Autosomal Recessive Nonsyndromic Hearing Loss 5 C1833319
Severe hearing loss present from birth caused by genetic mutations passed from carrier parents. This nonsyndromic form affects hearing only. 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
Profound congenital hearing loss
(otolaryngologic)Difficulty with speech discrimination
(otolaryngologic)Auditory neuropathy spectrum disorder features
(otolaryngologic)Otitis media with effusion
(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
CIC or ITC Hearing Aids
Completely-in-canal or in-the-canal custom hearing devices offering cosmetic discretion while providing amplification for mild-to-moderate loss.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Middle Ear Implant System
Surgically placed device that vibrates middle ear structures directly, useful when conventional aids cannot be used or provide insufficient benefit.
Additional treatment options
Auditory Rehabilitation Program
Structured therapy combining listening exercises, speech reading training, and communication strategies to maximize hearing device benefit.
physical_therapyOtoacoustic Emissions Monitoring
Non-invasive testing measuring cochlear outer hair cell function to monitor disease progression and treatment effectiveness over time.
monitoringCommunication Partner Training
Education for family members, educators, and coworkers on effective communication techniques including speech reading support and environmental modifications.
complementaryWhen 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 5 C1833319?
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.
Morton CC, Nance WE. Newborn hearing screening—a silent revolution. N Engl J Med. 2016.
Shearer AE, Hildebrand MS, Sloan CM, Smith RJ. Deafness in the genomics era. Hear Res. 2011.
Tekin M, Arnos KS, Pandya A. Advances in hereditary deafness. JAMA. 2012.
Dror AA, Avraham KB. Hearing impairment: a panoply of genes and functions. Neuron. 2010.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 5 C1833319. 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