Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Cochlear implants in young children with Usher's syndrome

N M Young1, J C Johnson, M B Mets

  • 1Division of Pediatric Otolaryngology, Children's Memorial Medical Center, Chicago, Illinois, USA.

The Annals of Otology, Rhinology & Laryngology. Supplement
|September 1, 1995
PubMed
Summary

Early cochlear implantation benefits children with Usher syndrome (US), a genetic disorder causing hearing loss and vision impairment. Prompt diagnosis through ophthalmologic evaluation is key for successful intervention before vision loss occurs.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Symposium on Pellagra.

Buffalo medical journal·2023
Same author

Some Observations on Gastric and Duodenal Ulcer.

Buffalo medical journal·2023
Same author

Antikamnia.

The Southern medical record·2022
Same author

The Real and Relative Value of Our Recent Antipyretics.

Atlanta medical and surgical journal (1884)·2022
Same author

Perinatal fluoxetine exposure results in social deficits and reduced monoamine oxidase gene expression in mice.

Brain research·2019
Same author

Incidence of variations in human cadaveric renal vessels.

Folia morphologica·2017

Area of Science:

  • Ophthalmology
  • Audiology
  • Genetics

Background:

  • Usher syndrome (US) is an autosomal recessive disorder causing congenital sensorineural hearing loss and retinitis pigmentosa.
  • Individuals with US often have severe to profound hearing loss, making them candidates for cochlear implantation.
  • Early identification and intervention are crucial for managing dual sensory deficits.

Observation:

  • A pediatric otology program focused on early identification and cochlear implantation for children with US.
  • Comprehensive ophthalmologic evaluations, including electroretinography, were used for early US diagnosis.
  • Four out of 19 pediatric cochlear implant recipients with congenital hearing loss were diagnosed with US.

Findings:

  • These four children with US were implanted before any visual symptoms manifested.

Related Experiment Videos

  • All implanted children demonstrated measurable benefits from cochlear implantation.
  • Early diagnosis facilitated timely intervention for Usher syndrome patients.

    • Implications:

    • Early cochlear implantation in Usher syndrome patients can significantly improve auditory outcomes.
    • Ophthalmologic screening is vital for identifying US in children with congenital hearing loss.
    • Addressing both hearing and vision impairments early is critical for individuals with Usher syndrome.