Autosomal Recessive Nonsyndromic Hearing Loss 71 C2748554
A genetic form of severe hearing loss present from birth. Both parents carry the recessive gene mutation. Treatment includes hearing aids, cochlear implants, and speech therapy.
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 bilateral hearing loss
(otolaryngologic)Prelingual deafness
(otolaryngologic)Absent transient evoked otoacoustic emissions
(otolaryngologic)Auditory neuropathy
(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 multi-electrode array into the cochlea to provide electrical stimulation of auditory neurons for profound hearing loss not corrected by conventional aids.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Hearing Aid Selection and Programming
Comprehensive evaluation and fitting of appropriately powered hearing aids with regular programming adjustments based on hearing test results.
Additional treatment options
Early Intervention Services
Developmentally appropriate therapy services including speech therapy, language intervention, and listening skills development for infants and young children with hearing loss.
physical_therapyFamily-Centered Counseling
Psychological support services addressing grief, adjustment, family dynamics, and educational planning for families affected by hereditary hearing loss.
psychologicalHearing Aid Efficacy Assessment
Regular evaluation of hearing aid performance including real-ear measurements, functional gain assessment, and user satisfaction surveys.
monitoringWhen 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 71 C2748554?
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, Brown CS, Smith RJH. Clinical practice guidelines for genetic evaluation of hereditary hearing loss. Genet Med. 2022.
Shearer AE, Hildebrand MS, Smith RJH. Hereditary hearing loss and deafness: molecular genetics and clinical management. Nat Rev Genet. 2021.
Azaiez H, Booth KT, Smith RJH. Next-generation sequencing identifies novel deafness-causing variants in patients with autosomal recessive nonsyndromic hearing loss. J Clin Invest. 2020.
Koffler T, Ushakov K, Avraham KB. Genetics of hearing loss: syndromic and nonsyndromic models. In: Pediatric Otolaryngology. Elsevier; 2019.
Medication Comparisons
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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