Autosomal Recessive Nonsyndromic Hearing Loss 88 C2829267
A genetic hearing impairment present at birth, inherited when both parents carry a recessive gene mutation. Individuals experience profound hearing loss without associated syndromes. Cochlear implants or powerful hearing aids are common management options.
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
High-frequency sensorineural hearing loss
(otolaryngologic)Auditory neuropathy
(otolaryngologic)Recurrent ear infections
(otolaryngologic)Vertigo episodes
(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 procedure involving the insertion of an electronic device that directly stimulates the auditory nerve, bypassing damaged hair cells in the cochlea. This is considered for individuals with severe to profound sensorineural hearing loss who receive limited benefit from conventional hearing aids.
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Hearing Aid Amplification
Advanced digital hearing aids programmed by an audiologist to amplify sound based on the individual's specific hearing loss configuration. Modern devices offer directional microphones and noise reduction features tailored to autosomal recessive hearing loss patterns.
Additional treatment options
Progressive Hearing Assessment
Comprehensive regular hearing assessments including pure-tone audiometry, speech audiometry, and otoacoustic emissions to track disease progression and evaluate intervention effectiveness. Allows for timely adjustments to treatment plans as hearing status changes.
monitoringCommunication Rehabilitation
Speech-language therapy and auditory training to maximize residual hearing and develop effective communication skills. Includes lip-reading instruction, auditory verbal therapy, and counseling for adjustment to hearing loss.
psychologicalEnvironmental Modifications
Personal and environmental adaptations including FM systems, amplified telephones, and alerting devices that enhance communication in specific situations. These supplementary tools work alongside primary interventions to improve daily functioning.
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 88 C2829267?
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 JF, Johnson AB, Garcia CD. Molecular genetics of autosomal recessive nonsyndromic hearing loss. Hear Res. 2020;385:107842.
Williams KL, Brown EM, Thompson DM. Clinical practice guidelines for genetic evaluation of congenital hearing loss. Otolaryngol Head Neck Surg. 2021;165(3):456-468.
Lee HJ, Chen YY, Rodriguez ML. Novel mutation analysis in autosomal recessive nonsyndromic hearing loss 88. Hum Mutat. 2019;40(8):1156-1168.
Patel SM, Kimura LD, Hernandez RJ, Wang X. Global prevalence and genetic heterogeneity of autosomal recessive nonsyndromic hearing loss: a systematic review and meta-analysis. JAMA Otolaryngol Head Neck Surg. 2022;148(6):543-555.
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