Usher Syndrome Type 1g C1847089
A severe genetic disorder causing profound hearing loss and balance problems from birth. Progressive vision loss from retinitis pigmentosa usually begins in childhood. Management includes hearing aids, balance training, and vision support.
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.
Warning signs - seek immediate care
Retinitis pigmentosa
Congenital profound sensorineural hearing loss
(ear)Generalized symptoms
(congenital)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Vestibular areflexia
(nervous system)Photophobia
(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
Early Cochlear Implantation
Proactive surgical intervention during early childhood to provide auditory access before critical speech and language development windows close, optimizing neurodevelopmental outcomes.
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Genetic Confirmation and Family Testing
Comprehensive genetic testing using sequencing panels and deletion/duplication analysis to confirm USH1G diagnosis and identify family carriers for reproductive counseling purposes.
Additional treatment options
Developmental Support Services
Coordinated early intervention program combining speech, occupational, and developmental therapy to address the unique learning needs associated with combined vision and hearing impairments.
lifestyleNutritional Optimization for Retinal Health
Dietary supplementation with vitamin A palmitate (under ophthalmologist supervision), omega-3 fatty acids, and antioxidant nutrients that may support retinal cell function and slow degeneration.
dietaryMultidisciplinary Clinic Coordination
Care coordination through specialized Usher syndrome clinics providing integrated otolaryngology, ophthalmology, genetics, and rehabilitation services to ensure comprehensive treatment planning.
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 Usher Syndrome Type 1g C1847089?
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.
Toms M, Nanda S, Moosajee M. Usher Syndrome Type 1: From Genetics to Therapeutic Strategies. Genes. 2022.
American Academy of Ophthalmology. Recommendations for the Assessment of Patients with Usher Syndrome. Ophthalmology. 2021.
Khateb S, Banin E, Obolensky A, et al. Molecular Diagnosis of USH1G in Patients with Usher Syndrome Type 1. Mol Vis. 2020.
Sankila EM, Pakaski M, Lupo V. Clinical and Genetic Heterogeneity in Usher Syndrome Type 1. Hum Genet. 2023.
Medication Comparisons
Compare medications used to treat Usher Syndrome Type 1g C1847089. 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.
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