Deafness in Vogt–Koyanagi–Harada syndrome

2006 ◽  
Vol 120 (5) ◽  
pp. 416-418 ◽  
Author(s):  
A Sil ◽  
P Chatrath ◽  
D J Gatland

Sudden onset sensorineural hearing loss is a well recognized entity frequently encountered in otolaryngological practice. However, the combination of such deafness as part of a wider systemic disorder is fortunately rare. Almost 100 years after the syndrome was classified, we describe a case of Vogt–Koyanagi–Harada syndrome occurring unusually in a Caucasian woman and characterized by sudden hearing loss. A brief review of this rare condition is presented.

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.


1992 ◽  
Vol 101 (10) ◽  
pp. 827-831 ◽  
Author(s):  
Leonard P. Berenholz ◽  
Christopher Eriksen ◽  
Fayne A. Hirsh

Sensorineural hearing loss of sudden onset may be the presenting symptom in up to 14% of patients with acoustic neuroma. We present the first reported case of sudden hearing loss in an only hearing ear with recovery to normal levels after steroid therapy on four separate occasions. Evaluation revealed a 1.5-cm acoustic neuroma. After middle cranial fossa decompression, a fifth episode with recovery after steroid use was documented. A review of the recent literature is presented, emphasizing the possible causation of sudden sensorineural hearing loss with recovery to normal in patients with acoustic neuroma. Modalities of therapy for the dilemma of the acoustic neuroma in an only hearing ear are discussed, including surgery, radiotherapy, and chemotherapy. An aggressive approach to the evaluation of the cause of sudden hearing loss is suggested.


2001 ◽  
Vol 115 (11) ◽  
pp. 907-910 ◽  
Author(s):  
H. S. Uppal ◽  
C. A. Ayshford ◽  
F. Wilson

Diffuse infiltration of the meninges by metastatic carcinoma (meningeal carcinomastosis) is a potential complication of systemic malignancy. It may present with a variety of neurological symptoms as any aspect of the neuraxis can be affected. Often there is a history of pre-existing malignancy.The authors describe a case with an initial presentation of sudden onset profound bilateral sensorineural hearing loss. The underlying pathology was found to be an occult breast carcinoma, a previously unreported finding.The role of cerebrospinal fluid cytology and radio-imaging in diagnosis is discussed. All previously reported cases of sudden hearing loss and meningeal carcinomatosis are reviewed.


2007 ◽  
Vol 137 (1) ◽  
pp. 74-78 ◽  
Author(s):  
Guillermo Plaza ◽  
Carlos Herráiz

OBJECTIVE: We sought to describe our experience with intratympanic steroid treatment of sudden sensorineural hearing loss after failure of intravenous steroid treatment. STUDY DESIGN AND SETTING: We conducted a nonrandomized prospective clinical trial. Fifty patients presenting with sudden onset idiopathic hearing loss were treated intravenously over five days. After this period, patients with treatment failure (18 cases) were offered intratympanic steroid treatment. Nine patients refused, whereas the other nine patients received three weekly injections of methylprednisolone. Recovery of hearing was reported as improvement of more than 15 dB in pure tone average. RESULTS: Intratympanic steroid treatment improved hearing loss in five patients (55%). This is significant compared with those patients who refused intratympanic treatment, who showed no further improvement ( P < 0.05). No serious adverse effects were observed. CONCLUSION: Intratympanic steroids significantly improve the recovery outcome of sudden hearing loss that had not recovered after intravenous steroid treatment. SIGNIFICANCE: Intratympanic steroids are an effective and safe therapy in sudden sensorineural hearing loss cases that are refractory to standard treatment.


1981 ◽  
Vol 89 (2) ◽  
pp. 283-287 ◽  
Author(s):  
Joe B. Colclasure ◽  
Sharon S. Graham

Reports of sudden hearing loss as the first sign of an intracranial aneurysm are sparse and published primarily in the neurologic literature. A case report is presented in which the initial signs and symptoms of a lobular aneurysm in the posterior communicating artery were the sudden onset of bilateral sensorineural hearing loss, tinnitus, and headache. Following evaluation and identification of the aneurysm, this patient underwent a craniotomy with clipping of the aneurysm; hearing sensitivity improved dramatically following surgery. The world literature is reviewed for cases in which aneurysms have initially occurred as hearing loss, tinnitus, or both. Intracranial aneurysm is discussed as a rare, potential source of a sudden sensorineural hearing loss.


2018 ◽  
Vol 23 (1) ◽  
pp. 58-62
Author(s):  
Kumiko Hosokawa ◽  
Seiji Hosokawa ◽  
Satoru Takebayashi ◽  
Hiroyuki Mineta

Acoustic neuroma sometimes presents with sudden-onset sensorineural hearing loss as a primary symptom. We investigated 848 untreated cases that included 20 cases with acoustic neuroma with sudden-onset sensorineural hearing loss and 828 cases without acoustic neuroma. Fourteen of the 20 acoustic neuroma and 90 of the 828 cases of sudden-onset sensorineural hearing loss showed a trough-shaped audiogram with the greatest amount of hearing loss in the mid-frequency range. The incidence of a trough-shaped audiogram was significantly higher in patients with acoustic neuroma than in those without (p < 0.01). This study suggests that a trough audiogram is a significant finding in patients with sudden-onset sensorineural hearing loss and indicates the presence of acoustic neuroma.


Life ◽  
2022 ◽  
Vol 12 (1) ◽  
pp. 96
Author(s):  
Magdalena B. Skarżyńska ◽  
Aleksandra Kołodziejak ◽  
Elżbieta Gos ◽  
Milaine Dominici Sanfis ◽  
Piotr H. Skarżyński

(1) Background: A retrospective clinical study was conducted to compare the effectiveness of different pharmacological and non-pharmacological regimens for treating sudden sensorineural hearing loss (SSNHL). (2) Methods: Adult patients (n = 130) diagnosed with sudden sensorineural hearing loss (SSNHL) and hospitalized between 2015 and 2020 were enrolled in this study. Depending on the treatment regimen applied, patients were divided into five groups. Inclusion criteria were as follows: (i) hearing loss of sudden onset; (ii) hearing loss of at least 30 dB at three consecutive frequencies; (iii) unilateral hearing loss; (iv) age above 18 years. Exclusion criteria were as follows: (i) no follow-up audiogram; (ii) bilateral hearing loss; (iii) recognized alternative diagnosis such as tumor, disorder of inner ear fluids, infection or inflammation, autoimmune disease, malformation, hematological disease, dialysis-dependent renal failure, postdural puncture syndrome, gene-related syndrome, mitochondrial disease; and (iv) age below 18 years. (3) Results: Complete recovery was found in 14% of patients (18/130) and marked improvement was found in 6% (8/130), giving an overall success rate of 20%. The best results were obtained in the second group (i.e., patients given intratympanic glucocorticoid + prolonged orally administered glucocorticoid) where the success rate was 28%. In general, the older the patient, the smaller the improvement in hearing, a correlation that was statistically significant. (4) Conclusions: In treating SSNHL, the highest rate of hearing recovery—28%—was in the group of patients given intratympanic corticoid plus prolonged treatment with orally administered glucocorticoid.


Author(s):  
Saeid Aarabi ◽  
Nasrin Yazdani ◽  
Javad Fakhri ◽  
Vida Rahimi ◽  
Parsa Cheraghipour ◽  
...  

Introduction: This study aimed to investigate the relationship between sudden sensorineural hearing loss, vestibular neuritis, and infection with COVID-19. Materials and Methods: In this study, a total of 56 Iranians (32 females and 24 males) with a Mean±SD age of 45.12±14 years were studied in Tehran City, Iran. Individuals diagnosed with Sudden Sensorineural Hearing Loss (SSNHL) or vestibular neuritis based on definitive diagnostic criteria were included in the study. The methodology comprised four sections of underlying Sudden Hearing Loss,, auditory and vestibular inspection, SARS-CoV-2 Reverse Transcription-Polymerase Chain Reaction (RT-PCR) test, and statistical analysis. Also, the videonystagmography test was used in participants with vertigo to diagnose vestibular neuritis. Pure tone audiometry confirmed SSNHL in some patients with a complaint of hearing loss. Furthermore, tuning fork, Rinne and Weber tests were also performed. Results: The results of SARS-CoV-2 RT-PCR in 56 subjects showed that eight subjects (22.2%) with vestibular neuritis and two with SSNHL (10%) had a positive RT-PCR test. The Chi- square and Fisher exact-tests with a 95% confidence interval revealed no statistically significant (P>0.05) relationship between COVID-19 infection and vestibular neuritis or SSNHL. Conclusion: The present study showed no statistically significant relationship between audiovestibular disorders and positive SARS-CoV-2 RT-PCR test. However, the possibility of this relationship cannot be ruled out, and there is a need for studies with larger sample sizes.


2013 ◽  
Vol 127 (2) ◽  
pp. 196-199 ◽  
Author(s):  
L-S Tseng ◽  
S-D Luo

AbstractObjective:We describe a rare case of sudden onset of unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease, which is characterised by progressive stenosis of the intracranial internal carotid arteries and their proximal anterior cerebral arteries and middle cerebral arteries.Method:Case report and review of the world literature regarding moyamoya disease with hearing loss.Results:The reported patient had moyamoya disease that initially presented as sudden, unilateral sensorineural hearing loss. Magnetic resonance imaging showed occlusion of the anterior cerebral, middle cerebral and distal internal carotid arteries bilaterally. The possible mechanism of this patient's sudden sensorineural hearing loss may have been vascular occlusion resulting from thrombotic narrowing or blockage by plaque.Conclusion:The described patient represents the first reported case of sudden onset, unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease. The possibility of a vascular lesion such as moyamoya disease should be considered in patients with sudden sensorineural hearing loss, especially children, young adults and Asian patients. Due to this disease's poor outcome, early diagnosis and treatment are important to prevent stroke.


2014 ◽  
Vol 151 (1_suppl) ◽  
pp. P238-P239
Author(s):  
Kavita K. Dedhia ◽  
David H. Chi

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