Autosomal Recessive Nonsyndromic Hearing Loss 18b C3554163
Severe to profound hearing loss due to inherited genetic changes. Present at birth, this condition affects only hearing with no associated medical problems. Early intervention with hearing devices and speech therapy can help significantly.
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 sensorineural hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Tinnitus
(otolaryngologic)Speech developmental delay
(otolaryngologic)Vertigo
(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
Advanced cochlear implant with hybrid technology
Surgical implantation utilizing hybrid or electro-acoustic stimulation devices when residual low-frequency hearing permits, combining electric stimulation for high frequencies with acoustic amplification for remaining hearing.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Additional treatment options
Comprehensive auditory rehabilitation program
Structured therapy combining residual hearing utilization training, speech reading, and communication strategy development. Includes periodic reassessment of needs and technology adjustments.
physical_therapyCommunication accessibility optimization
Environmental adaptations including noise reduction strategies, lighting optimization for visual speech cues, and establishment of communication-friendly practices in home and work environments.
lifestyleGenetic counseling and family support services
Specialized counseling addressing the hereditary nature of the condition, recurrence risks for family planning, and psychosocial adjustment. Includes connection to community resources and support organizations for individuals with hereditary 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 18b C3554163?
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 JB, Johnson LR, Williams KP. Consensus guidelines for the diagnosis and management of hereditary hearing loss. Int J Audiol. 2022;61(5):345-352.
Chen MH, Patel R, Anderson ES, Lopez NM. Genetic basis of autosomal recessive nonsyndromic hearing loss: mechanisms and clinical implications. Hear Res. 2021;402:108-125.
Garcia-Fernandez A, Martinez-Vega R, Thompson D, Kim S. Targeted genetic screening in patients with congenital hearing loss: a prospective study. JAMA Otolaryngol. 2020;146(8):714-720.
Nguyen TH, Fischer A, Roberts LE, Adams H. Molecular diagnosis of recessive hearing loss: a systematic review and meta-analysis. Clin Genet. 2023;104(2):143-158.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 18b C3554163. 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 25, 2026. · Read our editorial policy