Autosomal Recessive Nonsyndromic Hearing Loss 18a C1865870
Severe congenital hearing loss caused by genetic mutations passed from carrier parents. Present from birth, it affects hearing without other health problems. Treatment includes hearing aids, speech therapy, or 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.
Generalized symptoms
(otolaryngologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Sensorineural hearing loss
(otolaryngologic)Tinnitus
(otolaryngologic)Difficulty understanding speech in noisy environments
(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 placement of a cochlear implant device for individuals with severe to profound sensorineural hearing loss, providing direct electrical stimulation of the auditory nerve. Pre-operative assessment includes imaging of cochlear anatomy and hearing evaluation.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Additional treatment options
Auditory-verbal and speech-language therapy
Specialized rehabilitation focusing on developing listening skills, speech production, and language comprehension through amplification devices. Includes vibration feedback and multisensory approaches as appropriate.
physical_therapyPsychosocial support and counseling
Mental health support addressing adjustment to hearing loss, family dynamics, educational challenges, and social integration. Includes coping strategies, communication counseling, and connection to peer support networks.
psychologicalAssistive listening technology and environmental modifications
Implementation of alerting devices, closed captioning, visual alarm systems, and preferential seating arrangements in educational and work settings. Technology integration including smartphone-compatible hearing devices.
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 18a C1865870?
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.
Sheffield AM, Smith RJH. Molecular genetics of autosomal recessive nonsyndromic hearing loss. Hear Res. 2020;387:107858.
N Audiology Consensus Panel. Clinical practice guidelines for genetic hearing loss evaluation. JAMA Otolaryngol Head Neck Surg. 2022;148(3):234-241.
Brownstein Z, Avraham KB. Targeted next-generation sequencing for OTOA gene mutations in DFNB18A hearing loss. Mol Ther Nucleic Acids. 2021;24:178-189.
Friedman TB, Griffith AJ. Structure and function of genes causing hereditary hearing impairment. Nat Rev Genet. 2019;20(10):597-614.
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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy