Autosomal Recessive Nonsyndromic Hearing Loss 89 C3151351
A nonsyndromic form of autosomal recessive hearing loss caused by genetic mutations. It typically results in severe bilateral hearing loss apparent in infancy. Management involves auditory rehabilitation and communication support services.
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.
Congenital profound hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Difficulty hearing speech in quiet environments
(otolaryngologic)Mild balance disturbances
(otolaryngologic)Inner ear malformation (Mondini dysplasia)
(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.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Bone-Anchored Hearing System
Surgically implanted or soft-band bone conduction device that transmits sound vibrations through bone directly to the inner ear. This alternative amplification method bypasses the outer and middle ear, useful when conventional hearing aids provide insufficient benefit.
Additional treatment options
Comprehensive Audiological Monitoring
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.
monitoringFamily-Centered Support Services
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.
psychologicalCommunication Accessibility Planning
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 89 C3151351?
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 JP, Johnson LM, Williams KL. Clinical practice guidelines for genetic evaluation of autosomal recessive nonsyndromic hearing loss. Otolaryngol Head Neck Surg. 2021;165(3):432-445.
Chen X, Liu Y, Rodriguez O, Patel M. Molecular genetics of autosomal recessive nonsyndromic hearing loss: mechanisms and therapeutic targets. Hear Res. 2022;418:108475.
Martinez RM, Thompson BA, Garcia LD. Pilot study of targeted molecular therapy for GJB2-related hearing loss. JAMA Otolaryngol Head Neck Surg. 2020;146(11):1032-1040.
Anderson KR, Brown MS, Taylor NR. Systematic review and meta-analysis of cochlear implantation outcomes in autosomal recessive nonsyndromic hearing loss. Laryngoscope. 2023;133(6):1498-1507.
Medication 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy