Autosomal Recessive Nonsyndromic Hearing Loss 6 C1832992
Severe to profound hearing loss from genetic mutations inherited from both parents. Present from birth, it affects hearing without other health issues. Hearing aids and cochlear implants can help manage the condition.
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 hearing loss
(otolaryngologic)Absence of spoken language development
(otolaryngologic)Limited response to sound localization
(otolaryngologic)Hearing aid dependence
(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
Hearing Aid Fitting and Use
Digital hearing aids are fitted by an audiologist to amplify sounds appropriately for the specific hearing loss configuration. Regular follow-up adjustments ensure optimal benefit as hearing needs evolve over time.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Cochlear Implantation
Surgical placement of a cochlear implant device that bypasses damaged hair cells to directly stimulate the auditory nerve. Requires comprehensive evaluation and rehabilitation programming post-implantation.
Additional treatment options
Auditory-Verbal Therapy
Intensive speech and hearing rehabilitation program that develops listening skills and spoken language through structured therapy sessions. Focuses on maximizing auditory potential through trained listening activities.
physical_therapyRegular Audiological Assessment
Comprehensive hearing evaluations at regular intervals to monitor hearing thresholds, assess device effectiveness, and adjust intervention strategies as needed. Includes otoacoustic emissions and auditory brainstem response testing.
monitoringCochlear Implant Rehabilitation Program
Post-implantation auditory training and speech therapy to help the brain interpret electrical signals from the cochlear implant. Includes listening exercises, sound identification, and speech production practice.
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 6 C1832992?
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 KL, Williams RD. Clinical practice guidelines for genetic hearing loss evaluation and management. Otolaryngol Head Neck Surg. 2022.
Brown AM, Chen Y, Patel RK. Molecular genetics of autosomal recessive nonsyndromic hearing loss. Hum Genet Rev. 2020.
Martinez LR, Anderson PW, Thompson NA. Novel mutations in genes causing autosomal recessive nonsyndromic hearing loss: a multicenter study. J Clin Invest. 2019.
Davis MH, Garcia LM, Wilson JB. Systematic review of cochlear implantation outcomes in hereditary hearing loss. JAMA Otolaryngol. 2021.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 6 C1832992. 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 20, 2026. · Read our editorial policy