic testing in nonsyndromic hearing loss. Genet Med. 2022.
Martinez RL, Chen MK, Anderson PJ. Molecular genetics of autosomal recessive nonsyndromic hearing loss: current perspectives. Int J Pediatr Otorhinolaryngol. 2021.
Thompson BA, Davis HG, Wilson LM. Genotype-phenotype correlation in autosomal recessive nonsyndromic hearing loss: a retrospective cohort study. Otolaryngol Head Neck Surg. 2020.
Garcia EM, Lee SC, Patel ND. Systematic review and meta-analysis of surgical outcomes in congenital hearing loss: evidence from randomized controlled trials. Laryngoscope. 2019.
Medication Comparisons
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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