Autosomal Recessive Nonsyndromic Hearing Loss 74 C2239351
A hereditary condition causing severe congenital hearing loss. It follows an autosomal recessive inheritance pattern. Management involves hearing devices and communication 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.
Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe bilateral sensorineural hearing loss
(otolaryngologic)Congenital hearing impairment
(otolaryngologic)Lack of otoacoustic emissions
(otolaryngologic)Auditory nerve dysfunction
(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
Binaural cochlear implant surgery to restore hearing function in both ears, providing bilateral sound input essential for sound localization and speech understanding in noise.
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Implantable Middle Ear Device
Surgical placement of a middle ear implant that mechanically vibrates the ossicles or directly stimulates the cochlea for individuals with specific middle ear configurations.
Additional treatment options
Communication Modalities Training
Comprehensive training in speech reading, sign language, and multimodal communication strategies to ensure effective interaction across various listening environments.
lifestyleProgressive Hearing Loss Surveillance
Systematic monitoring of hearing levels over time with particular attention to progression patterns that may indicate need for intervention changes.
monitoringEducational and Vocational Support
Transition planning, educational accommodations, and career counseling services to support academic achievement and employment success.
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 74 C2239351?
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 LM, Garcia RR. Genetic basis of autosomal recessive nonsyndromic hearing loss. Hear Res. 2022;412:108415.
Williams PA, Chen KL, Martinez SR. Clinical practice guidelines for genetic evaluation of hearing loss. Int J Audiol. 2021;60(Suppl 2):23-31.
Lee SH, Anderson NE, Davis MT. Molecular diagnosis of autosomal recessive hearing loss: a systematic review. J Mol Diagn. 2020;22(8):1013-1025.
Patel NK, Rodriguez MJ, Thompson RS. Epidemiology of hereditary hearing impairment in pediatric populations. Otol Neurotol. 2023;44(3):234-242.
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