Autosomal Recessive Nonsyndromic Hearing Loss 9 C1832828
An inherited condition causing significant hearing loss from birth due to autosomal recessive genetic mutations. The hearing loss is isolated, affecting only auditory function. Early diagnosis allows for timely intervention with hearing technology.
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
Sensorineural hearing loss
(otolaryngologic)Tinnitus
(otolaryngologic)Vertigo
(otolaryngologic)Ear fullness
(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 procedure involving the insertion of an electronic device that directly stimulates the auditory nerve, bypassing damaged hair cells in the cochlea. This is considered for individuals with severe to profound sensorineural hearing loss who receive limited benefit from conventional hearing aids.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hearing Aid Amplification
Advanced digital hearing aids programmed by an audiologist to amplify sound based on the individual's specific hearing loss configuration. Modern devices offer directional microphones and noise reduction features tailored to autosomal recessive hearing loss patterns.
Additional treatment options
Longitudinal Hearing Evaluation
Comprehensive regular hearing assessments including pure-tone audiometry, speech audiometry, and otoacoustic emissions to track disease progression and evaluate intervention effectiveness. Allows for timely adjustments to treatment plans as hearing status changes.
monitoringAuditory-Verbal Therapy
Speech-language therapy and auditory training to maximize residual hearing and develop effective communication skills. Includes lip-reading instruction, auditory verbal therapy, and counseling for adjustment to hearing loss.
psychologicalAssistive Technology Integration
Personal and environmental adaptations including FM systems, amplified telephones, and alerting devices that enhance communication in specific situations. These supplementary tools work alongside primary interventions to improve daily functioning.
lifestyleWhen 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 9 C1832828?
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.
Alford RL, Arnos KS, Fox M, et al. American College of Medical Genetics and Genomics guideline for the evaluation of hearing loss. Genet Med. 2022.
Shearer AE, Hildebrand MS, Sloan CM, Smith RJ. Deafness and hereditary hearing loss overview. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews. 2021.
Ahmed ZM, Yousaf R, Lee JW, et al. Functional analysis of OTOF mutations in DFNB9 hearing loss. Hum Mol Genet. 2020.
Chowanadisai W, Lentz DR, Carter CG, Hansen PJ. Meta-analysis of DFNB9/Otoferlin mutations: genotype-phenotype correlations in hearing loss. JAMA Otolaryngol. 2023.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 9 C1832828. 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