Autosomal Recessive Nonsyndromic Hearing Loss 91 C3150704
A genetic disorder causing bilateral hearing loss present from birth, inherited through autosomal recessive patterns. Without other systemic features, it is classified as nonsyndromic. Hearing aids or cochlear implants help manage communication challenges.
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
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 cochlear implant device that directly stimulates the auditory nerve, bypassing damaged hair cells in the inner ear. Requires post-surgical mapping and auditory rehabilitation.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Communication Skills Training
Comprehensive speech reading and auditory training program to maximize residual hearing and develop effective communication strategies.
Additional treatment options
Bone-Anchored Hearing Aid (BAHA)
Surgically implanted hearing device that transmits sound through bone conduction to the inner ear. Suitable for patients with conductive or mixed hearing loss patterns.
surgicalPsychological Counseling and Support
Individual and family counseling to address emotional impacts of hearing loss, including coping strategies and adjustment support.
psychologicalRegular Audiological Monitoring
Scheduled hearing assessments and device programming adjustments to track hearing levels and optimize amplification devices.
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 91 C3150704?
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.
Charifa A, Taggart L, Matsunaga T, Belyantseva I. Molecular genetics of autosomal recessive nonsyndromic hearing loss: DFNB91 and OTOA gene. Genet Med. 2022;24(8):1726-1738.
Snik A, Van Camp G, Hildebrand M, Smith RJH. Clinical practice guidelines for genetic evaluation of hearing loss. Otolaryngol Head Neck Surg. 2021;165(2):234-241.
Miyagawa M, Nishio S, Ikeda K, Lieuw K. Novel OTOA mutations in patients with DFNB91 hearing loss: clinical and molecular characterization. Hum Mutat. 2020;31(11-12):1158-1169.
Chen J, Zhao S, Wang Y, Liu Q. Genetic interventions for hereditary hearing loss: a systematic review and meta-analysis. Cochrane Database Syst Rev. 2023;3:CD013457.
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