gms | German Medical Science

102. Jahrestagung der DOG

Deutsche Ophthalmologische Gesellschaft e. V.

23. bis 26.09.2004, Berlin

Evidence-based study on the ocular changes in Usher’s syndrome and their connection to the pathology in ear, vestibular analyzer and brain

Meeting Abstract

Search Medline for

  • corresponding author C. Vidinova - Clinic of Ophthalmology, Military Medical Academy,Sofia, Bulgaria
  • V. Zahov - ORL, Otoneurology Ward, St.Ivan Rilski University Hospital, Sofia, Bulgaria
  • K. Vidinov - Clinic of Ophthalmology, Military Medical Academy,Sofia, Bulgaria

Evidenzbasierte Medizin - Anspruch und Wirklichkeit. 102. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft. Berlin, 23.-26.09.2004. Düsseldorf, Köln: German Medical Science; 2004. Doc04dogP 139

The electronic version of this article is the complete one and can be found online at:

Published: September 22, 2004

© 2004 Vidinova et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.




To study the possible correlations between the severity of ocular changes in patients with Usher's syndrome and the extent of damage in the ear, vestibular analyzer and brain.


A prospective clinical study on 16 Usher patients was performed. They were examined with routine ophthalmologic techniques-VA, perimetry, colour vision tests and ERG. Furthermore a complete otoneurological examination, X-ray and magnetic resonance (MRI) were performed in all cases.


Our results showed that in patients with initial damage of the eye function: VA 20/20, slightly narrowed borders of the perimetry, ERG between 100-200 mV, a slowly progressing cochlear hearing loss was found. Symmetrical hyporeflexy of low to medium grade was commonly detected. The X-ray and MRI data showed cerebral and cerebellar atrophy and re-arrangements in the structure of the flat bones like in discrete osteoporosis. In patients with severe changes in the eye function: residual central visual field of 5-10 degrees, changes in the colour vision and very low ranges of ERG, ear and vestibular pathology was discrete. Pure tone threshold audiometry was almost normal for the age. In some cases narrowed and deformed optokinetic fields were detected. The atrophic changes in the brain were insignificant in comparison to the previous group, while bone structure re-arrangements were similar.


In our study no direct relation between the severity of eye and ear damage was found. In most cases one of the two analyzers was severely impaired, while the other was relatively spared. All patients were with different degree of brain atrophy and re-arrangements in the bone structure.