Sensorineural hearing loss in MELAS syndrome

1997 ◽  
Vol 111 (3) ◽  
pp. 279-281 ◽  
Author(s):  
P. D. Warrick ◽  
P. Wardrop ◽  
D. W. Sim

AbstractA case of sensorineural hearing loss (SNHL) in MELAS syndrome, a variety of mitochondrial cytopathy, is presented. Mitochondrial cytopathies have gone almost unreported in the otolaryngology literature, despite evidence from a recent review that about 60 per cent of such patients suffer from SNHL (Gold and Rapin, 1994). The same review revealed that only one of 117 case reports in the period 1984–1993 contained an audiogram (Swift and Singh, 1988), and none presented sequential audiograms. However, audiometry has since been published on 23 members of a family with a mitochondrial point mutation causing only sensorineural hearing loss with no other symptoms (Vernham et al., 1994). We present a case of mitochondrial cytopathy three years after diagnosis with two sequential audiograms.

2007 ◽  
Vol 125 (3) ◽  
pp. 191-195 ◽  
Author(s):  
Ney Penteado de Castro Junior ◽  
Clemente Isnard Ribeiro de Almeida ◽  
Carlos Alberto Herrerias de Campos

Sudden sensorineural hearing loss and vertigo (SSNHLV) has multifactorial causes, of which viral, autoimmune and vascular insufficiency are the most common. The therapeutic management for SSNHLV includes antiviral drugs, corticosteroids, vasodilators, normovolemic hemodilution therapy and hyperbaric oxygen therapy. Vertebrobasilar occlusive disease and carotid occlusive disease are seldom related to SSNHLV. Discussions concerning SSNHLV caused by occlusive vascular disease are important and necessary for both neurologists and otolaryngologists, since their therapeutic management and prognosis are very different from other causes of hearing loss and vertigo. Here, we present our experience with three cases managed with interventional treatment and conduct a review and discussion on the relevant literature. We conclude that investigation of vertebrobasilar and carotid occlusive diseases is necessary in patients over 50 years of age who present SSNHLV, mild neurological symptoms and a history of arteriosclerosis, high blood pressure or thrombosis.


1989 ◽  
Vol 103 (11) ◽  
pp. 1071-1072 ◽  
Author(s):  
C. I. Timon ◽  
M. A. Walsh

AbstractTwo case reports of HIV positive patients presenting with sudden sensorineural hearing loss are described. The importance of CT scanning and cerebrospinal fluid examination are stressed. The possible aetiology and pathology of the condition are also discussed.


Cephalalgia ◽  
2012 ◽  
Vol 33 (2) ◽  
pp. 80-86 ◽  
Author(s):  
Chia-Huei Chu ◽  
Chia-Jen Liu ◽  
Jong-Ling Fuh ◽  
An-Suey Shiao ◽  
Tzeng-Ji Chen ◽  
...  

Background The pathophysiology of idiopathic sudden sensorineural hearing loss (SSNHL) is poorly understood. Few case reports have proposed a possible link between migraine and SSNHL. Objectives This study aimed to explore the risk of idiopathic SSNHL in patients with migraine in a nationwide, population-based cohort study. Methods We identified patients with migraine from the Taiwan National Health Insurance Research Database (NHIRD) between 2000 and 2009. Each migraine patient was matched with four randomly selected subjects without migraine for age, sex, and comorbidities. Both cohorts were followed up until the end of 2009. We compared the incidence rates of SSNHL in the two cohorts and identified the risk factors. Results A total of 10,280 migraine patients and 41,120 matched controls were enrolled with a median follow-up of five years. The migraine cohort had a greater risk of developing SSNHL than the matched cohort (81.6 vs. 45.7 per 100,000 person-years, incidence rate ratio (IRR) = 1.8; 95% confidence interval (CI) 1.22–2.61, p = 0.002). The Cox proportional hazards analysis revealed that among migraine subjects, comorbidity with hypertension was associated with a trend of developing SSNHL (hazard ratio (HR) = 1.92, 95% CI 0.97–3.79, p = 0.06). Conclusions This population-based study demonstrates that migraine is associated with an increased risk of idiopathic SSNHL, which, however, is a rare condition.


2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Chadi Saifan ◽  
Daniel Glass ◽  
Iskandar Barakat ◽  
Suzanne El-Sayegh

Background.Sudden sensorineural hearing loss (SSHL) caused by opiate abuse or overuse has been well documented in the medical literature. Most documented case reports have involved either heroin or hydrocodone/acetaminophen. Recently, case reposts of methadone induced SSHL have been published.Case Report.We present the case of a 31-year-old man who developed SSHL after a methadone overdose induced stupor. He was subsequently restarted on methadone at his regular dose. On follow-up audiometry exams, he displayed persistent moderately severe sensorineural hearing loss bilaterally.Discussion.This case is notable because unlike all but one previously reported case, the patient—who was restated on methadone—did not make a complete recovery.Conclusion. Methadone overuse in rare cases causes SSHL.


2016 ◽  
Vol 155 (1) ◽  
pp. 81-86 ◽  
Author(s):  
Roger Baxter ◽  
Ned Lewis ◽  
Pamela Bohrer ◽  
Theresa Harrington ◽  
Laurie Aukes ◽  
...  

Objective Case reports of sudden sensorineural hearing loss (SSHL) following vaccines have led to concerns that vaccines may rarely cause hearing loss. Because of this concern, we analyzed for an association between SSHL and vaccinations. Study Design We used a case-centered method, equivalent to a case control design using immunization dates from all matched members of the population to calculate exposure to vaccines, rather than sampling. Setting Kaiser Permanente Northern California (KPNC), 2007 to 2013. Subjects and Methods We searched KPNC databases from 2007 to 2013 for all first-time diagnoses of SSHL. We used the date of any hearing- or ear-related visit in the 60 days prior to the first SSHL diagnosis as the onset date. Using only SSHL cases immunized in the prior 9 months, we compared the vaccine exposure in several risk intervals prior to onset with the exposure to the same vaccine during the same time period in all KPNC membership, matched to sex and age. Results During the study period, >20 million vaccines were administered at KPNC. In all risk intervals prior to onset of SSHL, we found no evidence of increased risk of immunization compared with matched controls. The odds ratios for vaccination 1 week prior to SSHL were 0.965 (95% confidence interval, 0.61-1.50) for trivalent inactivated influenza vaccine (TIV); 0.842 (0.39-1.62) for tetanus, reduced diphtheria, and reduced acellular pertussis; and 0.454 (0.08-1.53) for zoster vaccine. Conclusion A large-scale analysis applying a case-centered method did not detect any association between SSHL and previous receipt of TIV or other vaccines.


2018 ◽  
Vol 132 (4) ◽  
pp. 368-371
Author(s):  
M Chroni ◽  
E Prappa ◽  
I Kokkevi

AbstractBackground:Septic emboli are an unusual cause of sudden sensorineural hearing loss, for which few reports exist in the literature.Case report:This paper presents two cases of sudden sensorineural hearing loss, initially considered as idiopathic, but which were caused by septic emboli. Hearing loss in these cases was bilateral, sequential and total. The first patient had mild fever one week prior to their presentation with sudden sensorineural hearing loss; the other patient had no additional symptoms at presentation. These patients were later diagnosed with infective endocarditis, at two and seven months following the sudden sensorineural hearing loss respectively, showing that septic emboli had been the cause of sudden sensorineural hearing loss.Conclusion:Septic emboli should be considered as a possible cause of sudden sensorineural hearing loss in cases of total hearing loss. This form of hearing loss should prompt the otolaryngologist to further investigate for infective endocarditis.


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