Cochlear implant in Cogan syndrome

Bovo, Roberto; Ciorba, Andrea; Trevisi, Patrizia; Aimoni, Claudia; Cappiello, Lucia; Castiglione, Alessandro; Govoni, Marcello

doi:10.3109/00016489.2010.535214

Cited by 32 publications

(24 citation statements)

References 19 publications

(34 reference statements)

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“…Cochlear implantation for patients with Cogan’s syndrome has been reported in several studies [Pasanisi et al, 2003; Aschendorff et al, 2004; Kontorinis et al, 2010; Wang et al, 2010; Bovo et al, 2011; Bacciu et al, 2015]. Generally, postoperative hearing outcomes have been reported to be good to excellent, as was true in the current case.…”

Section: Discussionsupporting

confidence: 67%

“…Generally, postoperative hearing outcomes have been reported to be good to excellent, as was true in the current case. However, intraoperative and postoperative complications such as partial obliteration of the cochlea [Pasanisi et al, 2003; Aschendorff et al, 2004; Kontorinis et al, 2010], flap complications including exposure of the implant [Wang et al, 1990; Kontorinis et al, 2010], and postoperative deterioration of auditory performance [Bovo et al, 2011] have been reported. In the current case, although the preoperative MRI scan showed hyperintense lesions within the basal turn of both cochleae, full insertion of the electrode was achieved on both sides.…”

Section: Discussionmentioning

confidence: 99%

“…Aschendorff et al [2004] have reported that fibrous obliteration may occur as early as eight weeks after onset of deafness. Bovo et al [2011] have reported two cases of postoperative deterioration of auditory performance of the cochlear implant. In one case, deterioration of speech perception was attributed to progressive cochlear ossification.…”

Section: Discussionmentioning

confidence: 99%

“…Fibrous obliteration of the scala tympani was found as early as eight weeks after onset of complete deafness [Aschendorff et al, 2004]. Although deterioration of auditory performance after cochlear implantation has been described [Bovo et al, 2011], presumably secondary to an ongoing inflammatory response due to Cogan’s syndrome, stable postoperative hearing has been reported with follow-up of one year [Pasanisi et al, 2003], two years [Wang et al, 2010] and five years [Bacciu et al, 2015] with good to excellent word and sentence recognition scores suggesting that cochlear implantation provides excellent and stable hearing rehabilitation with long term follow-up in most patients.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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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.

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Section: Discussionsupporting

confidence: 67%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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“…For patients who progressed to deafness, cochlear implantation is indicated as soon as possible, because after a period of time fibrosis of organ of corti may appear and it can be difficult or impossible to perform cochlear implantation [7,[12][13][14][15].…”

Section: Discussionmentioning

confidence: 99%

Atypical Cogan's syndrome: A case report and summary of current treatment options

Jančatová

Zeleník

Komínek

et al. 2015

International Journal of Pediatric Otorhinolaryngology

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

Lee

Biggs

Muzaffar

et al. 2021

Laryngoscope Investig Oto

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Objectives To assess outcomes following cochlear implantation (CI) in patients with hearing loss secondary to primary or secondary autoimmune inner ear disease (AIED). Methods A systematic review and narrative synthesis was completed according to PRISMA guidelines. Databases searched included MEDLINE, PubMed, EMBASE, Web of Science, Cochrane Collection, and http://clinicaltrials.gov. No limits were placed on year of publication or language. Results A total of 551 studies were identified, of which 29 were included after removal of duplicates, and screening the title, abstract, and full text. All except one study were OCEBM grade IV. 114 of 115 patients displayed improvement in hearing following cochlear implantation. With implant use, roughly a third of these patients had hearing that improved over time, a third improved and plateaued, and a third remained stable. There was no additional risk of perioperative complications found in AIED patients compared what is generally accepted in general cochlear implantation, although two episodes of device failure after 6 months were noted, and four patients with secondary AIED displayed poor initial audiological outcomes. Conclusion CI in both primary and secondary AIED provides marked improvement in hearing. Early CI may be a valid management option, provide long‐lasting hearing in patients and reduce the side effects of long‐term systemic immunosuppressants. However, patients should be counseled residual hearing may be lost if there is cochlear ossification or fibrosis which may make implant insertion more traumatic. Level of Evidence NA.

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Cochlear implant in Cogan syndrome

Cited by 32 publications

References 19 publications

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

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

Atypical Cogan's syndrome: A case report and summary of current treatment options

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

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