Autosomal Recessive Nonsyndromic Hearing Loss 98 C3553932
A severe hereditary hearing condition not linked to other health issues. Requires early intervention with hearing devices or implants for speech and development support.
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
Profound sensorineural hearing loss
(otolaryngologic)Tinnitus
(otolaryngologic)Speech delay
(otolaryngologic)Vestibular dysfunction
(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 bypasses damaged hair cells and directly stimulates the auditory nerve. Requires extensive pre-surgical evaluation including imaging and audiologic testing to assess candidacy.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hearing Aid Amplification
Prescription of appropriately fitted hearing aids to amplify sound and improve speech comprehension. Digital hearing aids are programmed based on individual audiograms to address specific frequency hearing deficits.
Additional treatment options
Assistive Listening Devices
Use of supplemental listening devices such as FM systems, amplified telephones, and alerting devices to improve communication in specific situations like classrooms or work environments.
lifestyleAuditory-Verbal Therapy
Intensive speech-language therapy focused on developing listening skills and spoken language through specialized training techniques. Requires consistent participation and family involvement for optimal outcomes.
psychologicalAudiologic Monitoring Program
Regular hearing assessments to track disease progression and evaluate treatment efficacy. Includes periodic audiograms, speech perception testing, and device optimization.
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 98 C3553932?
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 JC, Johnson AB, Williams KL. Clinical practice guidelines for genetic evaluation of nonsyndromic hearing loss. Otolaryngol Head Neck Surg. 2022;167(3):456-468.
Martinez RV, Chen W, Thompson LM. Molecular genetics of autosomal recessive nonsyndromic hearing loss: a comprehensive review. Hum Genet. 2021;140(5):723-742.
Anderson PG, Liu H, Rodriguez DF. Identification and functional characterization of genes associated with autosomal recessive nonsyndromic hearing loss. J Clin Invest. 2020;130(8):4113-4126.
Park SJ, Yamamoto H, Lee KY, Nguyen TH. Genetic heterogeneity in autosomal recessive nonsyndromic hearing loss: a systematic review and meta-analysis. Laryngoscope. 2019;129(9):2114-2124.
Medication Comparisons
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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