Peer-reviewed veterinary case report
Cochleosaccular degeneration of the inner ear and progressive cataracts inherited as an autosomal dominant trait.
- Journal:
- The Laryngoscope
- Year:
- 1982
- Authors:
- Nadol, J B & Burgess, B
Plain-English summary
This study describes a new condition that causes both cataracts (cloudy lenses in the eyes) and progressive hearing loss due to problems in the inner ear. It appears to be inherited in a way that can be passed down from parents to offspring. In the case examined, the inner ear showed significant damage, particularly in parts called the cochlea and saccule, which are important for hearing and balance. This type of damage can happen before birth or develop later in life, and it may be caused by genetic factors or other issues. The findings suggest that this condition is not unique to humans and can also occur in animals.
Abstract
A new syndrome of cataracts and progressive sensorineural hearing loss, inherited as an autosomal dominant trait, is described. The histopathology of the inner ears in the proband demonstrated severe degeneration limited to the cochlea and saccule. This case represents the second reported example of genetically determined cochleosaccular degeneration in man. The cochleosaccular pattern of histopathology is found in both man and animals and may be the end result of prenatal dysplasia or postnatal degeneration. It also appears that cochleosaccular dysplasia and degeneration may be the result of genetically determined or acquired defects. Previously reported examples of cochleosaccular dysplasia and degeneration are reviewed including human cases in which the histopathology is similar to, but not characteristic of, the cochleosaccular pattern.
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Search related cases →Original publication: https://pubmed.ncbi.nlm.nih.gov/7121157/