Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

Abstract

The Cogan syndrome is a rare disorder characterized by nonsyphilitic interstitial keratitis and audiovestibular symptoms. Profound sensorineural hearing loss has been reported in approximately half of the patients with the Cogan syndrome resulting in candidacy for cochlear implantation in some patients. The current study is the first histopathologic report on the temporal bones of a patient with the Cogan syndrome who during life underwent bilateral cochlear implantation. Preoperative MRI revealed tissue with high density in the basal turns of both cochleae and both vestibular systems consistent with fibrous tissue due to labyrinthitis. Histopathology demonstrated fibrous tissue and new bone formation within the cochlea and vestibular apparatus, worse on the right. Severe degeneration of the vestibular end organs and new bone formation in the labyrinth were seen more on the right than on the left. Although severe bilateral degeneration of the spiral ganglion neurons was seen, especially on the right, the postoperative word discrimination score was between 50 and 60% bilaterally. Impedance measures were generally higher in the right ear, possibly related to more fibrous tissue and new bone found in the scala tympani on the right side.

Hearing loss in inner ear and systemic autoimmune disease: A systematic review of post‐cochlear implantation outcomes

“…Bacciu noted that this ossification may not be identified on pre‐operative imaging, with 3 cases having clear imaging but findings of intraoperative osteogenesis 23 (all patients had Cogan's syndrome). Of the 14 studies (43 patients) that mentioned intra‐operative findings, 53.5% (23 patients) were found to have unilateral or bilateral fibrosis or osteogenesis of a section of the cochlea (14 Cogan's syndrome, 1 Vogt‐Koyanagi‐Harada syndrome, 1 neurosarcoidosis, 1 PAN, 6 not specified), 6,8,9,23,28,30‐35 10 of which required a drill out (7 Cogan's syndrome, 1 PAN, 2 unknown) 8,9,23,28,31 . In 6 patients, electrodes were still unable to be placed within the scala tympani (ST) and therefore the scala vestibuli (SV) was used (4 Cogan's syndrome, 1 Vogt‐Koyanagi‐Harada syndrome, 1 neurosarcoidosis) 6,23,30,32 .…”

Meta-Analysis—Correlation between Spiral Ganglion Cell Counts and Speech Perception with a Cochlear Implant

“…A total of 13 studies were found to contain relevant data [11,12,[15][16][17][19][20][21][22][23][24][25][26], of which 10 studies were from MEEI [12,[15][16][17][21][22][23][24][25][26]. Duplicate subjects were identified and removed from the analysis, which left a total of 81 subjects.…”

“…PRISMA 2009 flow diagram. A total of 13 studies were included in this quantitative synthesis [11,12,[15][16][17][19][20][21][22][23][24][25][26].…”

Behçet- und Cogan-Syndrom

“…Serologisch finden sich häufig Anti-körper gegen Antigene aus dem Innenohr, Hitzeschock-Protein 70 (HSP 70) oder auch das "Cogan-Peptid (CD148/Connexin 26 Homolog), sowie selten p-ANCA. Am ehesten handelt es sich hier jedoch um sogenannte "Bystander Effekte" [15,16,17].…”