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OBJECTIVE: This study aims to investigate patterns of cochlear ossification (CO) in cadaveric temporal bones of patients who underwent vestibular schwannoma (VS) surgery via the translabyrinthine (TL), middle cranial fossa (MF), or retrosigmoid (RS) approaches. STUDY DESIGN: Histopathologic analysis of cadaveric temporal bones. SETTING: Multi-institutional national temporal bone repository. METHODS: The National Institute of Deafness and Communication Disorders and House Temporal Bone Laboratory at the University of California, Los Angeles and the Massachusetts Eye and Ear Otopathology Laboratory were searched for cadaveric temporal bones with a history of VS for which microsurgery was performed. Exclusion criteria included non-VS and perioperative death within 30 days of surgery. Temporal bones were analyzed histologically for CO of the basal, middle, and apical turns. RESULTS: Of 92 temporal bones with a history of schwannoma from both databases, 12 of these cases met the inclusion criteria. The approaches for tumor excision included 2 MF, 4 RS, and 6 TL approaches. CO was observed in all temporal bones that had undergone TL surgery. Among temporal bones that had undergone MF or RS surgeries, 5/6 had no CO, and 1/6 had partial ossification. This single case was noted to have intraoperative vestibular violation after RS surgery upon histopathologic and chart review. CONCLUSION: In this temporal bone series, all temporal bones that had undergone TL demonstrated varying degrees of CO on histological analysis. MF and RS cases did not exhibit CO except in the case of vestibular violation. When cochlear implantation is planned or possible after VS surgery, surgeons may consider using a surgical approach that does not violate the labyrinth.

Background. Nowadays cochlear implantation (CI) is a globally recognized method of rehabilitation of people with deafness, which allows not only to improve the quality of life of the patient, but also to integrate them into the hearing environment. Ossification of the labyrinth is one of the most challenging problems faced by a surgeon during a CI. Patients with cochlear ossification have a set of problems that an audiologist encounters. Among them: duration of deafness; previous difficulties with hearing aids due to lack of effectiveness and severity of discomfort when wearing a hearing aid; severity of damage to the microstructures of the ear, leading to the need to use high levels; potential incomplete insertion of an electrode or insertion of an electrode through a cochleostomy; the occurrence of non-auditory sensations in the form of stimulation of the facial nerve; Functional neuroimaging can provide insight into the neurobiological factors that contribute to differences in individual hearing outcomes after cochlear implantation. To date, measuring neural activity in the auditory cortex of cochlear implant users has been a difficult task, primarily because the use of traditional neuroimaging techniques is limited. Functional near-infrared spectroscopy (fNIRS) is a new technology that offers advantages to this group of patients because it is non-invasive, compatible with CI devices and is not subject to electrical interference. The knowledge gained using this technique makes it possible to objectify the quality of auditory-speech rehabilitation of patients by fixing the activity of the cerebral cortex. Aim. To describe a clinical case of a patient with subtotal ossification of the cochlea with an assessment of auditory-speech rehabilitation using neuroimaging techniques. Materials and methods. In November 2022, patient M. was admitted to Saint-Petersburg Research Institute of Ear, Throat, Nose and Speech with complaints of hearing loss up to deafness, tinnitus, discomfort when wearing hearing aids. It was known from the anamnesis that the patient had suffered from purulent meningitis of unknown etiology at the age of 5. The patient was monitored by sign-language specialists for a long time, and from the age of 15 she had hearing aids on both ears without significant effect. In 2021, she noted a complete loss of the effectiveness of hearing aids, and therefore decided to undergo an examination for the CI. The patient's speech was represented by complex sentences with agrammatism, speech perception was mainly performed visually by lip reading. CT scans of the temporal bones from 27.03.2022 revealed signs of subtotal ossification of the labyrinth structures on both sides with unreliable signs of partial patency of the spiral canal of the cochlea on the right. Due to the high risks of incomplete insertion of the electrode, up to the impossibility of its introduction, the decision on surgical treatment was made by a concilium consisting of 2 otosurgeons, an anesthesiologist, an audiologist and a neurologist. Intraoperatively, it was decided to use a short straight electrode with a length of 11 mm. Good auditory-speech results were obtained, the objectivity of the data obtained was confirmed by the method of near-infrared functional spectroscopy. Conclusion. The fNIRS technique made it possible to obtain an objective picture of the reaction of the temporal lobe cortex specifically to sound stimulation in a patient with RP without the use of invasive or radiological research methods, which in some cases may have an application error. We believe that the use of this technique is promising in patients with profound hearing loss before and after cochlear implantation.

This study describes the clinical profile, operative findings, surgical technique, type of implant and complications encountered in implantation of abnormal cochlea by the transcanal (veria) technique in a tertiary care centre. This is a retrospective study done amongst 337 patients who underwent cochlear implantation by veria technique between January 2013 to August 2023. Based on pre-operative imaging with high-resolution computed tomography and magnetic resonance imaging the cochleovestibular malformations in this study were classified according to Sennaroglu classification and the cochlear ossification was graded from I to IV. Amongst the 337 patients who underwent cochlear implantation during the study period there were 79 cases (23.4%) with abnormalities of cochlea. This included 45 patients (13.4%) with congenital malformations, 20 patients (5.9%) with cochlear fibrosis and 14 patients (4.2%) with cochlear ossification. The commonest cochlear malformation was Incomplete Partition 2 which was present in 21 patients (46.7%). The other common malformations were cochlear hypoplasia (31.1%), Incomplete Partition 1(13.3%) and common cavity (8.9%). Out of the 20 patients with cochlear fibrosis, 17 patients had fibrosis only in the basal turn. Out of the 14 patients with cochlear ossification 8 patients (57.1%) had a grade 1 ossification, 2 patients (14.3%) had a grade 2 ossification and 4 patients (28.6%) had a grade 3 ossification. One of the patients required re-exploration for correcting the placement of electrode. The type of implant were chosen depending on individual malformation or length of ossification and fibrosis. In the past, inner ear anomalies were considered as a contraindication for cochlear implantation however it is now possible to implant most of these abnormal cochlea by careful planning, a modification of the surgical technique and the type of electrode used. [ABSTRACT FROM AUTHOR]

Objective: To elucidate the differences in auditory performance between auditory brainstem implant (ABI) patients with tumor or nontumor etiologies. Data Sources: PubMed, Embase, and Web of Science Core Collection from 1990 to 2021. Review Methods: We included published studies with 5 or more pediatric or adult ABI users. Auditory outcomes and side effects were analyzed with weighted means for closed‐set, open‐set speech, and categories of auditory performance (CAP) scores. Overall performance was compared using an Adult Pediatric Ranked Order Speech Perception (APROSPER) scale created for this study. Results: Thirty‐six studies were included and underwent full‐text review. Data were extracted for 662 tumor and 267 nontumor patients. 83% were postlingually deafened and 17% were prelingually deafened. Studies that included tumor ABI patients had a weighted mean speech recognition of 39.2% (range: 19.6%‐83.3%) for closed‐set words, 23.4% (range: 17.2%‐37.5%) for open‐set words, 21.5% (range: 2.7%‐48.4%) for open‐set sentences, and 3.1 (range: 1.0‐3.2) for CAP scores. Studies including nontumor ABI patients had a weighted mean speech recognition of 79.8% (range: 31.7%‐84.4%) for closed‐set words, 53.0% (range: 14.6%‐72.5%) for open‐set sentences, and 2.30 (range: 2.0‐4.7) for CAP scores. Mean APROSPER results indicate better auditory performance among nontumor versus tumor patients (3.5 vs 3.0, P =.04). Differences in most common side effects were also observed between tumor and nontumor ABI patients. Conclusion: Auditory performance is similar for tumor and nontumor patients for standardized auditory test scores. However, the APROSPER scale demonstrates better ABI performance for nontumor compared to tumor patients. [ABSTRACT FROM AUTHOR]

Background: Kenny-Caffey Syndrome type 2 (KCS2) is a genetic disease affecting bone metabolism. However, cochlear implantation (CI) results have yet to be published in detail. Objective: This study presents the gene, clinical characteristics, surgical outcomes, and literature review of 2 patients with sensorineural hearing loss related to KCS2. To enhance diagnostic detection and accuracy, we also compare the differential diagnosis between KCS2, otosclerosis, and Cogan's syndrome (CS). Methods: Prior to CI, patients with KCS2 and CS underwent comprehensive audiological and radiological evaluations. Postoperative auditory speech outcomes and impedance values were recorded and analyzed statistically. A systematic search of the literature was conducted to summarize clinical characteristics. Results: Patients diagnosed with KCS2 exhibit more pronounced changes in the inner ear. The impedance values in the KCS2 cohort were considerably higher (Mean = 12.13 kΩ) than those with CS (Mean = 8.8 kΩ) one year post-activation. The literature review exhibits the clinical manifestations associated with KCS2. Conclusion: CI is an effective treatment for KCS2 to restore hearing loss. More frequent programming and accurate adjustment of stimulation is of great necessity. A thorough examination, including temporal bone HRCT, 3D-MRI, audiological evaluations, and whole-exome sequencing, is essential for the diagnosis and treatment of KCS2. [ABSTRACT FROM AUTHOR]

Purpose: In cochlear implantation, a scala vestibuli (SV) insertion of an electrode array is a rare occurrence and is reported to be linked to poor hearing outcomes. Using the same electrode array, the auditory performance of patients with a complete SV location was compared with that of patients having a complete scala tympani (ST) location 1 year after implantation. Methods: Thirty-three patients were included in this retrospective case–control study (SV, n = 12; ST, n = 21). The matching criteria were electrode array type, age at implantation, and duration of severe or profound deafness. The array location was analyzed using 3D reconstruction of postoperative CT scans. Postoperative audiological evaluation of the implanted ear was performed using pure-tone audiometry, speech recognition of monosyllabic words in quiet, and words and sentences in noise. Results: On the preoperative CT scan, six patients in the SV group presented with both round window (RW) and ST ossification, three with RW ossification alone, and three with no RW ossification. Auditory performance did not differ between SV and ST groups 1 year after cochlear implantation. Speech recognition of words was 49 ± 7.6% and 56 ± 5.0% in quiet and 75 ± 9.5% and 66 ± 6.0% in noise in SV and ST groups, respectively. Conclusion: ST insertion is the gold standard that allows the three cochlear scalae to preserve scalar cochlear integrity. However, 1 year after implantation, a planned or unexpected SV insertion is not detrimental to hearing outcomes, providing similar auditory performance in quiet and noise to ST insertion. [ABSTRACT FROM AUTHOR]

Introduction: In morphologically normal-appearing inner ears, measurements of the distance between the round window and carotid canal (RCD), the maximum diameter of the basal turn of the cochlea next to the round window (BD), and the thickness of the promontory (PT) just lateral to the basal turn may be used as guide for safe cochleostomy and implant placement.Materials and Methods:From January to March 2022, a cross-sectional observational study was carried out in a tertiary care hospital. The round window to carotid canal distance (RCD), the largest diameter of the cochlea's basal turn next to the round window (BD), and the thickness of the promontory immediately lateral to the basal turn (PT) were measured using CT temporal bone images of 150 persons without cochlear abnormalities. The values obtained were compared using Paired T-test for significance of difference between both genders and sides.Results:A total of 150 participants—75 men and 75 women—with a mean age of 37.5 years were enrolled in the study. With a range of 7.18 mm to 10.52 mm, the mean RCD was 8.84 mm (SD 0.8 mm). The mean BD was 2.27 mm (SD 0.4 mm), while the mean PT was 1.15 mm (SD 0. mm). The values obtained did not differ significantly in both the genders and the right and left sides (p = 0.37 and 0.24, respectively).Conclusion: The present study has defined and calculated pertinent measures at cochleostomy site that will aid safe electrode insertion and prevent misplacement.

Temporal bone fracture can cause posttraumatic deafness. Sequelae like ossification or obliteration of the cochlea can impact the outcome of cochlear implantation. This study highlights the effect of localisation of the fracture to morphologic, electric and functional criteria. The study group consists of patients suffering from hearing loss caused by temporal bone fracture (n = 61 ears). Patients were divided into otic capsule sparing (OCS) and otic capsule involving (OCI) fractures. The OCI group was additionally divided into subgroups with or without signs of ossification inside the cochlea. Postoperative imaging, hearing tests and electrode impedances were analysed. The results of postoperative hearing rehabilitation showed lower speech understanding scores for the OCI group, especially for the ossification group. OCI fractures with signs of ossification showed increased impedances. Patients in the OCI group suffered more frequently from facial nerve stimulation (FNS). FNS was most frequently observed within the ossification group. Cochlear implantation in patients with temporal bone fracture is adequate therapy for the treatment of fracture-induced deafness. In long-term observation, these patients show comparable results with regular cochlear implant (CI) patients. Implantation should be performed as soon as possible after hearing loss, before obstructing obliteration or ossification of the cochlea start. [ABSTRACT FROM AUTHOR]

Introduction: In morphologically normal-appearing inner ears, measurements of the distance between the round window and carotid canal (RCD), the maximum diameter of the basal turn of the cochlea next to the round window (BD), and the thickness of the promontory (PT) just lateral to the basal turn may be used as guide for safe cochleostomy and implant placement. Materials and Methods: From January to March 2022, a cross-sectional observational study was carried out in a tertiary care hospital. The round window to carotid canal distance (RCD), the largest diameter of the cochlea's basal turn next to the round window (BD), and the thickness of the promontory immediately lateral to the basal turn (PT) were measured using CT temporal bone images of 150 persons without cochlear abnormalities. The values obtained were compared using Paired T-test for significance of difference between both genders and sides. Results: A total of 150 participants--75 men and 75 women--with a mean age of 37.5 years were enrolled in the study. With a range of 7.18 mm to 10.52 mm, the mean RCD was 8.84 mm (SD 0.8 mm). The mean BD was 2.27 mm (SD 0.4 mm), while the mean PT was 1.15 mm (SD 0. mm). The values obtained did not differ significantly in both the genders and the right and left sides (p = 0.37 and 0.24, respectively). Conclusion: The present study has defined and calculated pertinent measures at cochleostomy site that will aid safe electrode insertion and prevent misplacement. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory disease involving multiple organs. Some studies have reported otological manifestations of IgG4-RD, although most studies describe involvement of the middle ear, and reports on inner ear manifestations are limited. Here, we describe a case of a 30-year-old man with IgG4-RD involving the inner ear. This case demonstrated that IgG4-RD affected the inner ear and caused cochlear ossification. Cochlear implants may be considered for milder cases, and hormone and immunosuppressive therapy may control disease progression. [ABSTRACT FROM AUTHOR]

Purpose: To report on our experience with the slim modiolar electrode (SME) especially focusing on the wide range of etiologies including inner ear anomalies, tumors, ossifications, and even revision surgeries. Methods: All the cochlear implantation cases performed from June 2018 to September 2019 by a single surgeon was prospectively recruited. The molecular/radiological etiology of hearing loss, intraoperative outcomes, and radiographic studies of cases where the SME was implanted was reviewed to evaluate compatibility of SME for the wide range of etiologies. For cases where SME replaced the other electrode as a revision, audiologic assessment was also made. Results: Among the 99 ears implanted during the study period, the SME was successfully implanted in 86 ears. These SME cases comprised inner ear anomaly/cochear nerve deficiency (n = 21) including cochlear hypoplasia type IV with the modiolus, intracochlear schwannoma (n = 1), far advanced otosclerosis (n = 1) and 7 revision cases. The SME was successfully used in 7 revision surgeries to replace the existing electrode. Shorter spiral diameter and decreased intracochlear position index for SME was found compared with their previous electrodes. Four out of the 6 patients who received revision implantation showed better speech perception after their surgeries. Conclusion: The SME can be implanted in any cases unless the integrity of the modiolus is totally compromised. Due to its slim design and tight modiolar-hugging feature, good functional outcome can also be anticipated. Additionally, it is suitable for revision surgeries possibly allowing better hearing outcomes which may be attributed to its closer proximity to the modiolus. [ABSTRACT FROM AUTHOR]

Cochlear ossification (CO) after meningitis can cause profound sensorineural hearing loss (SNHL). Cochlear implantation (CI) is the ideal treatment strategy for CO. To explore the strategy for CI in patients with CO after meningitis. In this retrospective study, the medical records of patients diagnosed with profound SNHL due to CO after meningitis and who underwent CI in our department between September 2010 and September 2021 were collected and reviewed. Their imaging and surgical findings were analyzed. The data of 26 patients with unilateral CI were reviewed. All patients underwent preoperative temporal high-resolution computed tomography (HRCT) and 22 patients magnetic resonance imaging (MRI). The sensitivity of HRCT was 61.5% (10/26), whereas that of MRI was 81.8% (18/22). Combined HRCT and MRI achieved a detection rate of 92.3% (24/26). Twenty-two and four patients underwent complete and partial electrode implantations, respectively. Preoperative temporal bone HRCT and MRI are essential for determining whether a patient is suitable for CI and surgical planning. A false-negative diagnosis is possible when diagnosing CO, but combined HRCT and MRI can improve the positive rate of preoperative diagnosis of CO post meningitis. Early CI is recommended. [ABSTRACT FROM AUTHOR]

BACKGROUND: The aim of this study was to evaluate the surgical and auditory outcomes of cochlear implantation in patients with cochlear ossification. METHODS: This study comprised 54 patients with cochlear ossification who underwent cochlear implantation in the cochlear implant center of a tertiary care hospital between January 1998 and May 2019. Clinical data were evaluated including surgical findings and audiological performances. The auditory outcomes of the implanted patients were assessed through the Categories of Auditory Performance-II test and Speech Intelligibility Rating test, respectively. The outcomes of patients with cochlear ossification were compared with those of 54 patients selected for the control group who underwent implantation with no cochlear ossification. RESULTS: Auditory outcomes were comparable between the study group and the control group. The control group obtained significantly higher scores than those of the study group when compared using the Categories of Auditory Performance-II test and Speech Intelligibility Rating test batteries. Patients with meningitis produced poorer outcomes within the group comparisons of the study group. None of the patients experienced surgical complications. The extent of ossification was analyzed in terms of its effectiveness on audiological performance. Patients with complete ossification had significantly lower Categories of Auditory Performance-II and Speech Intelligibility Rating test scores. CONCLUSION: Cochlear implantation is a safe and beneficial procedure, even in patients with cochlear ossification. The ossified cochlea may require varied drill techniques beyond traditional implantation surgery for the insertion of the electrode array. It is, however, still difficult to predict audiological outcomes in patients with cochlear ossification. [ABSTRACT FROM AUTHOR]

Conventional cochlear implantation (CI) is challenging in patients with coincidental anatomical or inflammatory middle ear pathologies. Performing subtotal petrosectomy (STP) simultaneously with CI allows to effectively overcome aforementioned problems. The aim of this study is to describe the benefits of simultaneous STP for our CI patients, review its indications, and analyze short-term outcomes. We describe five cases of successful CI using STP method, the indications for operation were advanced otosclerosis, vestibular schwannoma, chronic mastoiditis, cholesteatoma and previous implant bed infection. Five patients had a follow-up of 7–16 months. We had minor complications, which did not require medical intervention. We provide analysis of surgical strategy, postoperative complications and auditory performance of those patients. In this study, we found clear advantages of STP for our selected CI candidates. These were better exposure in cases with modified anatomy, radical eradication of the underlying disease and reduction of the secondary infection risk.

Introduction: Preoperative imaging, besides audiological evaluation, plays a major role in evaluation of candidacy for auditory implants, and in particular cochlear implants. It is essential to assess whether the basic criteria necessary for implantation are met. Diagnostic imaging is crucial not only in determining candidacy, but also determining the feasibility of cochlear implantation as it allow to anticipate surgical difficulties which could preclude or complicate the implantation of the device. The aim of the study is to present the protocol for the evaluation of preoperative imaging studies with particular focus on the factors potentially affecting clinical decisions in children qualified for cochlear implantation. Material and method: Preoperative imaging studies of 111 children performed prior to cochlear implantation were analyzed: high-resolution computed tomography (HRCT) of temporal bones and MRI. The assessment was made according to the presented protocol. Results: Pathologies and anomalies identified during the assessment of preoperative imaging studies significantly altered clinical decisions in 30% of patients. In the study group, in 17% of patients inner ear malformations were identified. 2.7% of children were disqualified from a cochlear implantation due to severe congenital inner ear malformations. 9% of the patients have had bacterial meningitis. In 50% of them difficulties related to complete or progressive cochlear ossification occurred. In 4.5% of patients less common surgical approaches other than mastoidectomy with a posterior tympanotomy were applied. Discussion: Preoperative imaging allow for the identification of significant pathologies and anomalies affecting qualification decisions and further treatment. HRCT and MRI are complementary to each other for preoperative imaging. The two modalities in combination allow accurate and optimal evaluation of the anatomical structures prior to implantation. Inner ear malformations and cochlear ossification following meningitis are relatively frequently encountered in children qualified for a cochlear implant. [ABSTRACT FROM AUTHOR]

Conventional cochlear implantation (CI) is challenging in patients with coincidental anatomical or inflammatory middle ear pathologies. Performing subtotal petrosectomy (STP) simultaneously with CI allows to effectively overcome aforementioned problems. The aim of this study is to describe the benefits of simultaneous STP for our CI patients, review its indications, and analyze short-term outcomes. We describe five cases of successful CI using STP method, the indications for operation were advanced otosclerosis, vestibular schwannoma, chronic mastoiditis, cholesteatoma and previous implant bed infection. Five patients had a follow-up of 7–16 months. We had minor complications, which did not require medical intervention. We provide analysis of surgical strategy, postoperative complications and auditory performance of those patients. In this study, we found clear advantages of STP for our selected CI candidates. These were better exposure in cases with modified anatomy, radical eradication of the underlying disease and reduction of the secondary infection risk. [ABSTRACT FROM AUTHOR]

Background/aim: To emphasize the role of cochlear implantation (CI) in the auditory rehabilitation of patients with otosclerosis (OS) and share our surgical experiences on this rare group of patients. Materials and methods: Retrospective analysis of the patients who have a diagnosis of otosclerosis and implanted between January 1998-May 2019 was performed. Preoperative and postoperative clinical, radiological, audiological and surgical findings are presented. Results: Among 2195 patients who have been implanted in our institution, 12 (0.54%) met the diagnostic criteria of OS according to their preoperative (clinical, radiological, audiological) and peroperative (surgical) findings. Electrode insertion was performed via "round window membrane and cochleostomy" in 8 and 4 patients, respectively. No major complications occured. All patients showed satisfactory performances by means of audiometric scores postoperatively. Nonauditory stimulation (NAS) which manifested as "facial twitching" was a challenging problem in one patient during the surgery and subsided after the operation. Conclusion: Our experience on CI in patients with OS revealed that the implantation was a relatively safe procedure and had satisfactory impact on audiological performances. [ABSTRACT FROM AUTHOR]

Abstract Objectives Bacterial meningitis can cause a labyrinthitis. Consequences often are intracochlear soft tissue neoformation (cochlear obliteration) or intracochlear osteoneogenesis (cochlear ossification) and deafness. Cochlear implantation becomes challenging and hearing rehabilitation is complicated. This retrospective case-control-study aimed to find correlations between morphologic, electric and functional parameters. Methods The study group included children, who lost hearing due to a bacterial meningitis (n = 35 cases). Using preoperative computed tomography and intraoperative findings we grouped into ‘unaltered cochleae’, ‘obliterated cochleae’ and ‘ossified cochleae’. Control group children suffered from deafness (n = 16) of other aetiology and presented with radiologically unchanged cochleae. Postoperative routine controls documented impedances, stimulation charge and hearing tests a various time points, which all were analysed. Results Control group patients showed a mean impedance of 6.3 kΩ and the mean charge applied was 19 nC. The study group averaged at 7.9 kΩ and 24.6 nC respectively. Patients with ossified cochleae had increased values of 8.6 kΩ and 29.7 nC. The control group reached a monosyllabic word understanding of 74% and the study group of 58%. Patients with ossified cochleae reached 36%. Conclusions Impedances and stimulation charge influence each other. Increased charge is necessary for higher cochlear implant output. Despite higher charges, patients with obliterated and patients with ossified cochleae significantly perform worse in hearing rehabilitation. Reduced audiological outcome in study group patients without morphologic cochlear changes furthermore hints at additional factors besides cochlear tissue neogenesis like postinflammational changes at the neural pathway. [ABSTRACT FROM AUTHOR]

Introduction: An auditory brainstem implant (ABI) is an option for auditory rehabilitation in patients with totally ossified cochleae who cannot receive a conventional cochlear implant. Objective: To evaluate the outcomes in audiometry and speech perception tests after the implantation of an ABI via the extended retrolabyrinthine approach in patients with postmeningitis hearing loss. Materials and methods: Ten patients, including children and adults, with postmeningitis hearing loss and bilateral totally ossified cochleae received an ABI in a tertiary center from 2009 to 2015. The extended retrolabyrinthine approach was performed in all the patients by the same surgeons. A statistical analysis compared pure tonal averages and speech perception tests before and at least 12 months after the ABI activation. Results: Eight patients (80%) showed improvements in tonal audiometry and the word and vowel perception tests after an average follow-up of 3.3 years. Two patients recognized up to 40% of the closed-set sentences without lip-reading. Two patients had no auditory response. Conclusions: The ABI improved hearing performance in audiometry and speech perception tests in cases of postmeningitis hearing loss. The extended retrolabyrinthine approach is a safe surgical option for patients with postmeningitis hearing loss and bilateral totally ossified cochleae. [ABSTRACT FROM AUTHOR]

Objective Vestibular schwannomas (VSNs) account for the vast majority of lesions located in the cerebellopontine angle (CPA). The goals of VSN surgery are possible total tumor removal, intact facial nerve function, and preservation of cochlear nerve function. In cases of pre- or postoperative deafness, restoration of hearing with auditory brainstem implant (ABI) or cochlear implant (CI) is a promising treatment option with normally better results in CI than in ABI. The aimof this retrospective study is to evaluate cochlear ossification or obliteration secondary to a retrosigmoid approach, which is important for later CI, especially in single-sided deafness. Materials and Methods We retrospectively reviewed data from our suboccipital retrosigmoid operation database for the period from January 2008 to February 2015. A total of 65 patients with VSN could be analyzed retrospectively. The patient's data (age, gender, side of operation, tumor entities, and the duration of follow-up) were evaluated. The most recent T2-weigted MRI exams were taken into account for the evaluation of cochlea. To compare left and right sides, the coronal reformatted images were reconstructed in a symmetrical way. Results Twenty-two out of 65 (33%) patients had cochlear obliteration in our survey. The cochlear obliteration was more common in females (44 vs. 20% in males). The median follow-up was 28 months. The size and the intrameatal extension of tumors could not be considered as factors influencing the cochlear obliteration rate. According to our survey, 33% of suboccipital approaches manifested cochlear obliteration to some extent. It was more common in females. The intrameatal extension of tumors played statistically no role in the occurrence of postoperative cochlear obliteration. Whether the prohibition of cochlear ossification can be best achieved by retrosigmoid approaches, compared to other approaches to the CPA, or not could be a subject of future studies. [ABSTRACT FROM AUTHOR]

Objective: This study aims to investigate patterns of cochlear ossification (CO) in cadaveric temporal bones of patients who underwent vestibular schwannoma (VS) surgery via the translabyrinthine (TL), middle cranial fossa (MF), or retrosigmoid (RS) approaches., Study Design: Histopathologic analysis of cadaveric temporal bones., Setting: Multi-institutional national temporal bone repository., Methods: The National Institute of Deafness and Communication Disorders and House Temporal Bone Laboratory at the University of California, Los Angeles and the Massachusetts Eye and Ear Otopathology Laboratory were searched for cadaveric temporal bones with a history of VS for which microsurgery was performed. Exclusion criteria included non-VS and perioperative death within 30 days of surgery. Temporal bones were analyzed histologically for CO of the basal, middle, and apical turns., Results: Of 92 temporal bones with a history of schwannoma from both databases, 12 of these cases met the inclusion criteria. The approaches for tumor excision included 2 MF, 4 RS, and 6 TL approaches. CO was observed in all temporal bones that had undergone TL surgery. Among temporal bones that had undergone MF or RS surgeries, 5/6 had no CO, and 1/6 had partial ossification. This single case was noted to have intraoperative vestibular violation after RS surgery upon histopathologic and chart review., Conclusion: In this temporal bone series, all temporal bones that had undergone TL demonstrated varying degrees of CO on histological analysis. MF and RS cases did not exhibit CO except in the case of vestibular violation. When cochlear implantation is planned or possible after VS surgery, surgeons may consider using a surgical approach that does not violate the labyrinth., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Hypothesis: Ossification of the cochlea was once considered to be a contraindication for cochlear implantation. Advances in cochlear implant technology and coding strategies have led to developments in different surgical procedures to manage cochlear ossification. The endoscopic technique allows a direct approach to the round window and the cochlea, especially in remodeled labyrinth, allowing a better vision of scala tympani. Background: Tertiary referral ENT center. Methods: Between January 2011 and February 2015 three patients with far advanced otosclerosis with partial obliteration of the cochlea were selected and underwent endoscopic-assisted cochlear implantation. Results: In far advanced otosclerosis, endoscopy allowed a magnification of the anatomy of the round window, permitting the surrounding anatomical structures forming the anatomy of the niche to be identified, and avoiding a blind dissection. No postoperative complications were noted, in particular, no surgical site infection, no vertigo, and no facial nerve injuries. Implant activation was routinely performed 1 month after surgery. All monitoring till date has indicated that the external auditory ducts are well ventilated and there are no signs of extrusion. Conclusions: Ossification may occur as a consequence of the pathology of meningitis, chronic otitis media, severe otosclerosis, autoimmune inner ear diseases, temporal bone traumas, and other diseases. Advances in cochlear implant technology and coding strategies have led to developments in different surgical procedures to manage cochlear ossification. Supported by a number of years of experience in the field of otoendoscopic surgery, we propose a technique for cochlear implantation under unfavorable conditions using endoscopic-assisted surgery, especially in advanced otosclerosis. This technique permits us to extend the indication for cochlear implantation, and in our opinion will reduce the morbidity associated with this surgical procedure. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: The aim of this study was to evaluate outcomes of postlingual postmeningitic patients who received an auditory brainstem implant (ABI).Study Design: Retrospective analysis was performed on postlingual postmeningitic patients with bilateral profound sensorineural hearing loss who underwent ABI between the years 2007 and 2014 METHODS: All patients were postlingually deaf due to cochlear ossification as a consequence of bacterial meningitis. The patients received a MED-EL or Neurelec ABI. All patients were operated on at different hospitals by the same primary surgeon. The patients were tested using Ling 5 sound detection, sound field implant thresholds between 250 Hz and 6 kHz, and 6 to 12 choice closed-set word and sentence tests.Results: Nine patients with postmeningitic cochlear ossification received an ABI. Five of nine ABI users (55.5%) wear their audio processors (AP) most of the time. Four (44.5%) with no perceivable benefit have become nonusers. Three of the five consistent ABI users reported good benefit. The other two ABI users who do wear their APs do not respond to sound in daily living but reported benefits such as "feeling sound" in a good way.Conclusions: In this study, five of nine patients (55.5%) with bilateral ossified cochlea had some degree of benefit from their ABI. An ABI may be useful in hearing restoration in postlingual patients with bilateral ossified cochlea due to meningitis. However, poor results may be related to side effects, which may necessitate deactivation of electrodes, long duration of auditory deprivation, or impairments in the auditory neural structures as a result of meningitis.Level Of Evidence: 4. Laryngoscope, 126:1889-1892, 2016. [ABSTRACT FROM AUTHOR]

Objectives To determine impedance values and charge consumption following cochlear implantation post-meningitic deaf children depending on the grade of cochlear ossification and obliteration. Methods Post-meningitic deaf (n = 49) and control (n = 43) children treated with cochlear implants were included in the study. Impedance and charge values were calculated for each group. The degree of ossification of the cochlea was evaluated from a high-resolution computed tomography (HRCT) scan whereas the degree of obliteration was determined intraoperatively by the surgeon. Results Pneumococci were the principal pathogen responsible for bacterial meningitis, followed by meningococci. In HRCT scans, the degree of ossification was 1 and 2 in 29% of patients. The results of the intraoperative assessment of the cochlea showed obliteration grade 1 in 38% and grade 2 in 23% of cases. Children in the meningitis group showed significant higher impedances comparing to the control group. A significantly increased charge consumption was observed in patients with a grade 2 ossification when compared to those without ossification (P = 0.02). Discussion Cochlea implanted children with meningitis-related deafness exhibit higher impedances, especially in the region of the basal and middle turn, however, not depending on the degree of cochlear ossification. High impedances and charge in the meningitis group may be explained by alterations in the central auditory pathway or on the electrode surface. Conclusion To optimize the outcome in post-meningitic deaf children, surgery is advisable at an early stage prior to the onset of cochlear ossification. [ABSTRACT FROM AUTHOR]

This study was designed to investigate the indication and advantages of contralateral implantation after postimplant meningitis (piM). Speech perception assessment, most comfortable levels and high-resolution computer tomography were used to monitor cochlear fibrosis/ossification and clinical changes in outcomes in 5 children affected by meningitis after a variable-time post-cochlear implantation. Ipsilateral ossification was found in 3 children, 1 of whom developed delayed contralateral ossification. These children were implanted on the contralateral side as they all showed deterioration of hearing performance. Results from the present paper and literature analysis suggest that, (1) piM can induce ipsilateral and contralateral ossification, (2) meningitis-induced cochlear ossification is more prone to develop in the presence of a normal cochlear structure and (3) contralateral implantation after piM has proven to be effective in restoring performance when a full electrode insertion is accomplished. Copyright © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

To study the outcomes of split electrode array cochlear implantation in ossified cochlea using the CAP scoring system. Retrospective case study. Tertiary referral center. Six cochleae in three adult and three pediatric patients with ossification. Intervention(s): All Patients underwent cochlear implantation with a split electrode array system. Major outcome parameter(s): Number of electrodes inserted during surgery, number of functioning electrodes on follow-up and auditory performance evaluation using the CAP score-Category of Auditory Perception [TSC Revised Version, based on Nottingham CI Program, 1995]. Six patients (three children and three adults) had insertion of split electrode array system. The mean number of electrodes inserted were 18.3 (range 15-21) and functioning electrodes at follow-up were mean of 14.3 (range 7-21). Auditory performance was measured using CAP score at 1 year post implant follow up, mean score in children was six and that in adult was eight. One pt had facial nerve twitching which was corrected by switching off the concerned electrode. No complications in sort of facial palsy or vestibular disorder were observed. Patients of ossified cochlea having profound deafness do well with split electrode array cochlear implantation as evaluated with CAP scoring system. The split electrode array results in more number of electrodes within the cochlear lumen. Retro graded apical array insertion has less chances of facial nerve stimulation as it is placed away from the nerve. [ABSTRACT FROM AUTHOR]

We report on a rare case of cochlear implantation in a patient affected by relapsing polychondritis (RP), which over time induced cochlear fibrosis/ossification and deterioration of previously excellent hearing performance. The clinical course was determined by CT scan, electrophysiology, and speech perception data. We conclude that RP is a severe autoimmune connective disorder that can cause profound sensorineural hearing loss. Cochlear implantation in these patients can provide excellent performance. Continuation of therapy may improve prognosis, but relapses involving inner ear structures can determine fibrosis/ossification of the modiolus and interfere with cochlear implant use. [ABSTRACT FROM AUTHOR]

Conclusions: Despite the need for special fitting strategies, improvements in speech discrimination tests support the use of cochlear implantation (CI) for patients with Cogan syndrome. Adequate preimplant counselling is mandatory, to prevent high expectations and to stress the necessity for bilateral implantation. Objective: In 60% of patients with Cogan syndrome, CI remains the only treatment option. Literature data agree that once the electrode array is properly inserted, functional outcomes are very good. Nevertheless, results may deteriorate due to progressive cochlear ossification. A few studies have documented the outcomes of CI in these patients, but none have reported the long-term results. Methods: This was a retrospective study describing the outcomes of 3 implanted patients with Cogan syndrome -- among 300 adult patients who received a cochlear implant, 3 had become deaf due to Cogan syndrome. Results: In one patient the cochlear ossification advanced and the speech perception abilities worsened from the highest category to identification of words in closed set. The second patient complained of an abrupt reduction of loudness at 18 months post-implant, which required an increased electrical stimulation. The third patient reached the identification category probably due to auditory dyssynchrony, as an atypical consequence of the syndrome. [ABSTRACT FROM AUTHOR]

Cochlear microphonics are electrical stimulus responses of the inner ear. They are generated by mechanical displacement of the hair cells caused by acoustic stimulation and can be recorded from the cochlear promontory via a needle electrode. In individuals with post-meningitic deafness, fibrous obliteration or ossification of the cochlea may occur. The aim of the present investigation was to establish whether obliteration or ossification leads to any alteration in the extent to which cochlear microphonics can be recorded (as compared with that in patients with a fluid-filled cochlea whose deafness was not post-meningitic), as an indication of changes in the mechanical displacement of the remaining hair cells. Cochlear microphonics in 15 individuals with proven obliteration or ossification of the cochlea were compared with those in 15 control subjects (deaf individuals whose hearing loss was not post-meningitic, and who had a fluid-filled cochlea). Our findings reveal no statistically significant differences between the two groups in the degree to which cochlear microphonics can be recorded. This study does not demonstrate evidence of a change in this property due to ossification or obliteration of the cochlea. [ABSTRACT FROM AUTHOR]

The aim of this multicentre study was to compare T1 with T2 weighted MRI scans of the labyrinth after meningitis and to investigate whether waiting with scanning improved the reliability of diagnosing an ongoing process such as cochlear osteogenesis. Forty-five patients were included who suffered from meningitis induced hearing loss (radiological imaging <1 year after meningitis). Twenty-one gadolinium enhanced T1 and 45 T2 weighted MRI scans were scored by two radiologists regarding the condition of the labyrinth. These radiological observations were compared with the condition of the cochlea as described during cochlear implantation. A higher percentage of agreement with surgery was found for T2 (both radiologists 73%) than for T1 weighted MRI scans (radiologist 1: 62%, radiologist 2: 67%), but this difference is not significant. There was no significant difference between early (0–3 months) and late (>3 months) scanning, showing that radiological imaging soon after meningitis allows early diagnosis without suffering from a lower agreement with surgical findings. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: The present study is a long-term follow-up of speech perception outcomes and cochlear implant use in three cases of meningitis that occurred after cochlear implantation. Study Design: Case series study. Methods: Study was performed on three children implanted with different models of Clarion® devices, two of them with positioner. Recognition and comprehension were assessed via the Italian adaptation of GASP (TAP) test, and phonetically balanced bi-syllabic words in open-set. High resolution computed tomography scan acquisition was performed to obtain axial coronal and oblique multiplanar reconstructions of the cochlea. Results: Two patients were affected by enlarged cochlear acqueduct and Mondini malformation the first carrying positioner. One patient had a normal cochlea, and the positioner could have been the main cause of bacterial spread. As a consequence of meningitis the child with normal cochlea and the other with enlarged vestibular acqueduct developed cochlear ossification, increased M-level and worsening of hearing outcomes. The child with Mondini malformation developed facial nerve stimulation. Contralateral implantation was performed in the first two patients. Conclusion: Bacterial meningitis occurring after cochlear implantation may induce cochlear ossification, facial nerve stimulation, and permanent or temporary loss of implant use. Planned follow-up with high resolution computed tomography and evaluation of M-levels could be useful prognostic tools in the management of these patients. [ABSTRACT FROM AUTHOR]

Hearing rehabilitation by cochlear implantation is not always possible in case of total ossification after pneumococcal meningitis. We report 3 cases of postmeningitis profound hearing loss with total cochlear ossification in adults who underwent auditory brainstem implantation (Nucleus 22®, Cochlear Inc., Lane Cove, Australia) between 1999 and 2004. The postoperative follow-up period ranged from 1 to 6 years. Eleven to 15 out of 22 electrodes were activated. All patients had significant speech discrimination in the sound-only mode and an enhanced lip-reading performance with the implant. Auditory brainstem implants are an efficient means of auditory rehabilitation and may be considered in selected cases of bilateral profound hearing loss with the impossibility of cochlear implantation. Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Summary: A 3-year, 9-month-old boy who contracted bilateral total deafness following bacterial meningitis underwent cochlear implant of the right ear 6 months after the onset of deafness. He became a successful cochlear implant user, while hearing in the non-implanted left ear began to improve 15 months after surgery. His hearing fluctuated in the early phase of recovery, but finally became stable at 60dB pure tone average 18 months later. At the age of 7 years, he started to use a conventional hearing aid in the left ear and a cochlear implant in the right ear. Now at 11 years old, he takes advantages of the bimodal hearing in speech recognition and sound localization. The present case suggests that close consultation with the parents is essential in deciding on the use of a cochlear implant, in this case explaining the possibility of cochlear ossification and language development delay on one side and the rare incidence of spontaneous recovery on the other side, since we have no means of predicting the outcome of postmeningitic deafness. [Copyright &y& Elsevier]

Conclusions . Subjects with cochlear ossification derived benefits in terms of speech performance similar to those of the non-ossified group. It is thus recommended that the insertion of short electrode arrays should be considered an alternative choice for patients with cochlear ossification. Objective . Cochlear ossification has been recognized as a major obstacle to the full insertion of a multichannel cochlear implant electrode array. To alleviate the technical difficulty of placing a standard electrode array and the possibility of causing undesirable trauma to the cochlea, a newly designed electrode array with the same number of electrodes compressed into a shorter length has been made available. The aim of the present study was to examine the speech perception performance of patients implanted with the MED-EL C40?+?S compressed electrode array and to compare their results with those of matched groups implanted with the MED-EL C40?+?standard electrode array. Material and methods . One pre-lingually and two post-lingually deaf subjects using short electrode arrays were matched with three groups of subjects using standard electrode arrays. The pre- and postoperative speech perception scores were evaluated. Results . All three subjects using compressed electrode arrays achieved speech perception scores comparable to those of matched subjects using standard electrode arrays. There was also a tendency for the subjects to show similar patterns of speech perception scores as a function of the difficulty of the tests. [ABSTRACT FROM AUTHOR]

Establish if total ossification of the cochlea due to meningitis can represent an impediment for the development of speech recognition abilities in prelingual children comparing with other children that suffered the illness, but having normal cochleae. To establish the differences of information processing in these children. To establish the necessity of later specific treatments, a sample was taken from 28 children.The development of speech recognition abilities was evaluated at 36 months of C.I. use. The neurolinguistic levels evaluated were semantic lexical, grammar, phonologic, memory, perceptive functions and lecto-writing.The children with ossified cochleae displayed neurolinguistic handicaps that included difficulty in the resolution of visual operations, inadequate inhibition, labile attention, disruptive conduct, difficulties in understanding instructions, upheavals of memory, echolalia and difficulty for the lecto-writing. The evolution was slower, not acceding to Open Set. They attend special schooling, with support of specific therapy of language together with the auditory one.The presence of total ossification and partial insertion was always accompanied by different degrees of difficulty in the processing of speech sounds, requiring special schooling and specific treatment of language. The development of abilities of speech perception was slower [Copyright &y& Elsevier]

Objective --To investigate the auditory rehabilitative results achieved in five patients with cochlear implants (CIs) who subsequently received, due to poor results, auditory brainstem implants (ABIs). Material and Methods --Between April 1997 and March 2003, 37 patients (age range 14 months to 70 years) were fitted with ABIs in our ENT Department. Fourteen subjects had neurofibromatosis type 2 and 23 were non-tumor patients who had cochlea or cochlear nerve disease. Five subjects had previously been treated with a CI and received an ABI owing to the poor results achieved. One child had bilateral undiagnosed cochlear nerve aplasia and one was suffering from auditory neuropathy; three adults had total cochlear ossification. Results --The open-set sentence recognition score (auditory-only mode) 6-8 months after ABI activation ranged from 0% to 100% in adults. In 1 subject the speech-tracking score was 56 words/min with the ABI. The two children who had achieved no hearing ability with their CI were able to detect sounds and words as early as 3 months after activation of the ABI. Conclusion --CI failure as a result of anatomical abnormalities can be remedied by an ABI. [ABSTRACT FROM AUTHOR]