scholarly journals The Long-Term Efficacy of Cochlear Implantation for Hearing Loss in Muckel-Wells Syndrome

2019 ◽  
Vol 15 (3) ◽  
pp. 454-458
Author(s):  
Bakushi Ogawa ◽  
◽  
Mitsuhiro Aoki ◽  
Hidenori Ohnishi ◽  
Toshimitsu Ohashi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Term Efficacy ◽  
Wells Syndrome

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.

The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis

2017 ◽  
Vol 22 (4-5) ◽  
pp. 205-217 ◽  
Author(s):  
Jacqueline J. Greene ◽  
Ilka C. Naumann ◽  
Janet M. Poulik ◽  
Kevin T. Nella ◽  
Lindsay Weberling ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Psychiatric Symptoms ◽  
Altered Mental Status ◽  
Rapid Onset ◽  
Normal Serum ◽  
High Dose ◽  
Leptomeningeal Enhancement ◽  
History Of

Background: A rare subset of sarcoidosis, neurosarcoidosis, is reported to occur in 5-7% of sarcoid patients and can manifest in a variety of ways. The most common are facial paralysis and optic neuritis, less commonly causing cochleovestibulopathy, blindness, anosmia, and other cranial nerve (CN) palsies. The sensory deficit may be severe and psychiatric symptoms may result from the effects of the disease or steroid treatment. Although MRI-compatible cochlear implants are now available, concerns about the feasibility of recoverable hearing with cochlear implantation in these patients as well as the practical difficulty of disease monitoring due to implant artifact must be considered. Results: We present 3 recent cases from different institutions. The first is a 39-year-old man with a history of progressively worsening hearing loss, followed by visual loss, delusions, agitation, ataxia, and musical auditory hallucinations, diffuse leptomeningeal enhancement on MRI with a normal serum angiotensin-converting enzyme (ACE) level but elevated cerebrospinal fluid (CSF) ACE levels, suggesting neurosarcoidosis, was treated with corticosteroids, and underwent successful cochlear implantation. The second is a 36-year-old woman with rapid-onset horizontal diplopia, left mixed severe sensorineural hearing loss (SNHL) and tinnitus, diffuse leptomeningeal enhancement on MRI, and progressive palsy of the left CNs IV, VI, VII, IX, X and XI, with altered mental status requiring admission following high-dose intravenous corticosteroids. The third is a 15-year-old boy who presented with sudden, bilateral, profound SNHL, recurrent headaches, and left facial weakness refractory to antivirals, ultimately diagnosed with neurosarcoidosis following an aborted cochlear implantation where diffuse inflammation was found, and histopathology revealed Schaumann bodies; he was treated with methotrexate and later underwent successful cochlear implantation. Conclusions: Neurosarcoidosis is an elusive diagnosis and can cause hearing loss and psychiatric symptoms. Cochlear implantation for patients with severe hearing loss should be considered once the diagnosis is confirmed, as it is possible to achieve a successful level of hearing. Psychiatric symptoms can manifest with the onset of neurosarcoidosis, result from CN deficits, or develop as a side effect from long-term, high-dose corticosteroids, and should be monitored carefully in patients with neurosarcoidosis.

scholarly journals Long‐term outcomes of cochlear implantation in patients with high‐frequency hearing loss

2018 ◽  
Vol 128 (8) ◽  
pp. 1939-1945 ◽  
Author(s):  
J. Thomas Roland ◽  
Bruce J. Gantz ◽  
Susan B. Waltzman ◽  
Aaron J. Parkinson
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
High Frequency ◽  
Long Term Outcomes ◽  
High Frequency Hearing Loss

Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder: Long-Term Outcomes

2012 ◽  
Vol 23 (01) ◽  
pp. 005-017 ◽  
Author(s):  
Alyce I. Breneman ◽  
René H. Gifford ◽  
Melissa D. DeJong
Keyword(s):  
Hearing Loss ◽  
Retrospective Study ◽  
Speech Recognition ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Auditory Neuropathy ◽  
Long Term Outcomes ◽  
Expected Outcomes ◽  
Cochlear Nerve Deficiency

Background: Best practices concerning the audiological management of the child diagnosed with auditory neuropathy spectrum disorder (ANSD) have not been definitively defined nor fully understood. One reason is that previous studies have demonstrated conflicting findings regarding the outcomes of cochlear implantation for children with ANSD. Thus, the question remains whether children with ANSD are able to achieve similar outcomes following cochlear implantation as those children with sensorineural hearing loss (SNHL). Purpose: To assess speech perception outcomes for children with cochlear implants who have a diagnosis of ANSD as well as their age-matched peers who have sensorineural hearing loss. Research Design: Retrospective study Study Sample: Thirty-five subject pairs (n = 70) ranging in age at implant activation from to 10 to 121 mo (mean 39.2 mo) were included in this retrospective study. Subjects were matched on variables including age at initial implant activation and months of implant use at postoperative test point. Data Collection and Analysis: Speech recognition scores for monosyllabic and multisyllabic stimuli were compared across the subject groups. For those not developmentally and/or linguistically ready for completion of open-set speech recognition testing with recorded stimuli, GASP (Glendonald Auditory Screening Procedure) word recognition and/or questionnaire data using either the LittlEARS or Meaningful Auditory Integration Scale were compared across the groups. Statistical analysis using a repeated-measures analysis of variance (ANOVA) evaluated the effects of etiology (ANSD or SNHL) on postoperative outcomes. Results: The results of this study demonstrate that children with ANSD can clearly benefit from cochlear implantation and that their long-term outcomes are similar to matched peers with SNHL on measures of speech recognition. There were no significant differences across the ANSD and SNHL groups on any of the tested measures. Conclusion: Cochlear implantation is a viable treatment option for children with a diagnosis of ANSD who are not making auditory progress with hearing aids that have been fit using the Desired Sensation Level method (DSL v5.0). Expected outcomes of cochlear implantation for children with ANSD, excluding children with cochlear nerve deficiency, are no different than for children with non-ANSD SNHL. These results are important for counseling families on the expected outcomes and realistic expectations following cochlear implantation for children with ANSD who demonstrate no evidence of cochlear nerve deficiency.

scholarly journals Identification of Novel Candidate Genes and Variants for Hearing Loss and Temporal Bone Anomalies

Genes ◽  
2021 ◽  
Vol 12 (4) ◽  
pp. 566
Author(s):  
Regie Lyn P. Santos-Cortez ◽  
Talitha Karisse L. Yarza ◽  
Tori C. Bootpetch ◽  
Ma. Leah C. Tantoco ◽  
Karen L. Mohlke ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Candidate Genes ◽  
Cochlear Implantation ◽  
Long Term Effects ◽  
Clinical Genetic ◽  
Exome Data ◽  
Novel Variants ◽  
Children With Hearing Loss ◽  
Bone Anomalies

Background: Hearing loss remains an important global health problem that is potentially addressed through early identification of a genetic etiology, which helps to predict outcomes of hearing rehabilitation such as cochlear implantation and also to mitigate the long-term effects of comorbidities. The identification of variants for hearing loss and detailed descriptions of clinical phenotypes in patients from various populations are needed to improve the utility of clinical genetic screening for hearing loss. Methods: Clinical and exome data from 15 children with hearing loss were reviewed. Standard tools for annotating variants were used and rare, putatively deleterious variants were selected from the exome data. Results: In 15 children, 21 rare damaging variants in 17 genes were identified, including: 14 known hearing loss or neurodevelopmental genes, 11 of which had novel variants; and three candidate genes IST1, CBLN3 and GDPD5, two of which were identified in children with both hearing loss and enlarged vestibular aqueducts. Patients with variants within IST1 and MYO18B had poorer outcomes after cochlear implantation. Conclusion: Our findings highlight the importance of identifying novel variants and genes in ethnic groups that are understudied for hearing loss.

scholarly journals Long-Term Asymmetric Hearing Affects Cochlear Implantation Outcomes Differently in Adults with Pre- and Postlingual Hearing Loss

PLoS ONE ◽  
2015 ◽  
Vol 10 (6) ◽  
pp. e0129167 ◽  
Author(s):  
Isabelle Boisvert ◽  
Catherine M. McMahon ◽  
Richard C. Dowell ◽  
Björn Lyxell
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation

scholarly journals Long Term Speech Perception Outcomes of Cochlear Implantation in Gap Junction Protein Beta 2 Related Hearing Loss

2017 ◽  
Vol 21 (2) ◽  
pp. 95-102 ◽  
Author(s):  
Sung Hee Kim ◽  
Rajendra Nepali ◽  
Myung Hoon Yoo ◽  
Kwang-Sun Lee ◽  
Jong Woo Chung
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Gap Junction ◽  
Cochlear Implantation ◽  
Junction Protein ◽  
Gap Junction Protein ◽  
Beta 2

Protective Effect of Systemic Administration of Erythropoietin on Auditory Brain Stem Response and Compound Action Potential Thresholds in an Animal Model of Cochlear Implantation

2011 ◽  
Vol 120 (11) ◽  
pp. 737-747 ◽  
Author(s):  
Stéphanie Quesnel ◽  
Yann Nguyen ◽  
Pierre Campo ◽  
Olivier Hermine ◽  
Jean-Antoine Ribeil ◽  
...  
Keyword(s):  
Animal Model ◽  
Hearing Loss ◽  
Brain Stem ◽  
Cochlear Implantation ◽  
Hearing Threshold ◽  
Systemic Administration ◽  
Auditory Brain Stem Response ◽  
Auditory Brain Stem ◽  
Compound Action

Objectives: An animal model of cochlear implantation has been developed, and the hearing threshold was evaluated after different surgical procedures. The effect of perioperative systemic administration of erythropoietin on the hearing loss induced by cochlear implantation was tested. Methods: Twenty-nine guinea pigs with normal hearing underwent implantation of a 254-μm-diameter array through a cochleostomy. The effects on hearing of cochleostomy and transient and long-term array implantation (21 days) were assessed by testing of the auditory brain stem responses and compound action potentials. Eleven implanted animals received intraperitoneal administration of erythropoietin. Selected computed tomographic scans and cochlear histologic studies were performed 1 month after implantation to confirm proper placement of the array. The erythropoietin concentration at the time of surgery was assessed in samples of perilymph, cerebrospinal fluid, and blood. Results: The cochleostomy and transient array insertion had no effect on hearing thresholds. Long-term array implantation induced a stable decrease of hearing threshold (30 dB), a decrease that was reduced by 12 dB in erythropoietin-treated animals. The erythropoietin-treated animals had better hearing preservation at higher frequencies. Fibrosis surrounding the array was seen in both groups. Conclusions: The hearing loss observed was probably due to the presence of the array in the cochlea. The intraperitoneal injection of erythropoietin improved the hearing threshold shift induced by implantation.

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