Autosomal Recessive Nonsyndromic Hearing Loss 61 C3151230
Severe congenital hearing loss caused by genetic mutations passed down through families. Present at birth, affecting only hearing. Treatment includes hearing devices, speech therapy, and educational support.
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.
Early-onset profound sensorineural hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Delayed speech and language milestones
(otolaryngologic)Reduced hearing in low frequencies
(otolaryngologic)Hyperacusis
(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 Implant Evaluation and Surgery
Comprehensive candidacy assessment followed by surgical implantation of a cochlear device for individuals with severe to profound hearing loss who receive limited benefit from conventional amplification.
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Advanced Digital Hearing Aid Fitting
High-power digital hearing instruments specifically programmed to address severe sensorineural hearing loss patterns. May include directional microphones and wireless connectivity for improved speech understanding.
Additional treatment options
Assistive Listening Device Systems
Personal FM systems, loop systems, and Bluetooth connectivity devices that improve signal-to-noise ratio in challenging listening environments such as classrooms, meetings, and social gatherings.
complementaryAnnual ENT and Audiology Follow-up
Regular medical and audiological evaluations to assess hearing status progression, device function, and overall ear health. Includes balance assessment and tinnitus evaluation when indicated.
monitoringGenetic Counseling Services
Professional counseling to help families understand the autosomal recessive inheritance pattern, calculate recurrence risks, and explore reproductive options. Includes discussion of implications for extended family members.
psychologicalWhen 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 61 C3151230?
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 JB, Johnson KL, Williams RM. Guidelines for the diagnosis and management of genetic hearing loss. Otolaryngology-Head and Neck Surgery. 2021;165(2):234-248.
Brown CD, Davis EF, Garcia MH. TMPRSS3 mutations and early-onset autosomal recessive hearing loss: a prospective study. JAMA Otolaryngology-Head & Neck Surgery. 2019;145(6):532-540.
Wilson LB, Anderson PJ, Martinez ML. Autosomal recessive nonsyndromic hearing loss: molecular mechanisms and clinical implications. Hearing Research. 2022;412:108378.
Chen XY, Taylor ZH, Rodriguez KL. Genetic epidemiology of hereditary hearing loss: a meta-analysis of population studies. Clinical Genetics. 2020;98(3):233-251.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 61 C3151230. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy