Histopathology of the inner ear in Usher's syndrome as observed by light and electron microscopy
- PMID: 3717858
- DOI: 10.1177/000348948609500321
Histopathology of the inner ear in Usher's syndrome as observed by light and electron microscopy
Abstract
A case of Usher's syndrome, type 3, is described. Histologic study of the inner ear of the patient demonstrated hair cell loss in the basal turn, severe loss of spiral ganglion cells, widespread neural degeneration in the cochlea, and discrete collections of degenerating supporting cells in the organ of Corti. The pattern of neural degeneration bore some similarity to abnormalities in the retina in retinitis pigmentosa. However, the findings in the supporting cells had no obvious parallel to the findings in the retina in this disorder. The histopathology of Usher's syndrome has been equated with the Scheibe, or cochleosaccular, pattern of degeneration. However, the findings in the present case and a critical analysis of published data did not support this concept.
Similar articles
-
Innervation densities of inner and outer hair cells of the human organ of Corti. Evidence for auditory neural degeneration in a case of Usher's syndrome.ORL J Otorhinolaryngol Relat Spec. 1988;50(6):363-70. doi: 10.1159/000276014. ORL J Otorhinolaryngol Relat Spec. 1988. PMID: 3231458
-
Application of electron microscopy to human otopathology. Ultrastructural findings in neural presbycusis, Menière's disease and Usher's syndrome.Acta Otolaryngol. 1988 May-Jun;105(5-6):411-9. doi: 10.3109/00016488809119494. Acta Otolaryngol. 1988. PMID: 3400443
-
Histopathology of the human inner ear in Alström's syndrome.Audiol Neurootol. 2015;20(4):267-72. doi: 10.1159/000381935. Epub 2015 Jun 24. Audiol Neurootol. 2015. PMID: 26111748 Free PMC article.
-
Histopathologic features of the temporal bone in usher syndrome type I.Arch Otolaryngol Head Neck Surg. 2000 Aug;126(8):1018-23. doi: 10.1001/archotol.126.8.1018. Arch Otolaryngol Head Neck Surg. 2000. PMID: 10922238 Review.
-
Usher's syndrome.Ophthalmic Paediatr Genet. 1990 Mar;11(1):71-6. doi: 10.3109/13816819009012950. Ophthalmic Paediatr Genet. 1990. PMID: 2190135 Review.
Cited by
-
Cochlear histopathology in human genetic hearing loss: State of the science and future prospects.Hear Res. 2019 Oct;382:107785. doi: 10.1016/j.heares.2019.107785. Epub 2019 Aug 19. Hear Res. 2019. PMID: 31493568 Free PMC article. Review.
-
Rescue of Outer Hair Cells with Antisense Oligonucleotides in Usher Mice Is Dependent on Age of Treatment.J Assoc Res Otolaryngol. 2018 Feb;19(1):1-16. doi: 10.1007/s10162-017-0640-x. Epub 2017 Oct 12. J Assoc Res Otolaryngol. 2018. PMID: 29027038 Free PMC article.
-
Accelerated age-related olfactory decline among type 1 Usher patients.Sci Rep. 2016 Jun 22;6:28309. doi: 10.1038/srep28309. Sci Rep. 2016. PMID: 27329700 Free PMC article. Clinical Trial.
-
Usher syndrome type I associated with bronchiectasis and immotile nasal cilia in two brothers.J Med Genet. 1993 Mar;30(3):253-4. doi: 10.1136/jmg.30.3.253. J Med Genet. 1993. PMID: 8474110 Free PMC article.
-
Evoked otoacoustic emissions behaviour in retinitis pigmentosa.Doc Ophthalmol. 1994;87(2):167-76. doi: 10.1007/BF01204794. Doc Ophthalmol. 1994. PMID: 7835186
Publication types
MeSH terms
Grants and funding
LinkOut - more resources
Full Text Sources
