Cochlear Implants in Young Children with Usher'S Syndrome

1995 ◽  
Vol 104 (9_suppl2) ◽  
pp. 342-345 ◽  
Author(s):  
N. M. Young ◽  
J. C. Johnson ◽  
M. B. Mets ◽  
T. C. Hain
Keyword(s):  
Hearing Loss ◽  
Early Diagnosis ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Autosomal Recessive Disorder ◽  
Sensorineural Hearing ◽  
Special Importance ◽  
Sensory Deficits ◽  
Usher’S Syndrome

Usher's syndrome (US) is an autosomal recessive disorder characterized by congenital sensorineural hearing loss and retinitis pigmentosa. The majority of affected individuals have severe to profound sensorineural hearing loss and are therefore potential cochlear implant candidates. A goal of our otology program has been early identification and implantation of children with US. At our center, early diagnosis of US has been achieved by comprehensive ophthalmologic evaluation including electroretinography. Four of our 19 pediatric cochlear implant recipients with congenital sensorineural hearing loss were identified with US and implanted prior to the onset of signs or symptoms of visual loss. All children have received measurable benefit from cochlear implantation. In light of the dual sensory deficits present in US, the need for early diagnosis and cochlear implantation is of special importance in this population.

Subdural haematoma: a complication of cochlear implantation

2004 ◽  
Vol 118 (12) ◽  
pp. 980-982 ◽  
Author(s):  
V.S. Sunkaraneni ◽  
A. Banerjee ◽  
R.F. Gray
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implants ◽  
Cochlear Implantation ◽  
Subdural Haematoma ◽  
Sensorineural Hearing ◽  
Deep Well ◽  
Major Complications ◽  
Emissary Veins

Cochlear implants have transformed the treatment of sensorineural hearing loss. They have few major complications. The authors describe the case of a man fitted with a cochlear implant who suffered a postoperative subdural haematoma. The haematoma is thought to have been caused by bleeding from emissary veins opened by the drill passages used to anchor the sutures for the receiver/stimulator. The authors have abandoned tie down sutures in cochlear implants, preferring an appropriately deep well with squared-off rims, which would secure the implant in place. They have had no further complications of this nature.

scholarly journals Translation in Ukrainian and adaptation of the English questionnaires: scales of meaningful auditory integration (MAIS) and scales of meaningful language use (MUSS) scales for assessing language development in children with deafness after cochlear implantation

2021 ◽  
pp. 22-36
Author(s):  
Victor Pysanko ◽  
Tetiana Kholodenko ◽  
Iryna Sapizhak ◽  
Larisa Kud
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
English Language ◽  
Sensorineural Deafness ◽  
Meaningful Use ◽  
Sensorineural Hearing ◽  
The World ◽  
Auditory Integration

Introduction: The world community is concerned with the assessment of the speech and hearing development of children with sensorineural deafness after cochlear implantation, since the most important result of rehabilitation is the success of social integration of such children. In most countries of the world, the Meaningful Auditory Integration Scale (MAIS) and the Meaningful Use of Language Scale (MUSS) are used to assess the auditory and language integration of children with sensorineural hearing loss after cochlear implantation (CI). Unfortunately, in Ukraine there are still no Ukrainian questionnaires for assessing the auditory development of children with sensorineural hearing loss after cochlear implantation. The aim of our study was to translate MAIS and MUSS manuals into Ukrainian, to adapt and validate them culturally. Materials and methods: Materials for translation and cultural adaptation were English-language versions of the Meaningful Auditory Integration Scale (MAIS) and Meaningful Use of Language Scale (MUSS), developed by the Nottingham Group of Cochlear Implantation, which are recognized and recommended for use by all cochlear implant manufacturers. Results obtained: The evaluation consisted of the following stages: preparatory stage – collection and analysis of literature; translation of original texts from English into Ukrainian; reversed translation by an independent translator of Ukrainian text into English; collation of the translation and comparison with the original questionnaire by an independent linguist; Cognitive analysis; field-testing of textbooks translated into Ukrainian with a small group of children after the CI; corrections, final revision of translations to identify and correct any grammatical, orthographic and other errors. The Ukrainian version of the questionnaire demonstrated a high level of internal compliance, testability and validity. Conclusions: Given the considerable relevance and demand ability of the materials, the tests can be recognized as valid and recommended for use in cochlear implant rehabilitation process.

scholarly journals Cochlear implantation in a patient with mucopolysaccharidosis IVA

2019 ◽  
Vol 7 ◽  
pp. 2050313X1987379 ◽  
Author(s):  
Kyoko Nagao ◽  
Cassidy Walter ◽  
William J Parkes ◽  
Michael Teixido ◽  
Mary C Theroux ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Multidisciplinary Team ◽  
Cochlear Implantation ◽  
Sensorineural Hearing ◽  
Mucopolysaccharidosis Iva ◽  
Morquio A ◽  
Auditory Performance

Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation. Although severe progressive sensorineural hearing loss is a common feature of mucopolysaccharidosis IVA, no detailed description of cochlear implantation for mucopolysaccharidosis IVA has been reported. To review the effectiveness and special considerations associated with cochlear implantation in patients with mucopolysaccharidosis IVA, we here report the case of cochlear implantation in mucopolysaccharidosis IVA by a multidisciplinary team. A retrospective chart review was conducted on a 34-year-old female with mucopolysaccharidosis IVA, who received a cochlear implant. Audiometric thresholds, speech perception scores, and cochlear implant processor mapping information were reviewed during the first 12 months following cochlear implantation. The results of audiological tests indicate improved hearing thresholds as well as remarkable enhancement of speech perception skills over 12 months of cochlear implant use. Cochlear implantation improved auditory performance in a mucopolysaccharidosis IVA patient with postlingually severe-to-profound sensorineural hearing loss. The benefits of cochlear implantation could be meaningful for other Morquio patients with progressive hearing loss, although the risks of surgery and anesthesia should be carefully considered by a multidisciplinary team of experts during the cochlear implant candidacy process.

Petrosectomy in cochlear implant surgery

2020 ◽  
Vol 9 (2) ◽  
pp. 1-6
Author(s):  
Katarzyna Amernik ◽  
Kazimierz Niemczyk ◽  
Renata Twardowska ◽  
Ewa Jaworowska
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Middle Ear ◽  
Cochlear Implantation ◽  
Sensorineural Hearing ◽  
Speech Understanding ◽  
Canal Wall ◽  
Canal Wall Down ◽  
Canal Wall Down Mastoidectomy

Introduction: Surgical treatment of deep heating loss with cochlear implant is well know and successful method which can be used both in children and adults. In patients with cholesteatoma or who underwent surgery canal wall down technique special surgical approach schooled be used – lateral/subtotal petrosectomy. Material and method: Patients witch deep bilateral sensorineural hearing loss and cholesteatoma of middle ear or after treatment of cholesteatoma with canal wall down mastoidectomy were included in the study. Retrospective analysis of patient’s data, radiological and audiological testing was performed. Results: In Department of otolaryngology for Children and Adults and Oncologic Laryngology Pomeranian University of Medicine between 2008 and 2018 90 surgeries of cochlear implantations were performed and among which 3 petrosectomies in one child (5years old) and two adults aged 62 and 73 years old who underwent canal wall down mastoidectomy in the past. In all patients single stage procedure was used, petrosectomy with cochlear implantation. Wound healing an postoperative period was uneventful in all cases. Follow up ranges from 26 to 32 months, in control computer tomography there is lack of indirect signs of recurrence of cholesteatoma. In a child, who accordingly is autistic and mentally retardated, good hearing result is observed and speech understanding, without spontaneous speech. In adult patients in free field audiometry hearing level is 35 and 40dB with speech understanding 80%. Discussion: Patients with chronic ear inflammation can be successfully and safely treated with cochlear implantation using petrosectomy technique. Subtotal/lateral petrosectomy is a method of choice when deep sensorineural hearing loss coexists with chronic inflammatory changes of the middle ear.

scholarly journals Cochlear Implantation Following Explorative Tympanotomy in Patients With Sudden Sensorineural Hearing Loss: Surgical Features and Audiological Outcomes

2021 ◽  
pp. 014556132110091
Author(s):  
Robin Rupp ◽  
Joachim Hornung ◽  
Matthias Balk ◽  
Matti Sievert ◽  
Sarina Müller ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Connective Tissue ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Round Window ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Round Window Membrane ◽  
Hearing Results ◽  
Round Window Niche

Objective: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center. Methods: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results. Results: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P = .005) after 2 years. Conclusion: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely.

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

2013 ◽  
Vol 127 (7) ◽  
pp. 708-711 ◽  
Author(s):  
A C Hall ◽  
A C Leong ◽  
D Jiang ◽  
A Fitzgerald-O'Connor
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Skin Lesions ◽  
Sudden Hearing Loss ◽  
Sudden Onset ◽  
Sensorineural Hearing ◽  
Urticarial Vasculitis ◽  
Wells Syndrome ◽  
Bilateral Sensorineural Hearing Loss

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

Bilateral profound sudden sensorineural hearing loss presenting a diagnostic conundrum in a child with sickle cell anaemia

2008 ◽  
Vol 123 (7) ◽  
pp. 811-816 ◽  
Author(s):  
A D Mace ◽  
M S Ferguson ◽  
M Offer ◽  
K Ghufoor ◽  
M J Wareing
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Sickle Cell ◽  
Cochlear Implantation ◽  
World Literature ◽  
Sickle Cell Anaemia ◽  
Early Recognition ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing

AbstractObjective:To present the first published case of a child with bilateral profound sudden sensorineural hearing loss found in association with sickle cell anaemia, and to demonstrate the importance of early recognition, investigation and empirical treatment of sudden sensorineural hearing loss.Method:Case report and review of world literature.Case report:The authors present the case of a seven-year-old child with known sickle cell anaemia, who presented with bilateral profound sensorineural hearing loss developing over a period of five days. There was a history of ophthalmological disease in the preceding weeks, and inflammatory markers were raised. The differential diagnosis included a vaso-occlusive or inflammatory aetiology such as Cogan's syndrome, and treatment for both was instigated. Hearing thresholds did not recover, and the patient underwent cochlear implantation 12 weeks later.Conclusion:Sudden sensorineural hearing loss has a variable aetiology and is rare in children. Immediate treatment for all possible aetiologies is essential, along with targeted investigations and early referral for cochlear implantation if no recovery is demonstrated.

Outcome of Cochlear Implantation in Postmeningitis Profound Sensorineural Hearing Loss at Rajavithi Hospital

2021 ◽  
Vol 104 (2) ◽  
pp. 260-263
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Sensorineural Hearing ◽  
Preoperative Imaging ◽  
Profound Hearing Loss ◽  
Language Status ◽  
Auditory Performance ◽  
Electrode Insertion ◽  
One Year

Background: Bacterial meningitis is one of the major factors in the etiology of acquired sensorineural hearing loss in children and adults. Cochlear implantation in these patients is challenging because of inner ear ossification and fibrosis, and this procedure sometimes achieves poorer outcomes in this scenario than with other causes of sensorineural hearing loss. There has been little research into the factors affecting the outcomes of this procedure. Objective: To evaluate the outcomes of cochlear implantation in patients with postmeningitis profound sensorineural hearing loss and to evaluate the factors that affect the results. Materials and Methods: A retrospective review was conducted of thirty patients who were diagnosed with post meningitis profound hearing loss and underwent cochlear implantation at Rajavithi Hospital between 2001 and 2016. Preoperative language status, duration of deafness, preoperative imaging, and degree of electrode insertion were recorded. Categories of auditory performance-II test (CAP-II) was evaluated in all cases, one year postoperative. Results: Thirty postmeningitis deafness patients underwent cochlear implantation. The median age at diagnosis of meningitis and age at implantation were 41 years (range 1 to 75) and 49.50 years (range 3 to 75), respectively. The median duration of deafness was 12 months (range 4 to 300), and the overall mean CAP-II at one year after surgery was 5.47±2.21. The postlinguistic group had a significantly higher CAP-II score than the prelinguistic one (p=0.006). Electrodes were successfully totally inserted in 19 patients (63.3%) and partially inserted in 11 (36.7%). The average CAP-II score in the group with fully-inserted electrodes was significantly higher than in the group with partially-inserted electrodes (p=0.045). There was no correlation between CAP-II score and age at meningitis diagnosis (p=0.069), age at time of surgery (p=0.105), duration of deafness (p=0.506), or preoperative CT (p=0.228) or MRI abnormality (p=0.078). Conclusion: Cochlear implantation in patients with postmeningitis profound hearing loss had high success rates and favorable outcomes. Preoperative language status and degree of electrode insertion were factors that affected auditory performance results. Keywords: Cochlear implantation, Postmeningitis hearing loss, Sensorineural hearing loss, Meningitis, Rajavithi Hospital

scholarly journals Relationship between Metabolomics Profile of Perilymph in Cochlear-Implanted Patients and Duration of Hearing Loss

Metabolites ◽  
2019 ◽  
Vol 9 (11) ◽  
pp. 262
Author(s):  
Trinh ◽  
Blasco ◽  
Emond ◽  
Andres ◽  
Lefevre ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Round Window ◽  
Univariate Analysis ◽  
Sensorineural Hearing ◽  
Easy Access ◽  
Metabolomic Analysis ◽  
Innovative Strategy ◽  
The Relationship

Perilymph metabolomic analysis is an emerging innovative strategy to improve our knowledge of physiopathology in sensorineural hearing loss. This study aims to develop a metabolomic profile of human perilymph with which to evaluate the relationship between metabolome and the duration of hearing loss. Inclusion criteria were eligibility for cochlear implantation and easy access to the round window during surgery; patients with residual acoustic hearing in the ear to be implanted were excluded. Human perilymph was sampled from 19 subjects during cochlear implantation surgery. The perilymph analysis was performed by Liquid Chromatography−High-Resolution Mass and data were analyzed by supervised multivariate analysis based on Partial Least-Squares Discriminant Analysis and univariate analysis. Samples were grouped according to their median duration of hearing loss. We included the age of patients as a covariate in our models. Statistical analysis and pathways evaluation were performed using Metaboanalyst. Nineteen samples of human perilymph were analyzed, and a total of 106 different metabolites were identified. Metabolomic profiles were significantly different for subjects with ≤ 12 or > 12 years of hearing loss, highlighting the following discriminant compounds: N-acetylneuraminate, glutaric acid, cystine, 2-methylpropanoate, butanoate and xanthine. As expected, the age of patients was also one of the main discriminant parameters. Metabolic signatures were observed for duration of hearing loss. These findings are promising steps towards illuminating the pathophysiological pathways associated with etiologies of sensorineural hearing loss, and hold open the possibilities of further explorations into the mechanisms of sensorineural hearing loss using metabolomic analysis.

scholarly journals Halláscsökkenést okozó etiológiai tényezők cochlearis implantáción átesett gyermekekben

2019 ◽  
Vol 160 (21) ◽  
pp. 822-828
Author(s):  
Nóra Kecskeméti ◽  
Anita Gáborján ◽  
Magdolna Szőnyi ◽  
Marianna Küstel ◽  
Ildikó Baranyi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Speech Development ◽  
Sensorineural Hearing ◽  
Diagnostic Process ◽  
Sufficient Information ◽  
Hearing Rehabilitation ◽  
Junction Protein ◽  
Newborn Hearing

Abstract: Introduction: Congenital sensorineural hearing loss is one of the most common sensory defects affecting 1–3 children per 1000 newborns. There are a lot of causes which result in congenital hearing loss, the most common is the genetic origin, but infection, cochlear malformation or other acquired causes can be reasons as well. Aim: The aim of this study was to establish the etiological factors of congenital profound sensorineural hearing loss in children who underwent cochlear implantation. Results: Our results show that the origin of the hearing loss was discovered in 62.9% of our patients. The most common etiological factor was the c.35delG mutation of the gap junction protein β-2 gene, the allele frequency was 38.7% in our cohort. Infection constituted to 10.1%, and meningitis and cytomegalovirus infection were the second most common cause. 79.9% of our patients received sufficient hearing rehabilitation before the end of the speech development’s period (6 years old), but 11.2% of our cases were still diagnosed late. Conclusions: Based on our data we can state that genetic evaluation is crucial in the diagnostic process of congenital profound sensorineural hearing loss. Sufficient hearing rehabilitation affects the whole life of the child, and by late cochlear implantation the speech development falls behind. We can decrease the ratio of the late implantation with the new protocol of newborn hearing screening, and with sufficient information provided to the colleagues, so the children may be referred to the proper center for rehabilitation without delay. Orv Hetil. 2019; 160(21): 822–828.

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