Usher Syndrome Type 1f C1865885
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.
Generalized symptoms
(congenital)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Profound congenital hearing loss
(ear)Retinitis pigmentosa with peripheral visual field loss
(eye)Severe vestibular hypofunction
(vestibular)Ocular cataracts
(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 with Extended Electrode Array
Surgical procedure using specialized electrode arrays designed to preserve residual hearing while providing electrical stimulation, potentially beneficial for the specific auditory characteristics of type 1F.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
UV Protection and Light Management
Implementation of specialized eyewear with UV and blue light filtering, along with environmental lighting modifications to reduce light sensitivity and potentially slow retinal degeneration progression.
Additional treatment options
Low Vision Device Assessment
Comprehensive evaluation by low vision specialists to identify and prescribe appropriate assistive devices including bioptic telescopes, electronic readers, and smartphone applications for the visually impaired.
procedureTransition Planning Services
Age-appropriate programming addressing educational, vocational, and social transitions, particularly focused on building self-advocacy skills and preparing for adult services and employment.
psychologicalAssistive Technology Training
Instruction in use of technological aids including screen readers, refreshable Braille displays, and smart home systems to maintain independence in daily activities and information access.
complementaryWhen 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 1f C1865885?
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.
Sankila EM, Pakarinen MP. Molecular genetics of Usher syndrome subtypes. Ophthalmic Genet. 2020.
American Academy of Ophthalmology. Recommendations for genetic testing in inherited retinal diseases. Ophthalmology. 2022.
Bonnet C, Grati M. Clinical and molecular characterization of Usher syndrome type 1f patients. JAMA Ophthalmol. 2021.
Saunders CJ, Pieke FR. Gene therapy approaches for Usher syndrome. Hum Gene Ther. 2023.
Medication Comparisons
Compare medications used to treat Usher Syndrome Type 1f C1865885. 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