scholarly journals Two Down Syndrome Patients with Bilateral Profound Hearing Loss: Case Report and Literature Review

2018 ◽  
Vol 1 (2) ◽  
pp. 8
Author(s):  
Yu Zheng ◽  
Juan-Mei Yang ◽  
Meng Zhao ◽  
Xiao-Qing Qian ◽  
Fang-Lu Chi
Keyword(s):  
Hearing Loss ◽  
Down Syndrome ◽  
Cochlear Implantation ◽  
Review Literature ◽  
Profound Hearing Loss ◽  
Rehabilitation Process ◽  
Hearing Rehabilitation ◽  
Suitability Assessment ◽  
First Case ◽  
Low Incidence

Hearing loss is not uncommon among patients with Down syndrome (DS). It has been reported in 38–78% of the Down syndrome population. However, profound hearing loss in DS patients is rarely noticed due to its low incidence. In this article, we reported two Down syndrome patients with bilateral profound hearing loss in two cases. The first case involved an eight-year-old DS child experiencing extremely severe defects in terms of language and severe defects in terms of gross motor function, adaptability, and sociability. The second case revolved around another DS child with bilateral cochlear nerve absence. We review literature on the DS patients with hearing loss and conclude that profound sensorineural hearing loss in those patients has not received enough attention so far. We also recommend that cochlear implantation (CI) suitability assessment and timely intervention via cochlear implantation are necessary in DS patients. Besides, benefits from CI would be limited and hearing rehabilitation process could be much slower when compared with children without additional inabilities.

Secondary, profound, sensorineural hearing loss after recovery from haemolytic uraemic syndrome due to enterohaemorrhagic Escherichia coli, and subsequent cochlear implantation, in two Japanese children

2013 ◽  
Vol 127 (3) ◽  
pp. 306-310
Author(s):  
S B Minami ◽  
H Takegoshi ◽  
Y Shinjo ◽  
K Kaga
Keyword(s):  
Escherichia Coli ◽  
Hearing Loss ◽  
Cochlear Implantation ◽  
Haemolytic Uraemic Syndrome ◽  
Disease Onset ◽  
Profound Hearing Loss ◽  
Enterohaemorrhagic Escherichia Coli ◽  
First Case ◽  
Hearing Function ◽  
One Year

AbstractObjectives:To describe two cases of profound hearing loss secondary to enterohaemorrhagic Escherichia coli infection, and to report the efficacy of subsequent cochlear implantation.Results:The first case was a four-year-old girl admitted to hospital with Escherichia coli O157 infection and haemolytic uraemic syndrome. Mild hearing loss was confirmed five months after discharge, progressing to profound loss three months later. At the age of seven years, she underwent cochlear implantation, with remarkable improvement in speech perception and production. The second case was a three-year-old boy admitted with haemolytic uraemic syndrome caused by Escherichia coli O111 infection. One year after disease onset, profound hearing loss was confirmed. Cochlear implantation at the age of five years produced significant recovery of auditory function.Conclusion:This study represents the first published report of secondary hearing loss after recovery from haemolytic uraemic syndrome caused by enterohaemorrhagic Escherichia coli. It indicates that cochlear implantation can restore hearing function in such patients.

scholarly journals Hearing Rehabilitation with Cochlear Implants after CyberKnife Radiosurgery of Vestibular Schwannoma: A Report Based on Four Clinical Cases

2021 ◽  
Vol 11 (12) ◽  
pp. 1646
Author(s):  
Sophia M. Häußler ◽  
Agnieszka J. Szczepek ◽  
Stefan Gräbel ◽  
Carolin Senger ◽  
Franziska Löbel ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Vestibular Schwannoma ◽  
Cochlear Implantation ◽  
Pure Tone Audiometry ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Profound Hearing Loss ◽  
Hearing Rehabilitation ◽  
Auditory Rehabilitation ◽  
Open Set

Severe sensorineural hearing loss can be a symptom of the benign tumor vestibular schwannoma (VS). The treatment of VS with non-invasive stereotactic radiosurgery (SRS) offers a high local tumor control rate and an innovative possibility of sequential hearing rehabilitation with cochlear implantation. This study evaluated the feasibility, complications, and auditory outcomes of such a therapeutic approach. Three males and one female (mean age 65.3 ± 9.4 years) scheduled for cochlear implantation and diagnosed with sporadic VS classified as T1 or T2 (according to Samii) were enrolled in this study. All patients had progressive hearing loss qualifying them for cochlear implantation. First, the tumor was treated using CyberKnife SRS. Next, sequential auditory rehabilitation with a cochlear implant (CI) was performed. Clinical outcomes and surgical feasibility were analyzed, and audiological results were evaluated using pure tone audiometry and speech recognition tests. All patients exhibited open-set speech understanding. The mean word recognition score (at 65 dB SPL, Freiburg Monosyllabic Test, FMT) improved after cochlear implantation in all four patients from 5.0 ± 10% (with hearing aid) preoperatively to 60.0 ± 22.7% six months postoperatively. Our results suggest that in patients with profound hearing loss caused by sporadic vestibular schwannoma, the tumor removal with SRS followed by cochlear implantation is an effective method of auditory rehabilitation.

Hearing Rehabilitation in a Patient with Sudden Sensorineural Hearing Loss in the Only Hearing Ear

2008 ◽  
Vol 19 (03) ◽  
pp. 267-274 ◽  
Author(s):  
David B. Hawkins
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
The Other ◽  
Profound Hearing Loss ◽  
Hearing Rehabilitation ◽  
Aural Rehabilitation ◽  
Product Manager ◽  
Software Product

A case report is presented of a 62-year-old software product manager who had normal hearing in one ear and a congenital profound hearing loss in the other ear and then sustained a sudden sensorineural hearing loss in the only hearing ear. The approach to amplification decisions, cochlear implant evaluation, and rehabilitation options are discussed. Providing aural rehabilitation and continually updating and providing new amplification options and accessories are described. Se presenta un reporte de caso de un gerente de productos de software de 62 años de edad quien tenía audición normal en un oído y un sordera congénita profunda en el otro, y quién súbitamente sufrió una sordera sensorineural súbita en el único oído con audición. Se discute el enfoque de decisiones de amplificación, la evaluación para implante coclear, y las opciones de rehabilitación. Se describen las pautas para proveer rehabilitación aural y para actualizar continuamente y aportar nuevas opciones de amplificación.

Cochlear Implant Outcomes in Patients with Neurosarcoidosis

2021 ◽  
pp. 1-7
Author(s):  
Dario Ebode ◽  
Fleur Cohen-Aubart ◽  
Stéphanie Trunet ◽  
Evelyne Ferrary ◽  
Ghizlène Lahlou ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Case Series ◽  
Corticosteroid Treatment ◽  
Auditory Neuropathy ◽  
Disease Exacerbation ◽  
Profound Hearing Loss ◽  
Hearing Recovery ◽  
Speech Performance

<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.

Delayed Cochlear Implantation in Adults With Prelingual Severe-to-Profound Hearing Loss

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

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

Cochlear Implantation Outcomes in Children With CDH23 Mutations–Associated Hearing Loss

2021 ◽  
pp. 019459982110574
Author(s):  
Kaitian Chen ◽  
Bixue Huang ◽  
Jincangjian Sun ◽  
Yue Liang ◽  
Guanxia Xiong
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Outer Hair Cell ◽  
Cell Function ◽  
Hearing Level ◽  
Case Review ◽  
Retrospective Case ◽  
Profound Hearing Loss ◽  
Tertiary Referral Center ◽  
Cadherin 23

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.

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.

Cochlear implantation in chronic otitis media

2004 ◽  
Vol 118 (1) ◽  
pp. 3-7 ◽  
Author(s):  
A. Incesulu ◽  
S. Kocaturk ◽  
M. Vural
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Cochlear Implantation ◽  
Surgical Procedure ◽  
Disease Process ◽  
Chronic Otitis Media ◽  
Profound Hearing Loss ◽  
Potential Risks ◽  
Radical Cavity

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.

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