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Retinitis pigmentosa and progressive hearing loss
The Journal of Speech and Hearing Disorders
|August 1, 1983
Summary
Usher syndrome can present with progressive hearing loss after speech development, not just before. This study explores these cases and offers diagnostic and rehabilitation strategies for individuals with Usher syndrome and acquired hearing loss.
Area of Science:
- Ophthalmology
- Audiology
- Genetics
Background:
- Usher syndrome typically involves prelingual deafness and retinitis pigmentosa.
- Some patients present with postlingual, progressive hearing loss alongside retinitis pigmentosa.
Purpose of the Study:
- To investigate Usher syndrome variants with postlingual hearing loss.
- To present case histories and audiological data.
- To offer diagnostic and rehabilitative approaches for these patients.
Main Methods:
- Case study analysis.
- Audiologic assessments.
- Review of diagnostic and rehabilitative techniques.
Main Results:
- Confirmation of Usher syndrome variants with progressive postlingual hearing loss.
- Detailed audiologic findings in affected individuals.
- Evaluation of tailored interventions for dual sensory impairment.
Conclusions:
- A subset of Usher syndrome patients exhibits postlingual, progressive hearing loss.
- Early diagnosis and intervention are crucial for managing Usher syndrome with acquired hearing loss.