Case study: cochlear implantation in cochlear otospongiosis

Marfatia, Hetal; Shah, Keya; Pareek, Ankur; Chatterjee, Chitra; Goyal, Pankaj

doi:10.1080/14670100.2019.1678894

Cited by 5 publications

(6 citation statements)

References 7 publications

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“…Table 6 reports each study’s results corresponding to their latest follow-up in the short-term. In all cases, the cochlear implant recipients were adults, except for the cases reported by Migirov et al 64 and Marfatia et al 54 , in which the individuals were 6 and 14 years old, respectively, at the time of surgery.…”

Section: Resultsmentioning

confidence: 85%

“…A non-successful outcome was related to a placement failure of the electrode array 65 . In all cases, the implantation was challenging because of ear hypervascularization, severe demineralization of the otic capsule, proliferation of the round window niche, and lack of anatomical landmarks typical of the OI population 27 , 53 , 54 , 66 . A high incidence of facial nerve stimulation after cochlear implantation was also common in people with OI 27 , 53 , 54 , 64 , 66 , 67 , and in three cases it was controlled through programming strategies 27 , 53 , 67 .…”

Section: Resultsmentioning

confidence: 99%

“…Hearing aids are widely used in the OI population until they show no longer benefit caused by the progressing hearing loss 8 , 22 , 31 , 54 , 70 . However, no study was found reporting data about their efficacy.…”

Section: Resultsmentioning

confidence: 99%

“…Documented otologic microsurgeries in some OI individuals also revealed tectorial membrane distortion and perilymph hemorrhage 52 . In other OI cases, sclerotic thickening of the cochlear otic capsule and a brittle scutum has been reported 53 , and demineralization of the bone around the cochlea and vestibule and partial ossification of the basal turn of the cochlea 54 . In OI, mixed hearing loss often arises with fenestral involvement causing conductive hearing loss and progresses toward the retrofenestral components with age, causing sensorineural hearing loss 6 , 8 , 12 , 21 , 24 .…”

Section: Introductionmentioning

confidence: 93%

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Treatments for hearing loss in osteogenesis imperfecta: a systematic review and meta-analysis on their efficacy

Ugarteburu

Cardoso

Richter

et al. 2022

Sci Rep

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About 70% of people with osteogenesis imperfecta (OI) experience hearing loss. There is no cure for OI, and therapies to ameliorate hearing loss rely on conventional treatments for auditory impairments in the general population. The success rate of these treatments in the OI population with poor collagenous tissues is still unclear. Here, we conduct a systematic review and meta-analysis on the efficacy of treatments addressing hearing loss in OI. This study conforms to the reporting standards of the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA). Data sources include published articles in Medline via PubMed, Web of Science, Scopus, and Embase, from their inception to November 2020. Studies included individuals with OI undergoing a hearing loss treatment, having pre- and postoperative objective assessment of hearing function at a specified follow-up length. Our search identified 1144 articles, of which 67 were reviewed at full-text screening. A random-effects meta-analysis was conducted on the selected articles (n = 12) of people with OI that underwent stapes surgery. Success was assessed as the proportion of ears with a postoperative Air–Bone Gap (ABG) ≤ 10 dB. A systematic review was conducted on the remaining articles (n = 13) reporting on other treatments. No meta-analysis was conducted on the latter due to the low number of articles on the topic and the nature of single case studies. The meta-analysis shows that stapes surgeries have a low success rate of 59.08 (95% CI 45.87 to 71.66) in the OI population. The systematic review revealed that cochlear implants, bone-anchored hearing aids, and other implantable hearing aids proved to be feasible, although challenging, in the OI population, with only 2 unsuccessful cases among the 16 reviewed single cases. This analysis of published data on OI shows poor clinical outcomes for the procedures addressing hearing loss. Further studies on hearing loss treatments for OI people are needed. Notably, the mechanisms of hearing loss in OI need to be determined to develop successful and possibly non-invasive treatment strategies.

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Section: Resultsmentioning

confidence: 85%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 93%

See 2 more Smart Citations

Treatments for hearing loss in osteogenesis imperfecta: a systematic review and meta-analysis on their efficacy

Ugarteburu

Cardoso

Richter

et al. 2022

Sci Rep

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show abstract

“…Thirty-eight studies had details of preimplantation imaging involving at least 289 patients (2,5,8,9,17,20–53).…”

Section: Resultsmentioning

confidence: 99%

Cochlear Implant Outcomes in Patients With Otosclerosis: A Systematic Review

et al. 2022

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ObjectiveThis study aimed to establish hearing outcomes after cochlear implantation in patients with otosclerosis.Materials and MethodsWe conducted a systematic review and narrative synthesis. Databases searched were as follows: MEDLINE, PubMed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits were placed on language or year of publication. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement.ResultsSearches identified 474 abstracts and 180 full texts, with 68 studies meeting the inclusion criteria and reporting outcomes in a minimum of 481 patients with at least 516 implants. Patient-reported outcome measures (PROMs) were reported in five studies involving 51 patients. Intraoperative adverse events/surgical approach details and preoperative radiological assessment were reported in 46 and 38 studies, respectively. The methodological quality of included studies was modest, predominantly consisting of case reports and noncontrolled case series with small numbers of patients. Most studies were Oxford Centre for Evidence Based Medicine grade IV.DiscussionAccess to good rehabilitation support is essential to achieving the good hearing outcomes and PROMs that can be expected by 12 months after implantation in most cases. There was a significant association between the radiological severity of otosclerosis and an increase in surgical and postoperative complications. Postoperative facial nerve stimulation can occur and may require deactivation of electrodes and subsequent hearing detriment.ConclusionsHearing outcomes are typically good, but patients should be counseled on associated surgical complications that may compromise hearing. Modern diagnostic techniques may help to identify potentially difficult cases to aid operative planning and patient counseling. Further work is needed to characterize PROMs in this population.

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A new phenomenon of cochlear otosclerosis: an acquired or congenital disease? – A clinical report of cochlear otosclerosis

Wei

Chen

et al. 2021

Acta Oto-Laryngologica

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Case study: cochlear implantation in cochlear otospongiosis

Cited by 5 publications

References 7 publications

Treatments for hearing loss in osteogenesis imperfecta: a systematic review and meta-analysis on their efficacy

Treatments for hearing loss in osteogenesis imperfecta: a systematic review and meta-analysis on their efficacy

Cochlear Implant Outcomes in Patients With Otosclerosis: A Systematic Review

A new phenomenon of cochlear otosclerosis: an acquired or congenital disease? – A clinical report of cochlear otosclerosis

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