scholarly journals Cochlear implantation in osteogenesis imperfecta in India: a case report

Author(s):  
Rajesh Kumar ◽  
Ashvanee Kumar Chaudhary ◽  
Sunil Kumar ◽  
Vishwambhar Singh ◽  
Ashutosh A. Abinashi ◽  
...  

<p class="abstract">A 25 years old male patient with osteogenesis imperfecta presented with bilateral profound hearing loss, not benefitted with hearing aid. Cochlear implantation of 12 channel intracochlear implant was done successfully, intracochlear insertion of the 12 electrodes resulted in hearing improvement of 80 dB at frequency 250 Hz to 4 KHz. The patient complained of facial twitching on first visit after switching on speech processer device, reducing the current level at the electrode 6 abolished the non-acoustic nerve excitation with preservation of acoustic performances. Osteogenesis imperfecta patient may involve a risk for non-acoustic nerve activation in cochlear implant patients possibly resulting from a decrease impedance to current spread, it happens because of abnormal bone tissue. Non acoustic nerve activation can be overcome by modification in mapping.</p>

2021 ◽  
pp. 1-7
Author(s):  
Dario Ebode ◽  
Fleur Cohen-Aubart ◽  
Stéphanie Trunet ◽  
Evelyne Ferrary ◽  
Ghizlène Lahlou ◽  
...  

<b><i>Introduction:</i></b> Audiovestibular symptoms are rare in sarcoidosis, but they may also be the first manifestation of the disease. Sudden or progressive bilateral hearing loss is usually associated with vestibular impairment. The mechanism of hearing loss remains unclear, but clinical presentation and magnetic resonance imaging suggest a retrocochlear site for the lesion in most patients. Several cases of hearing recovery after corticosteroid treatment have been reported. In patients with severe or profound hearing loss, the benefit of cochlear implantation is challenging to predict in the case of auditory neuropathy and is rarely described. We present a case series of cochlear implantation in patients with documented neurosarcoidosis. <b><i>Results:</i></b> Seven cases of cochlear implantation in 4 patients with neurosarcoidosis are reported. All of the patients showed a great improvement very quickly in both quiet and noise. Speech performance remained stable over time with a follow-up ranging from 4 to 11 years, even in patients who had disease exacerbation. <b><i>Conclusion:</i></b> Cochlear implantation is possible in deaf patients with neurosarcoidosis. The excellent benefit obtained in our patients suggests a particular type of neuropathy, but endocochlear involvement cannot be entirely ruled out.


2011 ◽  
Vol 32 (2) ◽  
pp. 223-228 ◽  
Author(s):  
Won Sun Yang ◽  
In Seok Moon ◽  
Hee Nam Kim ◽  
Won-Sang Lee ◽  
Sung Eun Lee ◽  
...  

2021 ◽  
Vol 104 (2) ◽  
pp. 260-263

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


2021 ◽  
pp. 019459982110574
Author(s):  
Kaitian Chen ◽  
Bixue Huang ◽  
Jincangjian Sun ◽  
Yue Liang ◽  
Guanxia Xiong

Objective Mutations in the cadherin 23 gene ( CDH23) have been reported to cause cochlear damage, but few studies have investigated the auditory and speech outcome of patients after cochlear implantation. Here, we describe the genetic, auditory, and postoperative outcomes of patients with CDH23 mutations who received cochlear implants. Study Design Retrospective case review. Setting Tertiary referral center. Methods Targeted deafness-related gene panels were sequenced in Chinese families with profound sensorineural hearing loss. The clinical features of subjects carrying potentially pathogenic CDH23 mutations were analyzed. Results Between 2017 and 2019, we identified 5 children with prelinguistically profound hearing loss at our center who harbored 6 variants of CDH23 that segregated with the disease. Of these, 4 variants were novel (c.2591G>T, c.4785G>C, c.5765A>G, and c.9280_9281insTT). All affected individuals had a loss of outer hair cell function, with an average residual hearing level of 3 to 10 dB SPL. Cochlear implantations were arranged for the patients at 11 to 36 months of age. All children made gains in their hearing, language, and speech performances 14 to 120 months after surgery. Their auditory outcomes improved during follow-up intervals. Conclusion This study revealed that children with congenital cochlear defects caused by CDH23 variants can acquire an acceptable auditory and speech outcome after cochlear implantation. Early genetic detection and prenatal counseling for rare deafness genes such as CDH23 remain a priority for the future.


2004 ◽  
Vol 118 (1) ◽  
pp. 3-7 ◽  
Author(s):  
A. Incesulu ◽  
S. Kocaturk ◽  
M. Vural

Patients with chronic otitis media (COM) may have profound sensorineural hearing loss either due to the disease process or secondary to a surgical procedure. Some patients who are candidates for cochlear implantation may have COM coincidentally. The patients in this group need special attention when cochlear implantation is applied. The aim of this study is to evaluate the potential risks and complications in patients with COM.Cochlear implantation was performed in six patients with COM or an infected radical cavity and profound hearing loss. Five of them underwent a two-stage operation, and one had a single-stage operation. Cochlear implantation was performed in all patients without complications. The follow-up period was uneventful.Although such patients have some potential risks, when certain surgical rules are followed very strictly cochlear implantation can be successfully performed in patients with COM.


2011 ◽  
Vol 2011 ◽  
pp. 1-9 ◽  
Author(s):  
B. Y. Roukema ◽  
M. C. Van Loon ◽  
C. Smits ◽  
C. F. Smit ◽  
S. T. Goverts ◽  
...  

Objective. To describe the audiological, anesthesiological, and surgical key points of cochlear implantation after bacterial meningitis in very young infants.Material and Methods. Between 2005 and 2010, 4 patients received 7 cochlear implants before the age of 9 months (range 4–8 months) because of profound hearing loss after pneumococcal meningitis.Results. Full electrode insertions were achieved in all operated ears. The audiological and linguistic outcome varied considerably, with categories of auditory performance (CAP) scores between 3 and 6, and speech intelligibility rating (SIR) scores between 0 and 5. The audiological, anesthesiological, and surgical issues that apply in this patient group are discussed.Conclusion. Cochlear implantation in very young postmeningitic infants is challenging due to their young age, sequelae of meningitis, and the risk of cochlear obliteration. A swift diagnostic workup is essential, specific audiological, anesthesiological, and surgical considerations apply, and the outcome is variable even in successful implantations.


1998 ◽  
Vol 37 (6) ◽  
pp. 382-395 ◽  
Author(s):  
Jan Kiefer ◽  
Christoph von Ilberg ◽  
Bärbel Reimer ◽  
Rainald Knecht ◽  
Volker Gall ◽  
...  

2009 ◽  
Vol 19 (1) ◽  
pp. 32-42 ◽  
Author(s):  
Tamala S. Bradham ◽  
Geneine Snell ◽  
David Haynes

Abstract Technological advances, specifically cochlear implants, have significantly impacted the treatment of children with severe to profound hearing loss. There are, however, very few professional guidelines or resources providing direction for hearing healthcare providers who are serving children with cochlear implants. The following article discusses a comprehensive management protocol for interdisciplinary teams providing cochlear implant services for children.


2021 ◽  
Vol 9 (1) ◽  
pp. 50
Author(s):  
Santosh Kumar Swain

Cochlear implantation is indicated in patients with severe to profound hearing loss that cannot be adequately treated by other auditory rehabilitation measures. The definitive indication of cochlear implantation is made on the basis of an extensive interdisciplinary clinical, audiological, radiological, and psychological diagnostic work-up. There are numerous changes are happening in cochlear implant candidacy. These have been associated with concomitant changes in surgical techniques, which enhanced the utility and safety of cochlear implantation. Currently, cochlear implants are approved for individuals with severe to profound unilateral hearing loss rather than previously needed for bilateral profound hearing loss. Studies have begun using the short electrode arrays for shallow insertion in patients with low-frequency residual hearing loss. The advancement in designs of the cochlear implant along with improvements in surgical techniques reduce the complications and result in the safety and efficacy of the cochlear implant which further encourages the use of these devices. This review article aims to discuss the new concepts in the candidacy of the cochlear implant, cochlear implant in younger children and hearing preservation, a cochlear implant for unilateral deafness, bilateral cochlear implant, and cochlear implant with neural plasticity and selection of patients for the cochlear implant.


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