Cochlear implantation in a bilateral sensorineural hearing loss patient with relapsing polychondritis

2010 ◽  
Vol 32 (2) ◽  
pp. 479-482 ◽  
Author(s):  
Young Joon Seo ◽  
Jae Young Choi ◽  
Si Hong Kim ◽  
Tae-Jung Kim
2013 ◽  
Vol 127 (7) ◽  
pp. 708-711 ◽  
Author(s):  
A C Hall ◽  
A C Leong ◽  
D Jiang ◽  
A Fitzgerald-O'Connor

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.


2007 ◽  
Vol 17 (2) ◽  
pp. 148-152 ◽  
Author(s):  
Tokutaro Tsuda ◽  
Ayako Nakajima ◽  
Sayumi Baba ◽  
Kiyoko Tanohara ◽  
Ikuko Masuda ◽  
...  

Author(s):  
Masoud Motasaddi Zarandy ◽  
Hakima Abdullah ◽  
Mina Motasaddi Zarandy ◽  
Mohammadreza Firouzifar ◽  
Farzad Moubedshahi

Background: Osteopetrosis (OP) is a rare disease of the skeletal system that can be associated with complications such as bone fracture, nerve dysfunction and deafness due to increased bone density and reduced bone quality. In this regard and due to the challenge that it can cause for cochlear implantation (CI), in this study we aimed to report CI conducted on two patients with OP in Iran. The Case: Patients were two women diagnosed with OP and bilateral sensorineural hearing loss (SNHL) who underwent CI in the right ear. Preoperative PTA showed a hearing threshold decrease of more than 100 dB in both patients. A standard cochleostomy was performed in one patient and endoscopic surgery in the other patient through the external ear canal. One month after surgery, the hearing threshold improved b y 60−90 dB in both patients. No facial nerve palsy or implant extrusion/migration was observed after surgery. Conclusion: Although technically challenging, CI seems to be a safe and effective method to improve the SNHL in patients with OP. The path for electrode insertion should be tailored to meet the conditions and anatomy of patients. Keywords: Cochlear implantation; osteopetrosis; sensorineural hearing loss


2019 ◽  
Vol 73 (3) ◽  
pp. 1-5 ◽  
Author(s):  
Witold Szyfter ◽  
Michał Karlik ◽  
Alicja Sekula ◽  
Simon Harris ◽  
Wojciech Gawęcki

Introduction: Surgical treatment of deafness by cochlear implants is used for more than 40 years, and during this period permanently, gradual and significant expansion of indications for this surgery has been observed. Material and methods: In our Department in the years 1994-2018 1480 cochlear implantations were performed, both in adults (647) and in children (883). In this study current indications and the rules for eligibility of patients based on 25 years of experience are presented. Results: Indications for cochlear implantation in adults are: 1) bilateral postlingual deafness, 2) bilateral sensorineural hearing loss - in pure tone audiometry > 70 dB HL (average 500-4000 Hz) and in speech audiometry in hearing aids understanding < 50% of words for the intensity of the stimulus 65 dB, in the absence of the benefits of hearing aids, 3) bilateral profound hearing loss for high frequency with good hearing for low frequency, in the absence of the benefits of hearing aids, 4) some cases of asymmetric hearing loss with intensive tinnitus in the deaf ear. An indication in children is bilateral sensorineural hearing loss > 80dB HL confirmed by hearing tests, after about 6 months of rehabilitation with the use of hearing aids. Discussion: Although cochlear implantation is used for more than 40 years, the indications for this treatment underlies constant modifications. They concern the age of eligible patients, implantation in patients with partially preserved hearing, as well as treatment for patients with difficult anatomical conditions. In many countries, bilateral implantations are commonly performed, and more and more centers recommend this treatment in the case of unilateral deafness or asymmetric hearing loss, especially with the accompanying tinnitus in the deaf ear.


1998 ◽  
Vol 77 (4) ◽  
pp. 300-303 ◽  
Author(s):  
Eric W. Sargent ◽  
Douglas L. Beck

Although 1–4% of all cases of sudden sensorineural hearing loss (SSHL) are bilateral, all such patients reported to date have experienced significant recovery of hearing in at least one ear. We report a case of profound, bilateral idiopathic SSHL without recovery which was treated with cochlear implantation; the first such report to our knowledge. The patient achieved open-set spondee recognition. Individuals with sudden bilateral hearing loss in whom treatable causes have been eliminated may benefit from cochlear implantation.


2009 ◽  
Vol 124 (1) ◽  
pp. 105-107 ◽  
Author(s):  
S Cheng ◽  
M da Cruz

AbstractObjective:We report a case of Sweet's disease associated with rapid, profound loss of hearing, against a background of progressive, bilateral, sensorineural hearing loss.Results:The clinical features were indistinguishable from those of immune-mediated inner ear disease. Establishment of a definitive diagnosis was a challenge due to the absence of a reliable diagnostic test. The patient was unresponsive to extensive immunosuppressive therapy and subsequently underwent cochlear implantation, with good hearing outcomes.Conclusions:Profound, bilateral, sensorineural hearing loss in the context of Sweet's disease may be related to the underlying immunological aetiology. Cochlear implantation can successfully restore hearing when immunotherapy fails.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Mauro Scarpelli ◽  
Francesca Zappini ◽  
Massimiliano Filosto ◽  
Anna Russignan ◽  
Paola Tonin ◽  
...  

Hearing impairment is common in patients with mitochondrial disorders, affecting over half of all cases at some time in the course of the disease. In some patients, deafness is only part of a multisystem disorder. By contrast, there are also a number of “pure” mitochondrial deafness disorders, the most common probably being maternally inherited. We retrospectively analyzed the last 60 genetically confirmed mitochondrial disorders diagnosed in our Department: 28 had bilateral sensorineural hearing loss, whereas 32 didn't present ear's abnormalities, without difference about sex and age of onset between each single group of diseases. We reported also a case of MELAS patient with sensorineural hearing loss, in which cochlear implantation greatly contributed to the patient's quality of life. Our study suggests that sensorineural hearing loss is an important feature in mitochondrial disorders and indicated that cochlear implantation can be recommended for patients with MELAS syndrome and others mitochondrial disorders.


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