Intratympanic injection of dexamethasone after failure of intravenous prednisolone in simultaneous bilateral sudden sensorineural hearing loss.

PURPOSE: This study aimed to analyze outcomes of intratympanic injection of dexamethasone after failure of intravenous prednisolone in simultaneous bilateral sudden sensorineural hearing loss (SSNHL). MATERIALS AND METHODS: The cases of simultaneous bilateral SSNHL treated in our hospital from March 2007 to March 2018 were retrospectively analyzed. During the earlier period (March 2007 to February 2012), the cases were treated by intravenous prednisolone only, and classified into group A. During the late period (February 2012 to March 2018), intratympanic injection of dexamethasone after failure of intravenous prednisolone therapy was employed to treat simultaneous bilateral SSNHL, and these patients were enrolled in group B. Effective rates of the two treatment modalities in groups A and B were compared. RESULTS: In group A, 3 of 40 ears obtained complete recovery, and 4 ears achieved partial recovery after intravenous prednisolone treatment, with the effective rate of only 17.5% (7/40 ears). In contrast, 6 of 44 ears in group B achieved complete recovery, and 10 ears got partial recovery, with the effective rate of 36.4% (16/44 ears). There was significant difference in the effective rate between the two groups. CONCLUSION: Intratympanic injection of dexamethasone after failure of intravenous prednisolone therapy was a better choice for simultaneous bilateral SSNHL compared to traditional intravenous prednisolone therapy.

Case Report: Neurobrucellosis with Plastered Spinal Arachnoiditis: A Magnetic Resonance Imaging-Based Report.

Diffuse spinal arachnoiditis in neurobrucellosis is a rare manifestation. We report a boy aged 17, presenting with hearing impairment and recurrent vomiting for 18 months, weight loss for 12 months, dysphagia, dysarthria, hypophonia for 6 months, and gait unsteadiness for 5 months. He had bilateral 5th (motor) to 12th cranial nerve palsy, wasting and weakness of limbs, fasciculations, absent tendon reflexes, and positive Babinski's sign. Cerebrospinal fluid (CSF) showed raised protein and pleocytosis. Magnetic resonance imaging (MRI) showed extensive enhancing exudates in cisterns and post-contrast enhancement of bilateral 5th, 6th, 7th, and 8th nerves. Spine showed clumping with contrast enhancement of the cauda equina roots and encasement of the cord with exudates. Serum and CSF were positive for anti-Brucella antibodies. He showed significant improvement with antibiotics. At 4 months follow-up, MRI demonstrated near complete resolution of cranial and spinal arachnoiditis. It is important to recognize such rare atypical presentations of neurobrucellosis.

Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis presenting with acute sensorineural hearing loss: a case report and review of the literature.

Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening catastrophic and rarely seen complication of EBV infection especially in adults. While typical presentation of EBV infection is easily diagnosed as mononucleosis syndrome in teenagers and adults, some atypical clinical presentations may be challenged. We did not encounter any patient presenting with sudden sensorineural hearing loss associated with EBV infection in our English medical literature research (1966-2016). In this study, we report an adult patient who was complicated with EBV-HLH under high dose steroid therapy after diagnosis as sensorineural hearing loss. Our aim is to emphasise the atypical presentation of EBV infection and to discuss steroid therapy complication in sensorineural hearing loss that had been simply defined as idiopathic.

Oral steroids for the resolution of otitis media with effusion (OME) in children (OSTRICH): study protocol for a randomised controlled trial.

BACKGROUND: Otitis media with effusion (OME) is an accumulation of fluid in the middle ear affecting about 80 % of children by the age of 4 years. While OME usually resolves spontaneously, it can affect speech, behaviour and development. Children with persistent hearing loss associated with OME are usually offered hearing aids or insertion of ventilation tubes through the tympanic membrane. Oral steroids may be a safe and effective treatment for OME, which could be delivered in primary care. Treatment with oral steroids has the potential to benefit large numbers of children and reduce the burden of care on them and on health services. However, previous trials have either been too small with too short a follow-up period, or of too poor quality to give a definite answer. The aim of the Oral Steroids for the Resolution of Otitis Media with Effusion in Children (OSTRICH) trial is to determine if a short course of oral steroids improves the hearing of children with OME in the short and longer term. METHODS/DESIGN: A total of 380 participants (children of 2 to 8 years of age) are recruited from Hospital Ear, Nose and Throat departments in Wales and England. A trained clinician seeks informed consent from parents of children with symptoms for at least 3 months that are attributable to OME and with confirmed bilateral hearing loss at study entry. Participants are randomised to a course of oral steroid or a matched placebo for 1 week. Outcomes include audiometry, tympanometry and otoscopy assessments; symptoms; adverse effects; functional health status; quality of life; resource use; and cost effectiveness. Participants are followed up at 5 weeks, and at 6 and 12 months after the day of randomisation. The primary outcome is audiometry-confirmed satisfactory hearing at 5 weeks. DISCUSSION: An important evidence gap exists regarding the clinical and cost effectiveness of short courses of oral steroid treatment for OME. Identifying an effective, safe, nonsurgical intervention for OME in children for use in primary care would be of great benefit to children, their families and the NHS. ISRCTN: ISRCTN49798431 (Registered 7 December 2012).

An open label study to evaluate the safety and efficacy of intratympanic golimumab therapy in patients with autoimmune inner ear disease.

OBJECTIVE: To evaluate the safety and efficacy of intratympanically injected golimumab (GLM), a TNF-α inhibitor, as a steroid-sparing agent for patients with steroid-dependent autoimmune inner ear disease (AIED). STUDY DESIGN: Open label. SETTING: Tertiary referral center. PATIENTS: Ten patients with steroid-dependent AIED were enrolled in Stage 2. The average patient age at enrollment was 59, with an average of 12.5 years from the start of bilateral hearing loss symptoms. The average dose of daily prednisone at the start of injections was 18 mg. INTERVENTION: Intratympanic injection of GLM. MAIN OUTCOME MEASURE: Hearing loss progression (treatment failure) was defined as either an increase in pure-tone thresholds by frequency or a decrease in word recognition score. RESULTS: There were no serious adverse events. Five of seven per-protocol subjects experienced stable pure-tone thresholds in the injected ear, whereas 4 had stable word recognition scores. Two subjects experienced an improvement in word recognition scores. The results support the hypothesis that GLM may be a promising treatment. CONCLUSIONS: The TNF-α inhibitor GLM stabilized hearing in 3 of 7 per-protocol subjects with AIED and allowed a complete tapering off of prednisone in those 7 subjects. Studies with larger samples sizes are warranted.

Pathologic erythrocyte deformability in patients with sudden sensorineural hearing loss.

OBJECTIVE: To evaluate if viscoelastic properties of blood influence suffering sudden sensorineural hearing loss and the capacity to respond after a specific therapy. PATIENTS AND METHODS: A longitudinal prospective study included 85 ears bearing sudden deafness. In them, the mean hearing loss compared to the healthy ear and the recovery ratio were measured at the onset and 6 months after a treatment with corticoids and piracetam. In addition, tinnitus or vestibular symptoms, whole blood filterability (WBF) and erythrocyte deformability -by means of the erythrocyte rigidity index (ERI)- were determined and noted at the beginning and the end of the study. RESULTS: Mean hearing loss was 30.3±19.7% at the onset, and 25.8±39% at the end. Forty-one ears showed a recovery of more than 75%. In these (48% of the entire study group), an increase in WBF and a decrease in ERI were observed (P<.001). Ears without tinnitus or vestibular crisis recovered more hearing at 6 months and showed a significant improvement in WBF and ERI, not detected among patients with these clinical findings. There were good correlations between mean hearing loss at onset and WBF, and between recovery and ERI at 6 months, but without statistical significance. Patients with arterial hypertension, cardiopathy and hypercholesterolemia were the most frequently detected, while hypertension and hyperuricaemia showed a better hearing recovery ratio. CONCLUSIONS: The blood viscosity parameters WBF and ERI offer useful information about the risk of suffering sudden deafness and the capacity to recover hearing with reactive therapies.

Autoimmune recurrent facial palsy and bilateral sudden sensorineural hearing loss following Ramsay Hunt-like syndrome.

OBJECTIVE: To describe the first published case of recurrent facial nerve palsy associated with bilateral sudden sensorineural hearing loss of autoimmune origin. CASE REPORT: A 33-year-old male presented with acute facial palsy on the left following a vesicular herpetic eruption in the external ear canal on the same side. Serologic measurements demonstrated an elevation of IgM antibodies against herpes simplex virus but not for varicella-zoster virus, confirming a Ramsay Hunt-like syndrome due to herpes simplex virus. The following four months, the patient exhibited other three episodes of facial palsy, well responded to steroid treatment. During the clinical course, a sudden sensorineural hearing loss was also diagnosed, initially on the left side and then on both sides. The autoimmune markers such as the antinuclear antibody and the anti-gangliosides antibodies (anti-GM1, anti-GQ1b) were found positive. Despite steroid treatment, hearing did not show any improvement, remaining moderate on the right and severe on the left. CONCLUSION: Recurrent facial nerve palsy and bilateral sudden sensorineural hearing loss could be the expression of autoimmune disturbances. The initial triggered factor could be the herpes simplex virus infection, such as a Ramsay Hunt-like syndrome.

Sudden sensorineural hearing loss after varicose vein surgery under general anesthesia. Case report.

Sudden sensorineural hearing loss following non-otologic surgery, under general anesthesia, is an extremely rare complication. Furthermore, postoperative deafness has more commonly been associated with cardiac surgery. This acute dysfunction of the inner ear consists a controversial subject in terms of etiopathogenic mechanisms and treatment modalities. We present the case of a 60-year-old male patient who underwent varicose vein surgery under general anesthesia and presented immediately after the operation bilateral and profound impairment of his hearing acuity. A high index of suspicion is required to early diagnosis of this complication, although prompt treatment does not guarantee a good outcome.

Sudden bilateral sensorineural hearing loss following polysubstance narcotic overdose.

BACKGROUND: Auditory disorders associated with substance abuse are rare. Hearing loss secondary to heroin and hydrocodone abuse has been described variously as not always responsive to steroid management, as not always reversible, and in some cases, as nonresponsive profound sensorineural hearing loss requiring cochlear implantation. We present a case of a teenager with sudden-onset moderate to severe bilateral sensorineural hearing loss after documented polysubstance "binging." The hearing loss improved substantially after high-dose steroid and vasoactive therapy. PURPOSE: The purpose of this report is to describe the hearing disorder of a patient who had awakened with a bilateral severe hearing loss following a night of recreational drug abuse. RESEARCH DESIGN: Case report and review of the literature. DATA COLLECTION AND ANALYSIS: The subject of this report is an 18-yr-old patient with a history of substance abuse. Data collected were magnetic resonance /computed tomography brain imaging; metabolic, infectious disease, and autoimmune evaluation; and extensive audiologic evaluation, including pure-tone and speech audiometry, immittance measures, distortion-product otoacoustic emissions, and auditory brainstem response testing. Serial audiograms were collected for 10 mo following the onset of symptoms. RESULTS: Two days of polysubstance abuse (heroin, benzodiazepine, alcohol, and crack [smoked cocaine]) resulted in moderately severe sensorineural hearing loss bilaterally. The loss responded to a 1 mo course of high-dose prednisone and a 10 mo course of pentoxifylline. Hearing sensitivity subsequently improved, leaving only residual high-frequency sensorineural hearing loss. CONCLUSIONS: This case report highlights the importance of "recreational" drug abuse in the evaluation of sudden hearing loss. Potential etiologies include altered pharmacokinetics, vascular spasm/ischemia, encephalopathy, acute intralabyrinthine hemorrhage, and genetic polymorphisms of drug-metabolizing enzymes.

Bilateral progressive hearing loss and vestibular dysfunction with inner ear antibodies.

Autoimmune inner ear disease (AIED) is a clinical syndrome of uncertain etiology. We present the neuro-otological findings of 2 cases of bilateral hearing loss, dizziness and the antibody profiles of the inner ears. Case 1 had bilateral progressive hearing loss, vestibular dysfunction and abnormal eye movement as the disease progressed. She had inner ear antibodies against 42 and 58kDa protein antigency on Western blot immune assay, and responded to glycocorticosteroid but not to immunosuppressant treatment. Intratympanic steroid injection temporally eliminated her symptoms. However, she developed idiopathic Cushing's syndrome and underwent labyrinthectomy. Case 2 became deaf as a teenager and experienced dizziness 10 years after becoming deaf. He reacted strongly to 68kDa protein and was a good responder to immunosuppressant with steroid. As we still lack a definitive diagnostic test for AIED, careful observation of the clinical course is critical for differential diagnosis regarding the bilateral progressive hearing loss.

Immune thrombocytopenic purpura-related hemotympanum presenting with hearing loss.

A 5-year-old boy was admitted to our center with a major complaint of bilateral hearing loss for 2 days. He was diagnosed with acute immune thrombocytopenic purpura 3 months before the admission and treated with high-dose methylprednisolone 2 months ago. Physical examination revealed wet purpura in the oral mucosa, serous nasal discharge, multiple petechiae and ecchymosis of the lower lip. Otomicroscopic ear examination revealed the presence of bilateral hemotympanum. The patient denied head trauma, ear pain, fever, hypertension and medications, including salicylates. The patient received high-dose intravenous methylprednisolone because of low platelet count and wet purpura for 7 days and oral prophylactic amoxicillin-clavulanate for 14 days. The onset of the response to corticosteroids was rapid, and significant hematologic improvement was observed within a few days. The 2-week follow-up examination revealed intact tympanic membranes with normal color and mobility, and the patient restored normal hearing. In this patient, hemotympanum developed rapidly, and no predisposing cause other than immune thrombocytopenic purpura was found. However, presence of a serous nasal discharge may be a sign of viral upper respiratory tract infection. Therefore, it can be speculated that sneezing or coughing might have caused bilateral hemotympanum by increasing the middle ear pressure abruptly. We would like to emphasize that bleeding may occur in unusual sites and, unlike in healthy people, may cause bizarre symptoms in patients with bleeding diathesis. Hemotympanum can be considered among the indications to start treatment in patients with acute immune thrombocytopenic purpura.

Neurolymphomatosis mimicking chemotherapy-induced ototoxicity.

OBJECTIVE: To present an unusual cause of bilateral sensorineural hearing loss mimicking chemotherapy-induced ototoxicity. PATIENT: A 76-year-old woman with bilateral sudden sensorineural hearing loss after chemotherapy for non-Hodgkin lymphoma was referred for cochlear implantation. INTERVENTIONS: Prednisone and acyclovir were administered empirically during the course of the diagnostic evaluation. MAIN OUTCOME MEASURES: Diagnostic tests were obtained, including audiometry, positron emission tomography/computed tomographic imaging, and magnetic resonance imaging scan of the brain with gadolinium. RESULTS: Audiometry revealed bilateral profound sensorineural hearing loss without improvement after steroid and acyclovir treatment. Postchemotherapy positron emission tomography/computed tomographic imaging obtained 1 week after onset of hearing loss suggested the lymphoma to be in remission. However, magnetic resonance imaging obtained a few weeks later, concurrent with progression of symptoms to include facial palsy and numbness, demonstrated enhancement and thickening of multiple cranial nerves, consistent with neurolymphomatosis (malignant lymphocytic infiltration of nerves). CONCLUSION: Particular vigilance must be given to the potential for malignant invasion of the peripheral nervous system in the context of new-onset sensorineural hearing loss after chemotherapy. Such a neurologic deficit should not be assumed to be chemotherapy induced even if the hearing loss is bilaterally symmetric and the only neurologic abnormality.

[Loss of sight and hearing and cognitive dysfunction - Susac's syndrome?]. / Synstab, hørenedsaettelse og kognitiv dysfunktion - Susacs syndrom?

Cognitive dysfunction, unexplained deafness or retinal branch artery occlusions in otherwise healthy persons are challenging conditions, in particular when occurring in young adults. Susac's syndrome is characterized by the clinical triad of encephalopathy, hearing loss and retinal branch arterial occlusions affecting mainly young women. Appropriate diagnosis and management requires multidisciplinary management of the disease.

Whipple disease: unusual presentation of a protean and sometimes confusing disease.

OBJECTIVES: To describe an unusual case of Whipple disease (WD) with confusing clinical features at onset and to discuss the diagnostic challenges for the clinician. METHODS: Description of a new case of this rare disease and thorough discussion of the atypical clinical manifestations at onset. A literature review, concerning the unusual onset, by means of a MEDLINE search from 1966 to 2007 was done. RESULTS: A 39-year-old man with sudden bilateral blurred vision due to retinal vasculitis and concomitant rapidly evolving symmetrical neurosensory bilateral hearing loss as initial features of WD is described. Due to the clinical manifestations resembling systemic vasculitis, high-dose corticosteroid and pulse cyclophosphamide therapy were started with subsequent appearance of gastrointestinal symptoms (diarrhea and weight loss) and spiking fever, suggesting superimposed infection. After a complete evaluation, including gastroscopy, extensive duodenal-jejunal mucosal involvement was seen, while diffuse infiltration of the duodenal lamina propria with periodic acid-Schiff-positive foamy macrophages was observed on the histological sample. The diagnosis was confirmed by reverse transcriptase-polymerase chain reaction for the DNA of Tropheryma whippelii. To our knowledge, no previous similar clinical onset of WD has been described. CONCLUSIONS: To avoid misdiagnosis and therapeutic mistakes, clinicians should be aware of unusual presentations of WD. Because this etiological agent is a difficult to isolate bacterium, diagnosis may be especially problematic in cases without intestinal involvement at onset.