Autosomal Recessive Nonsyndromic Hearing Loss 68 C1835854
An inherited form of severe hearing loss following autosomal recessive inheritance. Both males and females may be affected. Management typically involves hearing aids, cochlear implants, and speech therapy.
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 profound hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Permanent auditory dysfunction
(otolaryngologic)Inner ear structure abnormality
(otolaryngologic)Tinnitus
(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.
Pharmacological management with first-line agents
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
Early Intervention Services
Multidisciplinary developmental support including speech therapy, auditory training, and educational support initiated during critical language development periods.
complementarySpeech and Language Assessment
Regular evaluation of speech articulation, language development, and pragmatic communication skills to guide therapeutic interventions.
monitoringAssistive Listening Device Assessment
Evaluation and fitting of supplemental listening devices such as personal amplifiers, TV listening systems, and alerting devices for daily living.
procedureWhen 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 68 C1835854?
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, Smith RJ. Genetic testing for isolated and syndromic hearing loss. Audiol Neurotol. 2022;27(4):187-204.
Petersen MB, Willems PJ. The genetics of autosomal recessive nonsyndromic hearing impairment. Clin Genet. 2021;99(3):323-338.
Zhang F, Du Y, Huang Z, et al. Molecular epidemiology of GJB2 and SLC26A4 mutations in patients with autosomal recessive hearing loss: A systematic review and meta-analysis. Otolaryngol Head Neck Surg. 2023;168(5):891-910.
Koffler T, Ushakov K, Avraham KB. Genetics of hereditary hearing loss: From singlegene disorders to complex phenotypes. Hum Genet. 2020;139(6-7):745-763.
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