Cochleosaccular degeneration with progressive cataracts

Cochleosaccular Degeneration with Progressive Cataracts is a rare medical condition characterized by the progressive loss of hearing and vision due to the degeneration of the cochlea and saccule in the inner ear and the development of cataracts in the eyes.

Overview

Cochleosaccular degeneration is a condition that affects the inner ear, specifically the cochlea and the saccule. These are two key structures involved in hearing and balance. Degeneration of these structures leads to progressive hearing loss and balance problems.

Progressive cataracts refer to the gradual clouding of the lens in the eye, leading to a decrease in vision. Cataracts are a common condition, particularly in older adults, but in the case of cochleosaccular degeneration with progressive cataracts, the cataracts develop at a younger age and progress more rapidly.

Symptoms

The primary symptoms of cochleosaccular degeneration with progressive cataracts are hearing loss and vision loss. The hearing loss is typically sensorineural, meaning it is caused by damage to the inner ear or the nerve pathways from the inner ear to the brain. The vision loss is due to the development and progression of cataracts.

Other symptoms may include balance problems, due to the role of the cochlea and saccule in maintaining balance, and tinnitus, a ringing or buzzing noise in the ears.

Causes

The exact cause of cochleosaccular degeneration with progressive cataracts is unknown. It is likely to be a genetic condition, as it often runs in families. However, the specific genes involved have not been identified.

Treatment

Treatment for cochleosaccular degeneration with progressive cataracts focuses on managing the symptoms. Hearing aids may be used to improve hearing, while cataract surgery can restore vision. Balance problems may be managed with physical therapy.

See also

• Cochlea
• Saccule
• Cataract
• Hearing loss
• Sensorineural hearing loss
• Tinnitus
• Cataract surgery
• Hearing aid
• Physical therapy

NIH genetic and rare disease info

• NIH info on Cochleosaccular degeneration with progressive cataracts

Cochleosaccular degeneration with progressive cataracts is a rare disease.

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