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Retinitis pigmentosa and deafness.

R P Mills, D M Calver

    Journal of the Royal Society of Medicine
    |January 1, 1987
    PubMed
    Summary

    Retinitis pigmentosa (RP) patients with hearing loss often have Usher syndrome, showing cochlear damage. Other RP-related syndromes may affect different auditory system parts, suggesting varied lesion locations.

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    Area of Science:

    • Ophthalmology
    • Audiology
    • Genetics

    Background:

    • Retinitis pigmentosa (RP) is a group of inherited retinal diseases.
    • Hearing loss can be associated with certain RP subtypes.
    • Understanding the auditory involvement in RP is crucial for comprehensive patient care.

    Purpose of the Study:

    • To investigate audiological findings in patients with retinitis pigmentosa.
    • To identify patterns of hearing loss in different RP-related syndromes.

    Main Methods:

    • Audiological assessment of 17 patients diagnosed with retinitis pigmentosa.
    • Categorization of hearing loss based on audiological patterns.
    • Correlation of audiological findings with specific RP syndromes.

    Main Results:

    • Nine out of 17 RP patients exhibited significant hearing loss.
    • Six patients with hearing loss were diagnosed with Usher syndrome, presenting a cochlear pattern.
    • Three patients with hearing loss had Senior's syndrome, Kearne-Sayre syndrome, or Lawrence-Moon-Biedle syndrome; two had absent stapedius reflexes.

    Conclusions:

    • Hearing loss is a notable comorbidity in retinitis pigmentosa.
    • Usher syndrome is frequently associated with cochlear-type hearing loss in RP patients.
    • Distinct RP-associated syndromes may involve different anatomical sites within the auditory system.

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