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.

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.

scholarly journals Detailed clinical features and genotype–phenotype correlation in an OTOF-related hearing loss cohort in Japan

2021 ◽  
Author(s):  
Yoh-ichiro Iwasa ◽  
Shin-ya Nishio ◽  
Hidekane Yoishimura ◽  
Akiko Sugaya ◽  
Yuko Kataoka ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Clinical Characteristics ◽  
Clinical Information ◽  
Auditory Neuropathy ◽  
Hearing Level ◽  
Phenotype Correlation ◽  
Profound Hearing Loss ◽  
Genotype Phenotype Correlation ◽  
Auditory Performance

AbstractMutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype–phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype–phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a “typical” phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85–90% of the patients showed a hearing level of 20–39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed “true” auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype–phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype–phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

Metachronous Sudden Sensorineural Hearing Loss: Patient Characteristics and Treatment Outcomes

2020 ◽  
Vol 162 (3) ◽  
pp. 337-342
Author(s):  
Timothy Cooper ◽  
Ronak Dixit ◽  
Candace E. Hobson ◽  
Barry E. Hirsch ◽  
Andrew A. McCall
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Case Series ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Retrospective Case ◽  
Rare Presentation ◽  
Profound Hearing Loss ◽  
The Mean

Objectives Idiopathic sudden sensorineural hearing loss (ISSNHL) is a distressing condition that can significantly affect quality of life. Unilateral ISSNHL, occurring first in 1 ear and then the contralateral ear at a separate and discrete time, is a rare presentation that we refer to as metachronous ISSNHL. Our objective was to characterize the presentation of metachronous ISSNHL and report on management and hearing outcomes. Study Design Retrospective case series. Setting Otology clinic at an academic tertiary referral center. Subjects and Methods Patients ≥18 years old presenting with metachronous ISSNHL between April 2008 to November 2017 were identified through review of the clinic electronic medical record. Metachronous ISSNHL was defined as unilateral ISSNHL occurring in temporally discrete episodes (>6 months apart) affecting both ears. Patients with identifiable causes for sudden hearing loss were excluded. Patient demographics, comorbidities, management, and audiologic outcomes were recorded. Results Eleven patients with metachronous ISSNHL were identified out of 558 patients with ISSNHL. In patients with metachronous ISSNHL, the mean ± standard deviation age at the time of ISSNHL in the second ear was 58.6 ± 15.2 years (range, 31-77 years). The mean interval between episodes was 9.6 ± 7.5 years (range, 1-22 years). Patients were treated with systemic and intratympanic steroids with variable hearing recovery; 5 patients with resultant bilateral severe to profound hearing loss underwent successful cochlear implantation. Conclusion Metachronous ISSNHL is uncommon. Treatment is similar to ISSNHL, and cochlear implantation can successfully restore hearing in individuals who do not experience recovery.

Detailed Clinical Features and Genotype-Phenotype Correlation in an OTOF-Related Hearing Loss Cohort in Japan

2021 ◽  
Author(s):  
Yoh-ichiro Iwasa ◽  
Shin-ya Nishio ◽  
Hidekane Yoishimura ◽  
Akiko Sugaya ◽  
Yuko Kataoka ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Clinical Characteristics ◽  
Clinical Information ◽  
Auditory Neuropathy ◽  
Hearing Level ◽  
Phenotype Correlation ◽  
Profound Hearing Loss ◽  
Genotype Phenotype Correlation ◽  
Auditory Performance

Abstract OTOF is one of the most frequent causes of hereditary hearing loss and a main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype-phenotype correlation in the patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype-phenotype correlation. Detailed clinical information was available for 65 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.8%) showed a “typical” phenotype, prelingual and severe-to-profound hearing loss. Forty-seven patients (72.3%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85-90% of the patients showed a hearing level of 20-39dB with cochlear implant and a CAP scale (Categories of Auditory Performance) level 6 or better. Although truncating mutations and p.R1939Q were clearly related to severe phenotype, almost half of patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.H513R, p.I1573T and p.E1910K showed “true” auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype-phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype-phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

Modiolar ossification in paediatric patients with auditory neuropathy

2017 ◽  
Vol 131 (7) ◽  
pp. 598-601 ◽  
Author(s):  
L Wang ◽  
Z Wang ◽  
F Gao ◽  
K A Peng
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Comparison Group ◽  
Case Series ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Retrospective Case ◽  
Paediatric Patients

AbstractObjective:To describe our finding of increased ossification of the modiolus in paediatric patients with auditory neuropathy who met criteria for cochlear implantation.Methods:A retrospective case series with a comparison group at a tertiary referral centre is described. Seven paediatric patients with auditory neuropathy who met criteria for and underwent cochlear implantation were identified. Fifteen paediatric implantees with bilateral profound sensorineural hearing loss were included as the comparison group. All patients underwent pre-operative computed tomography. Attenuation at the modiolus was measured in all subjects by a neuroradiologist blinded to clinical information.Results:Attenuation values in the modiolus in the auditory neuropathy patients (mean ± standard deviation = 796.2 ± 53.0 HU) was statistically significantly higher than in the comparison sensorineural hearing loss patients (267.1 ± 45.6 HU; p < 0.05, t-test).Conclusion:Patients with auditory neuropathy who meet criteria for cochlear implantation demonstrate significantly higher modiolar attenuation on computed tomography imaging, consistent with increased ossification at the modiolus.

Cochlear implantation in elderly patients: stability of outcome over time

2016 ◽  
Vol 130 (8) ◽  
pp. 706-711 ◽  
Author(s):  
O Hilly ◽  
E Hwang ◽  
L Smith ◽  
D Shipp ◽  
J M Nedzelski ◽  
...  
Keyword(s):  
Elderly Patients ◽  
Cochlear Implantation ◽  
Short Form ◽  
Age Groups ◽  
The Elderly ◽  
Long Term Results ◽  
Profound Hearing Loss ◽  
Younger Patients ◽  
Over Time

AbstractBackground:Cochlear implantation is the standard of care for treating severe to profound hearing loss in all age groups. There is limited data on long-term results in elderly implantees and the effect of ageing on outcomes. This study compared the stability of cochlear implantation outcome in elderly and younger patients.Methods:A retrospective chart review of cochlear implant patients with a minimum follow up of five years was conducted.Results:The study included 87 patients with a mean follow up of 6.8 years. Of these, 22 patients were older than 70 years at the time of implantation. Hearing in Noise Test scores at one year after implantation were worse in the elderly: 85.3 (aged under 61 years), 80.5 (61–70 years) and 73.6 (aged over 70 years;p= 0.039). The respective scores at the last follow up were 84.8, 85.1 and 76.5 (p= 0.054). Most patients had a stable outcome during follow up. Of the elderly patients, 13.6 per cent improved and none had a reduction in score of more than 20 per cent. Similar to younger patients, elderly patients had improved Short Form 36 Health Survey scores during follow up.Conclusion:Cochlear implantation improves both audiometric outcome and quality of life in elderly patients. These benefits are stable over time.

scholarly journals Hearing Screening of Newborns using Distortion Product Otoacoustic Emissions

2021 ◽  
Vol 29 (2) ◽  
pp. 189-195
Author(s):  
Vybhavi MK ◽  
Srinivas V
Keyword(s):  
Hearing Loss ◽  
Otoacoustic Emissions ◽  
Screening Tool ◽  
Hearing Screening ◽  
Distortion Product Otoacoustic Emissions ◽  
Profound Hearing Loss ◽  
Distortion Product ◽  
Lost To Follow Up ◽  
Evoked Response Audiometry

Introduction  The present study was devised to estimate the prevalence of neonatal hearing loss and document the importance of using DPOAE as a screening tool for identifying hearing loss in newborns. Materials and Methods This hospital based descriptive study was conducted from August 2018 to August 2019. A total of 928 newborn babies were included in the study. These newborn babies were subjected to hearing screening by distortion product otoacoustic emissions (DPOAE) at 24-72 hrs after birth. For pass cases, no further testing was done. For refer cases, repeat testing with DPOAE was done within 15-30 days. Newborns with refer result on repeat DPOAE testing were subjected to Brainstem evoked response audiometry (BERA) within 3 months to confirm hearing loss. Results Nine hundred and twenty eight newborn babies were screened by DPOAE. 851 newborns passed the first DPOAE hearing screening and 77 newborns gave refer result. 21 newborns were lost to follow-up. 56 newborns underwent repeat DPOAE testing and 5 newborns were referred for BERA. Amongst the 5 newborns who underwent BERA testing, one newborn was diagnosed with bilateral profound hearing loss. Hence, the prevalence of hearing loss of 1.08 per thousand newborn babies was estimated in this study. Conclusion Hearing screening of newborns using DPOAE followed by BERA in refer cases to confirm hearing loss is useful for early detection followed by timely intervention and rehabilitation.

Cochlear Implantation in Children with Postlingual Progressive Steeply Sloping High-Frequency Hearing Loss

2017 ◽  
Vol 28 (10) ◽  
pp. 913-919 ◽  
Author(s):  
Margaret A. Meredith ◽  
Jay T. Rubinstein ◽  
Kathleen C. Y. Sie ◽  
Susan J. Norton
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
High Frequency ◽  
Case Series ◽  
Hearing Preservation ◽  
Rapid Improvement ◽  
Retrospective Case ◽  
Monosyllabic Word

Background: Children with steeply sloping sensorineural hearing loss (SNHL) lack access to critical high-frequency cues despite the use of advanced hearing aid technology. In addition, their auditory-only aided speech perception abilities often meet Food and Drug Administration criteria for cochlear implantation. Purpose: The objective of this study was to describe hearing preservation and speech perception outcomes in a group of young children with steeply sloping SNHL who received a cochlear implant (CI). Research Design: Retrospective case series. Study Sample: Eight children with steeply sloping postlingual progressive SNHL who received a unilateral traditional CI at Seattle Children’s Hospital between 2009 and 2013 and had follow-up data available up to 24 mo postimplant were included. Data Collection and Analysis: A retrospective chart review was completed. Medical records were reviewed for demographic information, preoperative and postoperative behavioral hearing thresholds, and speech perception scores. Paired t tests were used to analyze speech perception data. Hearing preservation results are reported. Results: Rapid improvement of speech perception scores was observed within the first month postimplant for all participants. Mean monosyllabic word scores were 76% and mean phoneme scores were 86.7% at 1-mo postactivation compared to mean preimplant scores of 19.5% and 31.0%, respectively. Hearing preservation was observed in five participants out to 24-mo postactivation. Two participants lost hearing in both the implanted and unimplanted ear, and received a sequential bilateral CI in the other ear after progression of the hearing loss. One participant had a total loss of hearing in only the implanted ear. Results reported in this article are from the ear implanted first. Bilateral outcomes are not reported. Conclusions: CIs provided benefit for children with steeply sloping bilateral hearing loss for whom hearing aids did not provide adequate auditory access. In our cohort, significant improvements in speech understanding occurred rapidly postactivation. Preservation of residual hearing in children with a traditional CI electrode is possible.

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