Cochlear implantation among patients with otosclerosis: a systematic review of clinical characteristics and outcomes.

PURPOSE: There have been considerable advancements in cochlear implants in different clinical scenarios; however, their use in patients with otosclerosis remains challenging. This review aimed to investigate the surgical and clinical outcomes of cochlear implantation in patients with otosclerosis. METHODS: An electronic literature search was performed using four main databases through February 2021 to identify original studies of cochlear implantation in patients with otosclerosis for inclusion in this systematic review. The study protocol was registered with the Prospectively Registered Systematic Reviews and Meta-analyses (reference number: CRD42021234753). RESULTS: A total of 23 studies including 3162 patients were enrolled. Of these patients, only 392 had otosclerosis and underwent cochlear implantation. The duration of deafness was reported in only eight studies, extending up to 50 years. Far-advanced otosclerosis was observed in 153 patients. A total of 56 patients used hearing aids. Stapedectomy and stapedotomy were performed in 118 and 63 patients, respectively. In three studies, the temporary success of stapedectomy and stapedotomy was 6 (43%) and 5 (71%) patients, respectively. Computed tomography was used as a preoperative assessment tool in most studies (n = 14, 60.9%). Incomplete implant insertion occurred in 17 patients, while facial nerve stimulation occurred in 36 patients after implantation. CONCLUSION: Cochlear implantation is a relatively safe modality that can provide promising audiological outcomes in patients with otosclerosis. However, several factors, including cochlear ossification, duration of deafness, and previous operations, can affect its outcomes. Further studies with a larger sample population are recommended.

[The results of bilateral cochlear implantation in the children who survived meningitis]. / Rezul'taty bilateral'noi kokhlearnoi implantatsii u detei, perenesshikh meningit.

The objective of the present study was the prospective analysis of the results of bilateral cochlear implantation (CI) in the children presenting with bilateral ossification of the cochlea after they had survived meningitis. A total of 15 patients underwent the surgical intervention. In those exhibiting bilateral ossification of the basal cochlear helix over the 5 mm segment (up to first bend of the cochlear turn) and partial ossification of the second helix (in 6 children), the affected portions were removed with the placement of two choleostomies, the lower one (from the ossified membrane of the cochlear window) and the upper one (toward the second helix). Activation of the speech processors of the CI systems was carried out within 4-6 weeks after surgery. The hearing abilities of the children were evaluated in accordance with the 'Estimation of the auditory perception categories', 'Estimation of the child's apprehension capacity', and 'Analysis of speech intelligibility rating' guidelines. In all the children with ossification over less than 5 mm of the basal cochlear helix, it proved possible to introduce the whole intracochlear electrode grid whereas only half of the electrode array was implanted in the cases of overall ossification of the basal helix. The first results obtained by telemetry and surdopedagogical testing gave evidence of the possibility of identifying various sources of non-verbal and speech stimuli in all the treated children at a small (up to 3 meters) distance.

Cochlear implantation in patients with Cogan syndrome: long-term results.

The objective of this study was to evaluate the long-term outcomes of patients with Cogan syndrome (CS) who have undergone cochlear implantation. Subjects consisted of 12 cochlear implant users with a typical form of CS. Measures included word and sentence recognition scores. The speech recognition performance was rated before cochlear implantation and at 1 and 5 years after implantation. The speech materials were presented in quiet only condition. The mean 12-month post-operative word and sentence recognition scores were 91.4 and 93.1%, respectively. Five years after implantation, the group means for word and sentence recognition tests were 94 and 96.3%, respectively. No patients in this series experienced flap complication or other local or systemic complications. This long-term study on 12 subjects with CS over 5 years of cochlear implant use reveals that cochlear implantation is safe in the long term and provides excellent and stable hearing results.

Evolution of speech perception in patients with ossified cochlea and short array cochlear implant.

Background: Short array cochlear implant is indicated as rehabilitation in patients with severe to profound deafness, especially when there is cochlear ossification. In these cases, with reduced intracochlear patency, total insertion becomes more difficult, requiring the use of this type of electrode (15 mm). Few studies have been published to evaluate auditory performance, presenting controversial audiological results.Aims/Objectives: To report the speech perception of users of cochlear implants (CI) with short array. Material and Methods: A retrospective analysis of medical records of patients who underwent surgery for cochlear implantation with a short array, between 2009 and 2020, at the Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo (HRAC-USP) was carried out. Results: There was performance evolution in the speech perception tests in the data analysis. Meningitis and congenital hearing loss were the main indications for CI in the sample. Conclusion. CI with a short array is an alternative in the management of patients with a history of cochlear ossification and severe or profound sensorineural hearing loss. Significance: To demonstrate the evolution of speech perception tests with short array cochlear implant in patients with or without ossified cochlea and its characteristics for application in clinical practice.

Special electrodes for demanding cochlear conditions.

Optimal matching of an electrode array to the cochlear anatomy plays a key role in bringing the best benefit of CI technology to the users. Even within the category of normal anatomy cochlea, the size variation is huge justifying MED-EL's FLEX electrode array to be available in five different lengths. Within the malformed inner-ear category the anatomical variation is huge, convincing MED-EL to custom-design the electrode array as per the request from the operating surgeons. Thanks to G. Bredberg, M. Beltrame, L. Sennaroglu, J. Gavilan, S. Plontke, T. Lenarz, J. Müller, and few others for their valuable suggestions on unique electrode designs satisfying various needs. Translational research efforts at MED-EL in cooperation with CI surgeons from across the world led to the implantation of a variety of electrode array designs in patients with special cochlear needs.

Estudo anatômico da cóclea para confecção de instrumental para a cirurgia de implante coclear com 2 feixes de eletrodos em cócleas ossificadas / Cochlear anatomy study used to design surgical instruments for cochlear implants with two bundles of electrodes in ossified cochleas

A ossificação da cóclea, decorrente principalmente de meningite, impede a inserção completa do implante coclear convencional. Os implantes com 2 feixes de eletrodos mais curtos do que o convencional foram desenvolvidos especialmente para cócleas ossificadas. Porém, durante essa cirurgia há um grande risco de lesão da artéria carótida interna (ACI). Portanto, a medida da profundidade das cocleostomias para inserir os dois feixes de eletrodos aumentaria a segurança desse procedimento. OBJETIVOS: 1) Obter as distâncias entre as cocleostomias e a ACI em ossos temporais de cadáver; 2) Confeccionar instrumento que possa ser usado na cirurgia de implante coclear com 2 feixes de eletrodos. FORMA DE ESTUDO: Experimental prospectivo. MATERIAL E MÉTODO: Em 21 ossos de cadáveres foi realizada: 1) mastoidectomia cavidade aberta; 2) cocleostomias nos giros basal e médio da cóclea; 3) identificação da ACI; 4) medida da distância entre as cocleostomias e a artéria. RESULTADOS: A medida média ± desvio padrão obtida para o túnel superior foi 8,2 ± 1,1mm e para o túnel inferior foi 8,1± 1,3mm. A menor distância encontrada foi 6,5mm para o túnel superior e 6,0mm para o túnel inferior. CONCLUSÃO: Apesar dos parâmetros calculados concluímos que a melhor medida para ser considerada na confecção do instrumento cirúrgico serão as mínimas medidas obtidas em cada um dos giros cocleares, pois é a maneira mais segura para evitar a lesão da ACI, que pode ser fatal.

Cochlear ossification, mainly secondary to meningitis, prevents the complete conventional cochlear implant insertion. Implants with two electrode bundles shorter than the conventional ones were specifically developed for ossified cochleas. However, during surgery there is a high risk of damaging the internal carotid artery (ICA). Therefore, measuring cochleostomy depth in order to insert the two electrode bundles would greatly increase the procedure's safety. AIMS: 1) Find the distances between cochleostomies and ICA in cadaver temporal bones. 2) Design an instrument that can be used in cochlear implant surgery to introduce an implant with two bundles of electrodes. STUDY DESIGN: Experimental prospective. MATERIALS AND METHODS: In 21 temporal bones from cadavers we performed: 1) canal wall down mastoidectomy; 2) cochleostomy in the cochlear basal and middle turns; 3) ICA identification; 4) Length determination between the cochleostomies and the artery. RESULTS: the average distance ± standard deviation obtained for the upper tunnel was of 8.2 ± 1.1 mm and for the lower tunnel it was of 8.1± 1.3 mm. The shortest distance found was of 6.5 mm for the upper tunnel and 6.0 mm for the lower tunnel. CONCLUSION: Despite the values calculated, we concluded that the best value to be considered in creating a surgical instrument are the minimum lengths obtained for each one of the cochlear turns, because this is the safest way to avoid damaging the ICA, that can be fatal.