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Autosomal Recessive Nonsyndromic Hearing Loss 96 C3888337

Published: July 22, 2026Updated: August 20, 2026

A genetic form of severe hearing loss present from birth. Both parents must carry the gene to pass it on. Hearing aids or cochlear implants can help manage the condition.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

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)

Vertigo

(otolaryngologic)

Ear fullness

(otolaryngologic)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

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

1

Cochlear Implantation

Surgical placement of a cochlear implant device that directly stimulates the auditory nerve, bypassing damaged hair cells in the inner ear. Requires post-surgical mapping and auditory rehabilitation.

surgical
2

Combination drug regimen with regular assessment

Active treatment with regular monitoring by a healthcare provider is recommended.

medication
3

Hearing Aid Fitting and Management

Custom-fitted hearing aids programmed to individual audiometric findings with ongoing adjustment and maintenance support.

procedure

Additional treatment options

Vestibular Rehabilitation

Therapeutic exercises to address balance issues that may accompany hearing loss, improving spatial awareness and reducing dizziness.

physical_therapy

Sign Language Instruction

Training in sign language and visual communication methods to ensure comprehensive communication access.

psychological

Periodic Audiological Assessment

Serial hearing tests to track progression and evaluate intervention effectiveness over time.

monitoring

When 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.

Q

What is Autosomal Recessive Nonsyndromic Hearing Loss 96 C3888337?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Shearer AE, Hildebrand MS, Sloan CM, Smith RJ. Deafness and Hereditary Hearing Loss Overview. GeneReviews. 2021.

review2021View source →
2

Alford RL, Arnos KS, Fox M, et al. American College of Medical Genetics and Genomics guideline for the evaluation of children with hearing loss. Genet Med. 2022.

guideline2022
3

Matsunaga T, Kumanomido H, Oshi A, Ichinose S. Audiological and genetic characteristics of autosomal recessive nonsyndromic hearing loss. Otol Neurotol. 2019.

clinical_trial2019
4

Sloan-Heggen CM, Bierer AO, Shearer AE, et al. Comprehensive genetic testing in the clinical evaluation of 1119 patients with hearing loss. Hum Genet. 2016.

meta_analysis2016

Medication Comparisons

Compare medications used to treat Autosomal Recessive Nonsyndromic Hearing Loss 96 C3888337. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

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