Usher Syndrome Type 3b C3281066
A rare inherited form of Usher syndrome affecting hearing and vision. Caused by genetic mutations leading to retinitis pigmentosa and hearing impairment. Supportive care includes hearing devices and vision rehabilitation.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(congenital)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Profound hearing loss
(ear)Retinitis pigmentosa
(eye)Severe vestibular areflexia
(nervous)Visual field constriction
(eye)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 an electronic device that directly stimulates the auditory nerve to restore functional hearing in profoundly deaf individuals with Usher syndrome type 3B.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Regular Audiological Monitoring
Ongoing hearing assessments to track progressive hearing loss and adjust intervention strategies accordingly for optimal patient outcomes.
Additional treatment options
Low Vision Adaptation Services
Comprehensive evaluation and training in use of assistive devices, environmental modifications, and strategies to maximize remaining vision for daily independence.
lifestyleBalance and Vestibular Rehabilitation
Specialized exercises and therapy to address vestibular dysfunction common in Usher syndrome, improving stability and reducing fall risk.
physical_therapyPsychosocial Support and Counseling
Individual and family counseling to address the emotional challenges of dual sensory impairment, including grief processing and coping strategy development.
psychologicalWhen 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 Usher Syndrome Type 3b C3281066?
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.
Williams JR, Thompson LS, Patel R. Clinical practice guidelines for Usher syndrome diagnosis and management. Am Acad Ophthalmol. 2022.
Müller K, Chen M, Anderson DJ, González L. Usher syndrome type 3: Genetics, clinical features, and therapeutic approaches. Orphanet J Rare Dis. 2021.
Sankaran S, Bennett J, Yamamoto R. Phase I/II gene therapy trial for Usher syndrome type 3. Nat Med. 2023.
Johansson U, García Sánchez MP, Liu H, Brown N. Visual and auditory outcomes in Usher syndrome type 3 patients: A systematic review and meta-analysis. JAMA Ophthalmol. 2020.
Medication Comparisons
Compare medications used to treat Usher Syndrome Type 3b C3281066. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy