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Literature DB >> 3560119

Retinitis pigmentosa and deafness.

Abstract

Seventeen patients with retinitis pigmentosa (RP) have been investigated audiologically. Of 9 found to have a significant hearing loss, 6 were examples of Usher's syndrome; these patients had a cochlear pattern of hearing loss. The other 3 were examples of Senior's syndrome, Kearne-Sayre syndrome and Lawrence-Moon-Biedle syndrome respectively. Two of these patients had absent stapedius reflexes. It is suggested that patients with different RP-deafness syndromes may have lesions in different parts of the auditory system.

Entities: Chemical

Mesh：

Year: 1987 PMID： 3560119 PMCID： PMC1290625 DOI： 10.1177/014107688708000108

Source DB: PubMed Journal: J R Soc Med ISSN： 0141-0768 Impact factor: 18.000

18 in total

1. Usher's syndrome. (Retinitis pigmentosa and deafness). A temporal bone report.

Authors: A Belal
Journal: J Laryngol Otol Date: 1975-02 Impact factor: 1.469

2. Retinal degeneration combined with obesity, diabetes mellitus and neurogenous deafness: a specific syndrome (not hitherto described) distinct from the Laurence-Moon-Bardet-Biedl syndrome: a clinical, endocrinological and genetic examination based on a large pedigree.

Authors: C H ALSTROM; B HALLGREN; L B NILSSON; H ASANDER
Journal: Acta Psychiatr Neurol Scand Suppl Date: 1959

3. Retinitis pigmentosa, external ophthalmophegia, and complete heart block: unusual syndrome with histologic study in one of two cases.

Authors: T P KEARNS; G P SAYRE
Journal: AMA Arch Ophthalmol Date: 1958-08

Retinitis pigmentosa and deafness.

1. Usher's syndrome. (Retinitis pigmentosa and deafness). A temporal bone report.

2. Retinal degeneration combined with obesity, diabetes mellitus and neurogenous deafness: a specific syndrome (not hitherto described) distinct from the Laurence-Moon-Bardet-Biedl syndrome: a clinical, endocrinological and genetic examination based on a large pedigree.

3. Retinitis pigmentosa, external ophthalmophegia, and complete heart block: unusual syndrome with histologic study in one of two cases.

4. Usher's syndrome with unusual otologic manifestations.

5. Mitochondrial cytopathy. A multisystem disorder with ragged red fibres on muscle biopsy.

6. The renal lesion of the Laurence-Moon-Biedl syndrome.

7. Heterogeneity of retinal degeneration and hearing impairment syndromes.

8. Vestibular and auditory function in Usher's syndrome.

9. Usher syndrome in four hard-of-hearing siblings.

10. Morphogenesis of the retinal rods; an electron microscope study.

1. The electroretinogram.