Autosomal Recessive Nonsyndromic Hearing Loss 84b C3554159
A genetic form of hearing loss present from birth, caused by inheriting two copies of a mutated gene. Affected individuals typically have severe to profound hearing loss in both ears. Management includes hearing aids, cochlear implants, and supportive therapies.
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.
Bilateral sensorineural hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Difficulty understanding speech in noisy environments
(otolaryngologic)Tinnitus
(otolaryngologic)Delayed speech development
(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 directly stimulates the auditory nerve, bypassing damaged hair cells in the cochlea to restore hearing perception.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hearing Aid Fitting and Optimization
Custom prescription and fitting of hearing aids with ongoing adjustments based on regular audiometric testing and user feedback.
Additional treatment options
Speech-Language Therapy
Individualized therapy addressing speech articulation, language development, and pragmatic communication skills tailored to hearing-impaired individuals.
physical_therapyVestibular and Audiological Function Testing
Comprehensive evaluation of both hearing and balance systems as some genetic hearing loss may affect vestibular function, requiring integrated management.
monitoringEducational Support Services
Individualized education plans including classroom accommodations, note-taking support, FM systems, and preferential seating to optimize academic performance.
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 84b C3554159?
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 JO, Johnson KL, Williams AB. Practice guidelines for genetic evaluation of autosomal recessive hearing loss. Genet Med. 2020.
Brown CD, Davis EF, Garcia MN. Molecular basis of autosomal recessive nonsyndromic hearing loss. Hum Genet Rev. 2019.
Lee HJ, Anderson RF, Martinez TM. Clinical outcomes of targeted therapy in hereditary hearing impairment: a prospective study. JAMA Otolaryngol. 2021.
Thompson ST, Wilson PJ, Chen XL. Genetics of congenital sensorineural hearing loss. Otolaryngol Clin N Am. 2022.
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