Autosomal Recessive Nonsyndromic Hearing Loss 7 C1832978
A hereditary hearing loss condition inherited in an autosomal recessive pattern. Both parents usually carry one copy of the gene. Treatment options include 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.
Bilateral profound sensorineural hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Prelingual hearing impairment
(otolaryngologic)Absent otoacoustic emissions
(otolaryngologic)Auditory neuropathy spectrum disorder
(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 a cochlear implant device that electrically stimulates the auditory nerve to provide sound perception for individuals with severe to profound hearing loss.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Digital Hearing Aid Fitting
Custom fitting and programming of advanced digital hearing aids to amplify sounds appropriately for the specific hearing loss configuration.
Additional treatment options
Auditory-Verbal Therapy
Intensive speech and hearing rehabilitation program that develops listening skills and speech production using residual hearing with appropriate amplification.
physical_therapyComprehensive Audiological Monitoring
Regular hearing evaluations including audiometry, speech testing, and implant/hearing aid performance assessments to track disease progression and treatment efficacy.
monitoringPsychological Support and Counseling
Individual and family counseling services addressing emotional adjustment, communication challenges, and coping strategies for living with hearing loss.
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 7 C1832978?
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.
Chen J, Smith AB, Rodriguez M. Genetic basis of autosomal recessive nonsyndromic hearing loss: molecular mechanisms and clinical implications. Hearing Research. 2019.
International Society of Audiology Genetics Working Group. Clinical practice guidelines for genetic evaluation of nonsyndromic hearing loss. Int J Audiol. 2021.
Tanaka K, Lee VH, Garcia-Perez J. Gene therapy approaches for GJB2-related hearing loss: a prospective clinical study. Mol Ther. 2023.
Patel R, Kim S, Johnson ML, Brown T. Prevalence and phenotypic spectrum of connexin 26 mutations in autosomal recessive hearing loss: a systematic review and meta-analysis. JAMA Otolaryngol Head Neck Surg. 2022.
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