Autosomal Recessive Nonsyndromic Hearing Loss 76 C3147083
A genetic hearing disorder causing severe loss from birth without other symptoms. Parents typically carry one copy of the gene. Hearing aids or implants may help.
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 congenital bilateral hearing loss
(otolaryngologic)Prelingual severe hearing impairment
(otolaryngologic)Otoacoustic emissions not detectable
(otolaryngologic)Auditory neuropathy disorder
(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 an electronic device that bypasses damaged hair cells and directly stimulates the auditory nerve. Requires comprehensive pre-operative evaluation including imaging and hearing assessments.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Digital Hearing Aids
Programmable amplification devices fitted by an audiologist to compensate for hearing loss. Modern devices offer directional microphones and noise reduction features.
Additional treatment options
Auditory-Verbal Therapy
Intensive speech-language therapy focused on developing listening skills and spoken language through structured training sessions with parents and caregivers.
physical_therapyGenetic Counseling
Family-centered counseling explaining inheritance patterns, recurrence risks, and family planning options for autosomal recessive conditions.
psychologicalRegular Audiological Monitoring
Ongoing hearing evaluations to track disease progression and assess effectiveness of hearing devices. Includes speech discrimination testing and device programming checks.
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 76 C3147083?
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, Arnos KS, Fox M, et al. American College of Medical Genetics and Genomics guideline for the evaluation of children with hearing loss. Genet Med. 2022.
Shearer AE, Hildebrand MS, Sloan CM, Smith RJ. Deafness in the genomics era. Hear Res. 2011.
Matsunaga T, Mutai H, Namba K, et al. Longitudinal study of auditory function in patients with autosomal recessive hearing loss. Otol Neurotol. 2018.
Sloan-Heggen CM, Smith RJ. Navigating genetic testing for sensorineural hearing loss. Curr Opin Otolaryngol Head Neck Surg. 2016.
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.
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