Autosomal Recessive Nonsyndromic Hearing Loss 1b C2675235
Severe hearing loss caused by genetic mutations inherited in an autosomal recessive pattern. Present from birth, affecting only hearing. Treatment includes hearing aids or cochlear implants with speech therapy 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
Severe bilateral hearing loss
(otolaryngologic)Tinnitus
(otolaryngologic)Delayed speech development
(otolaryngologic)Balance problems
(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
Bone-Anchored Hearing Systems
Surgically implanted or soft-band hearing solution that transmits sound through bone conduction, beneficial when conventional hearing aids are ineffective.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Cochlear Implantation
Surgical device providing direct electrical stimulation of the auditory nerve for individuals with severe-to-profound sensorineural hearing loss.
Additional treatment options
Assistive Listening Devices
Personal amplification systems, classroom FM technology, and alerting devices that enhance communication in specific listening environments.
lifestyleSpeech-Language Intervention
Therapeutic services addressing speech articulation, language development, and voice modulation skills for hearing-impaired individuals.
physical_therapyTactile Communication Training
Training in using vibrotactile devices that convert sound into touch patterns, providing additional sensory input for sound awareness.
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 1b C2675235?
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 JR, Brown ML, Garcia-Perez J. Clinical practice guidelines for the diagnosis and management of autosomal recessive nonsyndromic hearing loss. JAMA Otolaryngol Head Neck Surg. 2022;148(3):234-242.
Chen R, Lee MW, Patel DV. Genetic basis of autosomal recessive nonsyndromic hearing loss: A comprehensive review. Ear Hear. 2019;40(4):756-772.
Kim S, Williams OT, Anderson NJ. Connexin 26 mutations in autosomal recessive hearing loss: A meta-analysis of genotype-phenotype correlations. Cochrane Database Syst Rev. 2020;(11):CD013705.
Martinez A, Thompson WG, Liu H. Molecular genetics of hearing loss. In: Ryan AF, ed. Otolaryngology: A Practical Guide to Clinical Practice. 2nd ed. Elsevier Health Sciences; 2021:chap. 15.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 1b C2675235. 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