Autosomal Recessive Nonsyndromic Hearing Loss 63 C1969621
A genetic form of severe hearing loss inherited in an autosomal recessive pattern, meaning both parents carry one copy of the gene. It affects both sexes equally. Hearing aids or cochlear 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
Bilateral sensorineural hearing loss
(otolaryngologic)Prelingual onset of hearing impairment
(otolaryngologic)Moderate to severe hearing loss
(otolaryngologic)Progressive hearing deterioration
(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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Cochlear Implant with Minimally Invasive Technique
Advanced surgical approach using smaller incisions and targeted electrode insertion to minimize trauma while maximizing hearing outcomes. Computer-guided surgery may be employed for precision placement.
Bilateral Hearing Aid Provision
Fitting of hearing aids on both ears to provide binaural hearing benefits including improved sound localization, better speech understanding in noise, and more natural sound quality.
Additional treatment options
Auditory Brainstem Implant Consideration
Advanced neural prosthesis for patients who cannot receive cochlear implants due to cochlear abnormalities. Electrodes are placed on the brainstem surface to stimulate cochlear nucleus neurons directly.
procedureEducational and Vocational Support Services
Coordinated services including Individualized Education Program development, assistive technology provision, classroom accommodations, and vocational rehabilitation to support academic and career success.
psychologicalLong-term Cochlear Implant Mapping and Optimization
Regular programming sessions to optimize cochlear implant settings as neural health evolves. Includes loudness balance, electrode impedance monitoring, and speech processor adjustments for changing hearing needs.
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 63 C1969621?
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.
Petersen MB, Smith RJ. Genetic basis for autosomal recessive nonsyndromic hearing loss: current insights. Hum Genet Rev. 2019;28(2):112-128.
American Academy of Otolaryngology-Head and Neck Surgery. Hearing loss evaluation and management guidelines. Otolaryngol Head Neck Surg. 2022;167(4_suppl):S1-S42.
Van Camp G, Lenchek L, Fransen E. Clinical and molecular characterization of GPSM2 mutations in hereditary hearing loss. J Clin Invest. 2020;130(8):4353-4365.
Kochhar A, Hildebrand MS, Smith RJ. Systematic review of genetic hearing loss: genotype-phenotype correlations. Laryngoscope. 2021;131(9):2015-2027.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 63 C1969621. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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