NCT00016471

Brief Summary

Hearing loss and loss of vision can be very harmful to the well-being and life of people who suffer from them. Usher syndrome is the name of a disease where people have both hearing loss and visual loss. In fact more than half of people who are deaf and blind have Usher syndrome. In this study we are trying to find the causes of all types of Usher syndrome and to learn more about how the eyes and ears work. Usher syndrome is caused by changes in our genes that lead to mistakes in the functioning of our eyes and ears. We may conduct hearing tests called audiograms to test hearing and a vision test called an electroretinogram (ERG) to test how well the retina (the part of your eye that senses light) is working on participants in the study. From these tests we can tell what kind of Usher syndrome a participant may have. We will then get DNA from participants by drawing blood. The DNA will be studied, along with DNA from members of the participant's family and other families, to try to find the gene that is causing Usher syndrome in the participant. Once the gene is found we will be able to study it to learn more about how the eyes and ears work. If a subject has already been diagnosed we may just need copies of their medical records and blood can be drawn locally. In order to increase the power of the study and the likelihood of detecting relevant genes participants will be taken from the Ashkenazi Jewish population group only. This will make it much easier to find the genes.

Trial Health

87
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Timeline
Completed

Started Mar 2001

Shorter than P25 for all trials

Geographic Reach
1 country

1 active site

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Start

First participant enrolled

March 1, 2001

Completed
2 months until next milestone

First Submitted

Initial submission to the registry

May 7, 2001

Completed
2 days until next milestone

First Posted

Study publicly available on registry

May 9, 2001

Completed
9 months until next milestone

Study Completion

Last participant's last visit for all outcomes

February 1, 2002

Completed
Last Updated

June 24, 2005

Status Verified

November 1, 2003

First QC Date

May 7, 2001

Last Update Submit

June 23, 2005

Conditions

Interventions

AudiogramPROCEDURE

Eligibility Criteria

Age0 Years+
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • Any Ashkenazi Jewish individual with combined hearing and visual loss that is or may be any type of Usher syndrome, or a family member of said individual.

You may not qualify if:

  • Any individual who is not an Ashkenazi Jew or does not have combined hearing and visual loss or whose disease has been previously determined not to be Usher syndrome.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

Mount Sinai School of Medicine

New York, New York, 10029, United States

Location

Related Publications (2)

  • Ness SL, Ben-Yosef T, Bar-Lev A, Madeo AC, Brewer CC, Avraham KB, Kornreich R, Desnick RJ, Willner JP, Friedman TB, Griffith AJ. Genetic homogeneity and phenotypic variability among Ashkenazi Jews with Usher syndrome type III. J Med Genet. 2003 Oct;40(10):767-72. doi: 10.1136/jmg.40.10.767. No abstract available.

    PMID: 14569126BACKGROUND
  • Ben-Yosef T, Ness SL, Madeo AC, Bar-Lev A, Wolfman JH, Ahmed ZM, Desnick RJ, Willner JP, Avraham KB, Ostrer H, Oddoux C, Griffith AJ, Friedman TB. A mutation of PCDH15 among Ashkenazi Jews with the type 1 Usher syndrome. N Engl J Med. 2003 Apr 24;348(17):1664-70. doi: 10.1056/NEJMoa021502. No abstract available.

    PMID: 12711741BACKGROUND

MeSH Terms

Conditions

Usher SyndromesRetinitis Pigmentosa

Interventions

Hearing

Condition Hierarchy (Ancestors)

Deaf-Blind DisordersDeafnessHearing LossHearing DisordersEar DiseasesOtorhinolaryngologic DiseasesHearing Loss, SensorineuralSensation DisordersNeurologic ManifestationsNervous System DiseasesBlindnessVision DisordersRetinal DystrophiesRetinal DegenerationRetinal DiseasesEye DiseasesAbnormalities, MultipleCongenital AbnormalitiesCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesEye Diseases, HereditaryGenetic Diseases, InbornSigns and SymptomsPathological Conditions, Signs and Symptoms

Intervention Hierarchy (Ancestors)

Vestibulocochlear Physiological PhenomenaPhysiological PhenomenaSensationNervous System Physiological PhenomenaMusculoskeletal and Neural Physiological Phenomena

Study Design

Study Type
observational
Observational Model
DEFINED POPULATION
Sponsor Type
NIH

Study Record Dates

First Submitted

May 7, 2001

First Posted

May 9, 2001

Study Start

March 1, 2001

Study Completion

February 1, 2002

Last Updated

June 24, 2005

Record last verified: 2003-11

Locations