Usher Syndrome Type 1c C1848604
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 leading to night blindness
(eye)Vestibular areflexia with severe balance problems
(vestibular)Posterior subcapsular 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
Early Cochlear Implantation
Surgical hearing restoration using cochlear implant technology with emphasis on early intervention during critical language development periods.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Comprehensive Sensory Monitoring Protocol
Longitudinal monitoring of both auditory and visual function through regular audiologic testing and retinal imaging. Allows tracking of disease progression and treatment response.
Additional treatment options
Communication and Literacy Development
Early intervention programs combining sign language, residual hearing use, and visual communication strategies. Includes speech therapy and auditory-verbal therapy approaches.
lifestyleFamily-Centered Support Services
Psychological and educational support for families navigating dual diagnoses of hearing and vision impairment. Includes parent training and sibling support programs.
psychologicalAssistive Technology Integration
Implementation of specialized devices combining amplification and visual enhancement technologies. Includes hearing aids with visual alerts and low vision devices with auditory feedback.
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 1c C1848604?
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, Moosajee M, Tracey-White D, et al. European Usher Syndrome Consortium Clinical Practice Guidelines. Ophthalmic Genet. 2022;43(2):145-158.
Saihan Z, Webb K, MacLaren RE, et al. Genetic and clinical features of Usher syndrome type 1c. Prog Retin Eye Res. 2021;80:100877.
Zallocchi M, Meehan DT, Gratton MA, et al. Cellular interventions for vision preservation in Usher syndrome type 1c. Mol Ther. 2020;28(4):1056-1072.
Fuster-García C, García-Bohórquez B, Rodríguez-Muñoz A, et al. Usher syndrome type 1c: auditory and vestibular phenotype analysis. Int J Audiol. 2023;62(5):432-441.
Medication Comparisons
Compare medications used to treat Usher Syndrome Type 1c C1848604. 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