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Cochlear implantation in a patient with mitochondrial encephalopathy, lactic acidosis and stroke-like episodes syndrome.

OBJECTIVE: The aim of this study was to investigate the usefulness of a Nucleus C124M cochlear implant in a patient with mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome. MATERIAL AND METHODS: A 29-year-old woman with MELAS syndrome presented with sensorineural hearing loss and diabetes mellitus and underwent multichannel cochlear implantation. The follow-up period was 10 months. The case history, assessment of mitochondrial disease, indications for the cochlear implant and the benefits of cochlear implantation were evaluated. RESULTS: Nine months after…

Scala vestibuli insertion in cochlear implantation: a valuable alternative for cases with obstructed scala tympani.

Insertion of a sufficient number of electrodes is important for a successful use of cochlear implants. We investigated the results of scala vestibuli insertion for cochlear implantation in cases of obstructed scala tympani. In a series of 200 cochlear implantations, scala vestibuli insertion was successfully performed in 4 cases with obstruction of the scala tympani. Etiologies included a temporal bone fracture, severe otosclerosis and malformations of the cochlea. The maximum insertion depth obtained via the scala vestibuli was 30 mm. Postoperative results were comparable to patients in whom …

Cochlear implantation in a district general hospital: problems and complications in the first five years.

Fifty-three patients, 34 adults and 19 children have been implanted over the first five years of the cochlear implant programmed at the North Riding Infirmary (NRI). For a small centre, based at a district general hospital, our complication rate compare favourably with others: 11.7 per cent adult and 10.5 per cent paediatric major complications. Data on all patients was gathered prospectively as part of the national cochlear implant programme, and we report and discuss all complications from this centre.

Схемы нейронного дегенерации в кохлее человека и слуховом нерве: последствия для имплантации кохлеара.

Хотя личность всех переменных, которые могут повлиять на распознавание речи после кохлеарной имплантации, неизвестна, степень сохранения спиральных клеток ганглиона, как правило, считается первостепенной. Обобщается ряд экспериментов в наших лабораториях, направленных на количественную оценку выживших клеток спиральных ганглионов у глубоко глухих, оценку прогнозируемой ценности различных клинических параметров и оценку последствий имплантации во внутреннем ухе. Гистологическое исследование внутренних ушей пациентов, которые были оглушены в течение жизни, показало, что причина глухоты составляе

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Although the identity of all the variables that may influence speech recognition after cochlear implantation is unknown, the degree of preservation of spiral ganglion cells is generally considered to be of primary importance. A series of experiments in our laboratories, directed at quantification of surviving spiral ganglion cells in the profoundly deaf, eva…

Importance of auditory-verbal education and parents' participation after cochlear implantation of very young children.

Congenitally deaf children who receive a cochlear implant at a young age have the potential to achieve nearly normal language development. However, parents and rehabilitation therapists must realize that intensive and long-duration auditory-verbal education is required postoperatively to achieve this goal. Furthermore, the success of the therapeutic treatment is best realized if the parents themselves are trained to engage in linguistic interactions with the child within the natural context of the family. This is especially true if the parents are able to creatively guide auditory-verbal train

Failed cochlear implant followed by auditory brainstem implant in a patient with Mondini malformation and uncontrolled otomastoiditis: a case report.

BACKGROUND: Cochlear implant (CI) implantation is the standard intervention for children with severe-to-profound hearing loss, including those with inner ear malformations, such as Mondini malformation. Post-CI implantation infections, although uncommon, can lead to serious complications, including device failure and labyrinthine ossification (LO). CASE PRESENTATION: Here, we report the case of a 17-year-old Thai girl with bilateral Mondini malformation who underwent right-sided CI at 15 months of age. She developed recurrent otitis media that progressed to chronic otomastoiditis that was unr

Phoneme discrimination and localization performance in children with cochlear implants and contralateral auditory brainstem implants with inner ear malformations.

PURPOSE: This study compares unilateral and bilateral phoneme discrimination (PD) and azimuth localization abilities in children with severe inner ear malformations and cochlear nerve deficiencies who use a cochlear implant (CI) and a contralateral auditory brainstem implant (ABI). METHODS: A total of 17 children between the ages of 7 and 18 years with CI and contralateral ABI were included in the study. PD and localization tests were evaluated in three conditions: CI only, ABI only, and bilateral. All subjects completed a self-report Hearing-Related Quality of Life (HRQoL) scale. RESULTS: S

Functional and Speech Outcome of Unilaterally Cochlear Implanted Children after 1 year of Implantation in NIENT: A Retrospective Study.

Hearing loss can be congenital or acquired. Most of the time congenital hearing loss means profound sensorineural hearing loss. The invention of cochlear implantation has already an established role in improving the hearing capability in congenital sensorineural hearing loss. To speak properly after cochlear implant, one needs to go for speech therapy or audio-verbal therapy. Speech therapy has become an integral part of the cochlear implantation program. In Bangladesh cochlear implantation (CI) programs started around 15 years ago, which was limited to Bangabandhu Sheikh Mujib Medical Univers…

Comparing Speech Recognition Outcomes Between Cochlear Implants and Auditory Brainstem Implants in Patients With NF2-Related Schwannomatosis.

OBJECTIVE: To compare cochlear implant (CI) and auditory brainstem implant (ABI) performance in patients with NF2-related schwannomatosis (NF2). STUDY DESIGN: Historical cohort. SETTING: Tertiary academic center. PATIENTS: A total of 58 devices among 48 patients were studied, including 27 ABIs implanted from 1997 to 2022 and 31 CIs implanted from 2003 to 2022. Three patients had bilateral ABIs, three had bilateral CIs, three had an ABI on one side and a CI on the other, one had a CI that was later replaced with an ipsilateral ABI, and one had an ABI and CI concurrently on the same side. IN

The role of cochlear implant positioning on MR imaging quality: a preclinical in vivo study with a novel implant magnet system.

PURPOSES: To investigate the effects for Ultra 3D cochlear implant (CI) positioning on MR imaging quality, looking at a comprehensive description of intracranial structures in cases of unilateral and bilateral CI placement. METHODS: Four CI angular positions (90°, 120°, 135° and 160°) at 9 cm distance from the outer-ear canal were explored. The 1.5 T MRI assessment included our institutional protocol for the investigation of brain pathologies without gadolinium application. Three investigators (two experienced neuroradiologists and one experienced otoneurosurgeon) independently evaluated the

Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.

OBJECTIVE: To determine audiological outcomes of children who use a cochlear implant (CI) in one ear and an auditory brainstem implant (ABI) in the contralateral ear. DESIGN: Retrospective case review. SETTING: Tertiary referral hospital. PARTICIPANTS: Twelve children followed with CI and contralateral auditory brainstem implant (ABI) by Hacettepe University Department of Otorhinolaryngology and Audiology in Turkey. All children were diagnosed with different inner ear malformations with cochlear nerve aplasia/hypoplasia. CI was planned in the ear with better sound detection during behaviour

Baseline Function Predicts Gains in Both Speech Recognition and Quality of Life After Cochlear Implantation.

OBJECTIVE: To characterize patterns of improvement in speech recognition and quality of life (QOL) after cochlear implantation and examine how baseline performance, device use, and candidacy status ("borderline," qualifying in noise only vs. "traditional," qualifying in quiet) predict postoperative gains in both domains. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: One hundred one adult unilateral CI recipients implanted between 2019 and 2023 with complete preoperative and postoperative (6 or 12 mo) speech recognition and QOL data. INTERVENTIONS: U…

Language development after pediatric cochlear implantation: Why outcomes vary and how to optimize clinical management.

Pediatric cochlear implantation is the standard of care for infants and young children with congenital bilateral severe-to-profound sensorineural hearing loss who receive limited benefits from optimally fitted hearing aids. Despite significant advances in cochlear implant (CI) device technology and surgical safety, post-implant spoken language outcomes remain highly heterogeneous, ranging from robust speech recognition and age-appropriate language to minimal benefits in sound detection with limited speech perception and language delays. This narrative review integrates findings from the litera…

Cochlear Implantation in Arnold-Chiari Malformation Type 1: Expanding the Boundaries of Pediatric Neuro-Otology.

Arnold-Chiari malformation (ACM) is a rare congenital hindbrain anomaly, with types 1-4 depending of degree of herniation of posterior fossa … nsorineural hearing loss in both ears. After multidisciplinary clearance, she underwent right-sided cochlear implantation. Intraoperative challenges faced were dilated emissary veins, an anteriorly placed sigmoid sinus, and a low-lying dura. However, a full electrode insertion was achieved and confirmed on neural response telemetry. The postoperative course was unevent …

From thunder to silence: Cochlear implantation for bilateral profound sensorineural hearing loss after lightning strike.

INTRODUCTION: Lightning strikes can cause diverse neuro-otologic injuries. Tympanic membrane rupture is common, whereas bilateral profound sensorineural hearing loss with vestibular dysfunction and perilymphatic fistula is rare. Cochlear implantation for lightning-induced deafness has been reported only once. A farm worker struck by lightning sustained second-degree burns and intracranial hemorrhages requiring emergency neurosurgical management and cervical wound closure. During intensive care, clear left-sided otorrhea prompted ENT evaluation, revealing bilateral tympanic membrane perforation…

Cochlear Implantation in Children Without a Hearing Aid Trial: A Case Series of 22 Patients.

OBJECTIVES: Timely access to hearing technology is paramount to ensure early auditory access to the sounds needed for listening and learning in children with early hearing loss (HL). Although children typically move to cochlear implants (CIs) after first having tried hearing aids (HAs), there are some cases where the HA trial may create unnecessary additional delays. This clinical focus article presents three categories of patients where implantation appears warranted without an HA trial. METHOD: This study examines 3 years of retrospective chart data for pediatric CI patients at a tertiary c

Cochlear implantation with a dexamethasone-eluting electrode array: First-in-human safety and performance results.

Cochlear implantation is the standard of care for individuals with severe-to-profound sensorineural hearing loss. However, implantation itself can degrade residual hearing, for example due to insertional trauma and subsequent inflammatory processes. One potential method to mitigate this loss of residual hearing is through the local and sustained delivery of anti-inflammatory drugs released from the electrode array. To this end, a dexamethasone eluting electrode array (FLEX28 DEX) was developed by MED-EL. Here we present the results from a first-in-human feasibility study of the CIDEXEL system …

[Application of artificial neural network in assessing speech intelligibility in adult patients after cochlear implantation].

OBJECTIVE: An objective of the study was to evaluate the feasibility of using Voice Extraction (VE) technology in adult patients after cochlear implantation and to conduct a comparative analysis of speech intelligibility of a noise-free signal and a signal using VE technology in adult patients after cochlear implantation. MATERIAL AND METHODS: Sixty-seven patients took part in the study. All patients were diagnosed with chronic bilateral severe to profound sensorineural hearing loss. All patients underwent unilateral cochlear implantation. The observation period for patients ranged from 6 mon…

Personalized Anesthesia Strategies for Cochlear Implantation: Insights on Local Anesthesia From a Single-institution Experience.

OBJECTIVE: To assess the feasibility and safety of cochlear implantation (CI) under local anesthesia (LA) with minimal conscious sedation, and to present institution-specific criteria for anesthesia selection in high-risk adult patients. PATIENTS: Between January 2021 and February 2025, a total of 980 ears underwent CI at a single tertiary referral hospital. Of these, 17 ears from 16 adult patients (mean age, 65 y; range, 35 to 87) were selected for CI under LA based on predefined clinical indications. INTERVENTION: CI procedures were performed under LA with minimal sedation following a stan…