Deterioration of hearing in a cochlear implantee with relapsing polychondritis

Mancini, Patrizia; Attanasio, Giuseppe; Viccaro, Marika; Filipo, R.

doi:10.3109/00016489.2010.545077

Cited by 7 publications

(9 citation statements)

References 10 publications

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“…CT imaging showed increased density in the cochlea, thought to be fibrosis/ossification of the modiolus. 12 In our case, a profound hearing and vestibular loss occurred just 6 weeks after the patient developed a clogged feeling in both ears, an unusually rapid progression compared to previously reported cases. It was unresponsive to steroid treatment, and cochlear implantation was performed about 1 month after the hearing loss.…”

Section: Discussionsupporting

confidence: 48%

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Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Akdoğan

Exilus

Ward

et al. 2021

Ann Otol Rhinol Laryngol

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Objectives: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. Methods: Case report and literature review. Results: A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation. Conclusions: Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.

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Section: Discussionsupporting

confidence: 48%

“…CT imaging showed increased density in the cochlea, thought to be fibrosis/ossification of the modiolus. 12…”

Section: Discussionmentioning

confidence: 99%

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Akdoğan

Exilus

Ward

et al. 2021

Ann Otol Rhinol Laryngol

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“…Theoretically, osteoneogenesis inside the cochlea could lead to an increase in electrical impedance over time, resulting in reduced CI efficiency and function. However, of the 85 patients (10 studies) in this review that were reported with consecutive audiometric data post‐operatively (or compared short term with long term follow‐up data), 30% 8,23,25,35 (26 patients) showed improvement in CI outcomes over a few years, 33% 11,27,42 (28 patients) reported patients with a “generally stable” hearing level over time, 35% 10,35,36 (30 patients) reported initial improvement up to 1 year and then plateauing or mild worsening of hearing thereafter, and 1.2% (1 patient) showed good initial response but complete deterioration due to pain after 18 months 21 . In one study, 11 a further sub‐group analysis suggested that cochlear implantation may initially show poor results in primary AIED, but then improve after 12 months; however this studies length of follow‐up (<2 years) may not be sufficient as symptomatic osteoneogenesis may be a lengthier process.…”

Section: Discussionmentioning

confidence: 98%

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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“…Although the underlying pathophysiology is felt to differ, there are case reports of delayed CI performance degradation in patients with Cogan’s syndrome and relapsing polychondritis; still, stable objective hearing outcomes are achieved in the majority of these cases. 29 , 30 , 31 …”

Section: Discussion Of Cochlear Implantation In Susac’s Syndromementioning

confidence: 99%

“…While no previous cases of cochlear implantation in SS have noted fluctuating CI performance, our experience prompts consideration of additional transient central pathology that compromises implant performance. Although the underlying pathophysiology is felt to differ, there are case reports of delayed CI performance degradation in patients with Cogan's syndrome and relapsing polychondritis; still, stable objective hearing outcomes are achieved in the majority of these cases 29–31 …”

Section: Discussion Of Cochlear Implantation In Susac's Syndromementioning

confidence: 99%

Scoping review of cochlear implantation in Susac's syndrome

Perez

McCall

Hirsch

2021

World j. otorhinolaryngol.-head neck surg.

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Objective Scoping review of published literature to establish clinical characteristics and audiologic outcomes in patients diagnosed with Susac’s Syndrome(SS) who have undergone cochlear implantation (CI). Data sources All published studies of CI in SS and contribution of two of our own patients who have not been reported previously. Methods A comprehensive search of MEDLINE (via PubMed) was carried out in March 2020 using the following keywords and related entry terms: Susac’s Syndrome, Cochlear Implantation. Results Our search identified a total of five case reports of CI in SS. With the addition of our two patients reported here, we analyzed characteristics and outcomes in seven patients. Mean age at implantation was 30 years old (range 19–46), with six women and one man implanted. Mean time from onset of hearing loss to implantation was 17 months (range three months to four years). Best reported postoperative speech understanding was reported via different metrics, with six of seven patients achieving open set speech scores of 90% or better, and one subject performing at 68%. Vestibular symptoms were present preoperatively in four of seven patients (57%), with vestibular testing reported in two patients, and showing vestibulopathy in one patient. No complications were reported following cochlear implantation. Conclusion Cochlear implantation is a viable option for hearing rehabilitation in patients with SS, with levels of attainment of open set speech comparable to other populations of CI candidates.

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Deterioration of hearing in a cochlear implantee with relapsing polychondritis

Cited by 7 publications

References 10 publications

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

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

Scoping review of cochlear implantation in Susac's syndrome

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