Cochleosaccular Degeneration of the Inner Ear and Progressive Cataracts

Last update 01 Nov 2024

Basic Info

Synonyms

COCHLEOSACCULAR DEGENERATION WITH PROGRESSIVE CATARACTS, Cochleosaccular Degeneration, Cochleosaccular Degeneration of the Inner Ear with Progressive Cataracts

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Introduction

Rare syndrome with manifestation of progressive sensorineural hearing loss due to severe cochleosaccular degeneration and cataract. So far reported in two families. Transmission is autosomal dominant.

100 Clinical Results associated with Cochleosaccular Degeneration of the Inner Ear and Progressive Cataracts

100 Translational Medicine associated with Cochleosaccular Degeneration of the Inner Ear and Progressive Cataracts

0 Patents (Medical) associated with Cochleosaccular Degeneration of the Inner Ear and Progressive Cataracts

Literatures (Medical) associated with Cochleosaccular Degeneration of the Inner Ear and Progressive Cataracts

01 Dec 2020·BMC Veterinary ResearchQ2 · AGRICULTURAL & FORESTRY SCIENCE

The acoustically evoked short latency negative response (ASNR) in a unilaterally deaf cat with histologically-confirmed cochleosaccular degeneration

Q2 · AGRICULTURAL & FORESTRY SCIENCE

ArticleOA

Author: Bianchi, Ezio ; Poncelet, Luc ; Cantoni, Anna Maria

AbstractBackgroundA negative potential is occasionally recorded in humans and animals with profound deafness during brainstem auditory evoked potential (BAER) tests if loud intensities are used. This acoustically evoked short latency negative response (ASNR) is hypothesized to be of saccular origin. The sensitivity to sound of vestibular end organs is also used to produce vestibular evoked myogenic potentials (VEMP), a test that evaluates vestibular function. The same saccular origin is accepted also for VEMP.Case presentationA neutered male white domestic short hair cat presented with profound deafness and an ASNR in the left ear during BAER test performed when he was 8 months old. BAER tracings were substantially unchanged at the age of 12 years, immediately before euthanasia that was requested by the owner for the presence of an unrelated neoplastic disorder. The cat underwent a complete post-mortem necropsy including histopathology of the middle and inner ears. Histopathologic results confirmed the presence of a cochleosaccular degeneration of the left ear while the cochlea and sacculus of the right ear and the utriculus and semicircular canals of both ears were histologically normal.ConclusionsThis case report describes the auditory and histopathologic findings of a cat that showed an ASNR during BAER test despite the presence of cochleosaccular deafness. These results confirm that a saccular origin for the ASNR in this case, and in general in cats and dogs with congenital deafness associated with white pigmentation, is improbable. The hypothesis that the sacculus is the vestibular end organ responsible for the generation of the ASNR and VEMP in humans comes mainly from animal studies. The findings in this report may change the clinical interpretation of the results of BAER and VEMP not only in companion animals, but in humans as well.

01 Jan 2019·Canadian journal of veterinary research = Revue canadienne de recherche veterinaire

Cochleosaccular (Scheibe) dysplasia in dogs: A temporal bone study.

Article

Author: Paparella, Michael M ; Keskin, Nevra ; Sancak, Irem Gul ; Cureoglu, Sebahattin ; Albasan, Hasan

The objective of this study was to evaluate any otopathologic changes in temporal bone specimens from dogs with deafness related to cochleosaccular (Scheibe) dysplasia (CSD). We used the canine temporal bone collections of the Otopathology Laboratory at the University of Minnesota and of the Massachusetts Eye and Ear Infirmary at Harvard University in Boston. Our morphometric analysis included measuring the areas of the stria vascularis and the spiral ligament and counting the number of spiral ganglion cells. In addition, we noted the presence of the organ of Corti and cochlear hair cells, assessed the location of Reissner's membrane and the saccular membrane, and counted the number of both Type I and Type II vestibular hair cells in the macule of the saccule and vestibular ganglion cells. In the group of specimens from dogs with cochleosaccular dysplasia, we observed generalized degeneration in the cochlea and a significantly decreased number of Type I and Type II vestibular hair cells and vestibular ganglion cells. As hereditary deafness is presently untreatable with known therapeutic methods, dogs with cochleosaccular dysplasia should not be considered for breeding. Future therapeutic approaches, such as stem cell therapies, should be designed to target all the elements of the cochlea in addition to the saccule as it was found that both are affected in dogs with CSD.

01 Aug 2010·International Journal of Pediatric OtorhinolaryngologyQ3 · MEDICINE

Histopathological morphometric study of cochleosaccular dysplasia in Dalmatian dogs

Q3 · MEDICINE

Article

Author: Sebahattin Cureoglu ; Andre L.L. Sampaio ; Elizabeth Paine ; Michael M. Paparella ; Patricia A. Schachern ; Carolyn Sutherland ; Carlos A.C.P. Olivieira

OBJECTIVETo analyze temporal bones of deaf Dalmatian dogs from 5 days after birth to adulthood to better understand the pathogenesis of cochleosaccular dysplasia.METHODSThis is an experimental animal histopathological temporal bone study that included two groups of temporal bones. Group I consisted of 41 temporal bones from deaf Dalmatian dogs and group II of 25 temporal bones from 15 "normal" aged-matched, hearing Black Labradors. Morphometric analysis included: stria vascularis and spiral ligament area measurements, and cell counts of spiral ganglion, Scarpa's ganglion, and hair cells of saccular macula.RESULTSThe following findings were significantly less in deaf Dalmatian group compared to hearing Labradors: (1) cellular area of the stria vascularis in all cochlear turns; (2) cellular area of spiral ligament in the inferior part of the basal turn; (3) cellular density of spiral ganglion cells within segments III and IV; (4) number of Scarpa's ganglion cells; and (5) density of saccular hair cells types I and II. A borderline negative correlation was found between average density of spiral ganglion cells of segments III and IV and age in group I. Young deaf animals showed some cochlear hair cells, however in adult dogs all hair cells were replaced by supporting cells.CONCLUSIONGeneral pattern of cochleosaccular dysplasia is variable, even when only one etiology, the genetic one, is involved. The gradual degeneration of inner ear elements in the cochleosaccular degeneration might indicate that early intervention might be crucial to stop the progression of cochleosaccular dysplasia.

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Cochleosaccular Degeneration of the Inner Ear and Progressive Cataracts

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