Autosomal Recessive Nonsyndromic Hearing Loss 65 C1853248
A hereditary condition causing severe hearing loss passed from carrier parents to children. Both males and females can be affected. Communication support includes hearing aids and cochlear implants.
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.
Warning signs - seek immediate care
Speech and language developmental delay
Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Profound congenital hearing loss
(otolaryngologic)Bilateral inner ear dysfunction
(otolaryngologic)Absence of otoacoustic emissions
(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 to stimulate the auditory nerve directly, providing sound perception for severe-to-profound hearing loss.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Hearing Aid Fitting
Custom-fitted amplification devices programmed to individual hearing thresholds, improving speech comprehension and environmental awareness.
Additional treatment options
Auditory Brainstem Implant
Surgical implant that stimulates the brainstem directly, used when cochlear implantation is not possible due to auditory nerve damage.
procedureRegular Audiologic Monitoring
Periodic hearing assessments to track progression, adjust amplification devices, and assess candidacy for surgical interventions.
monitoringSign Language and Communication Therapy
Comprehensive communication training including speechreading, sign language, and auditory training to maximize functional communication skills.
complementaryWhen 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 65 C1853248?
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.
Shearer AE, Hildebrand MS, Sloan CM, Smith RJ. Deafness and Hereditary Hearing Loss Overview. GeneReviews. 2018.
World Health Organization. World Hearing Report: Hearing Care for All. Int J Audiol. 2021.
Sloan-Heggen CM, Bierer AO, Shearer AE, Kolbe DL, Nishimura CJ, Frees KL, et al. Comprehensive Genetic Testing in the Clinical Evaluation of 1119 Patients with Hearing Loss. Hum Genet. 2016.
American Academy of Otolaryngology-Head and Neck Surgery. Position Statement: Genetic Testing for Hearing Loss. Otolaryngol Head Neck Surg. 2020.
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 25, 2026. · Read our editorial policy