scholarly journals Double challenge: cochlear implantation in the only hearing ear with progressive hearing loss following meningitis and vestibular dysfunction after implantation

2020 ◽  
Vol 15 (2) ◽  
pp. 74-76
Author(s):  
Sertac Yetiser ◽  
Kutlay Karaman
2015 ◽  
Vol 124 (1_suppl) ◽  
pp. 100S-110S ◽  
Author(s):  
Keita Tsukada ◽  
Aya Ichinose ◽  
Maiko Miyagawa ◽  
Kentaro Mori ◽  
Mitsuru Hattori ◽  
...  

Objectives: To evaluate the clinical features of Japanese DFNA9 families with mutations of the COCH gene. Methods: Mutation screening was performed using targeted next-generation sequencing (NGS) for 63 previously reported deafness genes. The progression of hearing loss and vestibular dysfunction were evaluated by pure-tone audiometry, caloric testing, cVEMP, and computed dynamic posturography. Results: We detected 1 reported mutation of p.G88E and 2 novel mutations of p.I372T and p.C542R. The patients with the novel mutations of p.I372T and p.C542R within the vWFA2 domain showed early onset progressive hearing loss, and the patients with the p.G88E mutation showed late onset hearing loss and acute hearing deterioration over a short period. Vestibular symptoms were reported in the patients with p.G88E and p.C542R. Vestibular testing was performed for the family with the p.G88E mutation. Severe vestibular dysfunction was observed in the proband, and the proband’s son showed unilateral semicircular canal dysfunction with mild hearing loss. Conclusions: Targeted exon resequencing of selected genes using NGS successfully identified mutations in the relatively rare deafness gene, COCH, in the Japanese population. The phenotype is compatible with that described in previous reports. Additional supporting evidence concerning progressive hearing loss and deterioration of vestibular function was obtained from our study.


2009 ◽  
Vol 124 (1) ◽  
pp. 86-89 ◽  
Author(s):  
M J F de Wolf ◽  
J Honings ◽  
F B M Joosten ◽  
L Hoefsloot ◽  
E A M Mylanus ◽  
...  

AbstractObjective:Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma.Design:Case report.Subjects:Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss.Results:These patients had a c.1172G>A (p.Ser391Asn) mutation in the SLC26A4 gene, which has not previously been reported and which caused Pendred or enlarged vestibular aqueduct syndrome. The diagnosis was based on the perceptive hearing loss, computed tomography findings and mutation analysis. The patients were each fitted with a cochlear implant because of their severe, progressive, perceptive hearing loss with deep fluctuations. The results were good.Conclusion:Further testing for the presence of an enlarged vestibular aqueduct is recommended when children present with sudden progression in perceptive hearing loss, whether or not in combination with head trauma. Cochlear implantation is indicated in patients with persistent, progressive hearing loss that leads to deafness. Implantation can be undertaken successfully despite cochlear hypoplasia.


2020 ◽  
pp. 1-6
Author(s):  
Lisa R. Park ◽  
Elizabeth L. Perkins ◽  
Jennifer S. Woodard ◽  
Kevin D. Brown

<b><i>Introduction:</i></b> As pediatric cochlear implant (CI) candidacy expands, children with greater degrees of residual hearing are receiving CIs. These nontraditional candidates have audiometric thresholds that meet adult manufacturer labeling but are better than current pediatric guidelines allow. The purpose of this study was to determine the impact of delayed cochlear implantation on speech perception in nontraditional pediatric CI recipients. <b><i>Methods:</i></b> Pediatric CI recipients with a history of progressive hearing loss and a preoperative 4-frequency pure-tone average of ≤75 dB HL at the time of implantation were considered for this retrospective study. Preoperative serial audiograms and word recognition scores were reviewed, and a method was created to establish a date when each individual ear 1st met nontraditional candidacy. The length of time between the date of candidacy and implantation was calculated and defined as the “delay time.” A multiple linear regression investigated delay time, age at surgery, surgery type (1st vs. 2nd side), and array type as predictive factors of maximum postoperative Consonant-Nucleus-Consonant (CNC) word scores. A one-way ANCOVA was performed comparing the postoperative CNC scores between subjects grouped by delay time. <b><i>Results:</i></b> A significant regression was found (<i>F</i>(4, 38) = 5.167, <i>p</i> = 0.002, <i>R</i><sup>2</sup> = 0.353). Both age at implantation (<i>p</i> = 0.023) and delay time (<i>p</i> = 0.002) predicted CNC word scores. Longer delay time was associated with poorer word recognition scores, while older age at implantation correlated with higher CNC word scores in this progressive hearing loss group. A significant difference was noted between subjects implanted with &#x3c;1 year of delay and those with 3 or more years of delay (<i>p</i> = 0.003). All ears implanted within a year of candidacy achieved word recognition abilities that are generally accepted as above average (<i>M</i> = 84.91). <b><i>Conclusion:</i></b> CI candidacy for adults has evolved to allow for greater degrees of residual hearing, while audiometric guidelines for children have not changed since 2000. Our findings suggest that delay of cochlear implantation, even for children with significant levels of residual hearing, leads to poorer outcomes. Modified candidacy guidelines for children should be established to expedite referral to multidisciplinary CI teams and minimize delays in this population.


2010 ◽  
Vol 37 (2) ◽  
pp. 223-228 ◽  
Author(s):  
Kumiko Yukawa ◽  
Akira Hagiwara ◽  
Yasuo Ogawa ◽  
Nobuhiro Nishiyama ◽  
Shigetaka Shimizu ◽  
...  

2007 ◽  
Vol 3;10 (5;3) ◽  
pp. 467-472
Author(s):  
Jeffrey T. Vrabec

Background: The hydrocodone/acetaminophen combination is one of the most commonly used analgesic preparations. Isolated incidences of suspected association between hydrocodone abuse and rapidly progressive hearing loss have been reported. In this study, we describe the clinical characteristics of 5 patients presenting with progressive hearing loss and a history of hydrocodone use. Methods: Patients presenting with rapidly progressive bilateral hearing loss who had a documented history of hydrocodone use were selected for the study. The presentation, audiologic findings, associated comorbidities, and treatment outcomes were reviewed Results: All patients displayed rapidly progressive sensorineural hearing loss without vestibular symptoms. Hearing loss was asymmetric in 3 patients at initial presentation, but progressed to profound loss, usually within months. Steroid treatment has no effect on the progression of the hearing loss. The admitted quantity of hydrocodone consumed ranged from 10 to 300 mg per day. Hepatitis C was the most common comorbidity, present in 60% of the patients. All patients underwent cochlear implantation with satisfactory results. Conclusions: The chronic use of hydrocodone can be associated with progressive sensorineural hearing loss. Successful auditory rehabilitation can be achieved with cochlear implantation. Genetic polymorphisms of drug metabolizing enzymes as well as associated comorbidities such as hepatitis C infection may be significant in the development of hydrocodone ototoxicity, though additional investigations are necessary. Key words: hydrocodone, sensorineural hearing loss, cochlear implant


2018 ◽  
Vol 22 (4) ◽  
pp. 25-30
Author(s):  
Sergey A. Ivanov ◽  
Sergey G. Zhuravskii

Paget’s disease of bone is a localized disorder of bone remodeling. These functional and structural alterations, interacting with the specific characteristics of the site of involvement, account for most of the complications of the disease. Bilateral progressive hearing loss is the most frequently encountered complication of Paget’s disease, because of the involvement of the temporal bones. There may be difficulty in distinguishing patients with presbycusis from those with Paget’s disease-related hearing loss. Pagetic hearing loss seems to be stabilized by effective medical treatment. For patients with severe hearing loss, cochlear implantation may be recommended.


2014 ◽  
Vol 23 (2) ◽  
pp. 189-194 ◽  
Author(s):  
Celia Zazo Seco ◽  
Anne MM Oonk ◽  
María Domínguez-Ruiz ◽  
Jos MT Draaisma ◽  
Marta Gandía ◽  
...  

2020 ◽  
Vol 26 (1) ◽  
pp. 31-36
Author(s):  
Md Zakaria Sarkar ◽  
AHM Ferdows Nur ◽  
Utpal Kumar Dutta ◽  
Muhammad Rafiqul Islam ◽  
Debabrota Roy ◽  
...  

Objective: The aim of this study was to evaluate hearing outcome after stapedotomy in patients with Otosclerosis. Methods: This cross sectional study was carried out from July 2017 to January 2019 in National Institute of ENT, Unit V. About 22 patients with Otosclerosis were included in this study. Diagnosis of Otosclerosis was based on the history, medical status with Otoscopy, Tuning fork tests and Audiometric tests. We compiled data on the pre and post operative air-bone gap (ABG) at 0.5, 1, 2 KHZ. The ABG was Calculated using AC and BC thresholds on the same audiogram. Post operative hearing gain was then Calculated from the ABG before the operation minus the ABG of the last follow up examination Results: In this study most of the cases were age group 14-30 years (72.7%), female (54.5%). Most common symptoms was progressive hearing loss, tinnitus (77.8%).The average preoperative hearing loss in this study was (AC) was 48.31±7.68. The average post opt. hearing (AC) at follow up was 28.95±10.30 with an average hearing gain of 15.40±8.53 dB which was significant. The average pre-operative ABG was 28.99 dB ± 8.10. The average post opt. ABG was analyzed at 1 follow up showed ABG 13.18±8.09 dB which was found to be significant. Conclusion: Stapedotomy is an effective surgical procedure for the treatment of otosclerosis which leads to improvement in patient’s quality of life. A favorable hearing outcome can be obtained by the combination of experienced hands with minimal surgical trauma and appropriate surgical technique. Bangladesh J Otorhinolaryngol; April 2020; 26(1): 31-36


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