Bilateral Sudden Hearing Loss Research Articles

Role of DFNA5 in hearing loss and cancer - a comment on Rakusic et al.

Role of DFNA5 in hearing loss and cancer – a comment on Rakusic et al Lieselot Croes,1,2 Ken Op de Beeck,1,2 Guy Van Camp1 1Center of Medical Genetics (CMG), Department of Biomedical Sciences, University of Antwerp, Antwerp, Belgium; 2Center for Oncological Research (CORE), Department of Medicine, University of Antwerp, Antwerp, BelgiumWe would like to comment on the paper published by Rakusic et al about sudden bilateral hearing loss in gastric cancer as the only symptom of disease.1 The authors state that “Inactivated DFNA5, otherwise described in hereditary bilateral deafness, perhaps favors the development of deafness in patients with gastric cancer”.1 We believe this conclusion is erroneous. Although DFNA5 has been implicated in both hearing loss and cancer, the underlying molecular mechanisms are different and completely opposite (Figure 1). View original paper by Rakusic et al

Ototoxicity following Vinblastine chemotherapy in a patient of Hodgkin′s Lymphoma

Sudden hearing loss is a well-known complication of certain chemotherapeutic agents. However, vinblastine has seldom been implicated causing ototoxicity. We report a case of sudden bilateral hearing loss in a 36-year-old male patient of Mixed cellularity Hodgkin's lymphoma following standard adriamycin, bleomycin, vinblastine, and dacarbazine chemotherapy.

Contrast perilymphatic MRI findings of inner ear in sudden hearing loss with vertigo

Objective To explore the contrast perilymphatic MRI characteristics of inner ears with sudden hearing loss with vertigo. Methods Forty three patients with sudden hearing loss with vertigo and 35 patients with unilateral tinnitus diagnosed by the department of Otolaryngology-Head and Neck Surgery were retrospectively included. Forty eight ears (38 ears with unilateral sudden hearing loss and 10 ears in 5 cases with bilateral sudden hearing loss) were regarded as sudden hearing loss group, 35 asymptomatic ears (the opposite ears of the unilateral tinnitus ears) as control group. Thirty eight opposite ears of the 38 unilateral sudden hearing loss ears and 35 ears of the control group showed normal in the pure tone audiometry exam. One hundred and twenty one ears (48 hearing loss ears, 38 opposite ears of the 38 unilateral sudden hearing loss ears and the 35 control group's ears) underwent contrast perilymphatic MR exams, 0.4 to 0.5 ml gadolinium hydrate diluted with saline was injected through the tympanic membrane, twenty four hours later, three-dimensional fluid attenuated inversion recovery (3D-FLAIR) sequence was performed using a 3.0 T MRI scanner. After the contrast perilymphatic MR exam, success in the exam was judged through the raw image. The signal intensity ratio (SIR) of cochlea basal turn and homolateral brain stem was quantitatively calculated. The SIR difference of 48 ears with sudden hearing loss and 35 asymptomatic ears in control group was analyzed by t test. The SIR difference of the 38 unilateral sudden hearing loss ears and the contralateral asymptomatic ears was also analyzed. The presence of endolymphatic hydrops was judged. Results The contrast perilymphatic MRI of 121 inner ears with intratympanic gadolinium injection were all successful. Endolymphatic hydrops were observed in 12 sudden hearing loss ears with the occurrence rate of 25%(12/48). The SIR of basal turn and homolateral brain stem of the sudden hearing loss ears (2.062±0.907) were lower than the SIR of the control groups' asymptomatic ears (2.703± 0.640) with significant difference between them (t=3.619,P=0.001), the SIR of basal turn and homolateral brain stem of the unilateral sudden hearing loss ears (1.941±0.860) were also lower than the SIR of the contralateral asymptomatic ears (2.411±0.670) with statistical difference between them (t =3.270,P=0.002). Conclusions Endolymphatic hydrops were observed in sudden hearing loss with vertigo. The SIR of the cochlea in sudden hearing loss ears were lower than that of the asymptomatic ears, indicating the abnormal permeability of the round window membrane. Key words: Hearing loss; Sudden; Magnetic resonance imaging

Sudden bilateral hearing loss after spinal anesthesia for elective hemorrhoidectomy surgery

Sudden bilateral hearing loss after spinal anesthesia for elective hemorrhoidectomy surgery

Breast Cancer Presenting as Sudden Bilateral Hearing Loss: Case Report

Sudden sensorineural hearing loss is defined as a rapid onset hearing impairment occurring over a 72-h period, equal or greater than 30 dB in three consecutive frequencies. The annual incidence is 5-20 cases per 100,000, most being unilateral. Simultaneous bilateral involvement is rare, occurring in 1-2% of all cases. This is the first report of bilateral involvement as the presenting symptom of breast cancer diagnosed 1 year later. The authors present a case of a female patient, 63-year-old, who appeared in the emergency department with sudden bilateral deafness as the only symptom. The examination showed no further alterations. Additional studies were performed, and the patient was admitted in the hospital for monitoring, therapy and etiologic investigation. After investigation, we concluded this was a rare case of severe bilateral sensorineural hearing loss associated with occult carcinoma of breast, diagnosed later. This is of relevance not only to otolaryngologists, but also to clinicians who may deal with similar complaints, because only a third will have an underlying etiology that is diagnosed along the way. Sudden hearing loss is an ear emergence, in which fast evaluation by a specialist or someone who may recognize the complaints significantly improves the prognosis for hearing recovery. J Med Cases. 2014;5(4):220-223 doi: http://dx.doi.org/ 10.14740 / jmc1688w

Plasmodium falciparum and Salmonella Typhi Co-Infection: A Case Report

Malaria and salmonella infections are endemic especially in developing countries, however malaria and salmonella co-infection is a rare entity with high mortality. The basic mechanism in developing salmonella co-infection is the impaired mobilization of granulocytes through heme and heme oxygenase which are released from haemoglobin due to the breakdown of erythrocytes during malaria infection. Thus, a malaria infected person becomes more susceptible to develop infection with Salmonella spp. In this report a case with Plasmodium falciparum and Salmonella Typhi co-infection was presented. A 23-year-old male patient was admitted to hospital with the complaints of diarrhea, nausea, vomiting, abdominal pain, fatigue and fever. Laboratory findings yielded decreased number of platelets and increased ALT, AST and CRP levels. Since he had a history of working in Pakistan, malaria infection was considered in differential diagnosis, and the diagnosis was confirmed by the detection of P.falciparum trophozoites in the thick and thin blood smears. As he came from a region with chloroquine-resistant Plasmodium, quinine (3 x 650 mg) and doxycycline (2 x 100 mg/day) were started for the treatment. No erythrocytes, parasite eggs or fungal elements were seen at the stool microscopy of the patient who had diarrhoea during admission. No pathogenic microorganism growth was detected in his stool culture. The patient's blood cultures were also taken in febrile periods starting from the time of his hospitalization. A bacterial growth was observed in his blood cultures, and the isolate was identified as S. Typhi. Thus, the patient was diagnosed with P.falciparum and Salmonella Typhi coinfection. Ceftriaxone (1 x 2 g/day, 14 days) was added to the therapy according to the results of antibiotic susceptibility test. With the combined therapy (quinine, doxycycline, ceftriaxone) the fever was taken under control, his general condition improved and laboratory findings turned to normal values. However, on the fifth day of his anti-malaria therapy sudden bilateral hearing loss developed due to quinine use. Thus, the treatment was replaced with an artemisinin-based (arthemeter/lumefantrine) combination therapy. No adverse effects were detected due to artemisinin-based therapy, and the patient completely recovered. In conclusion, if a patient is diagnosed with malaria, he/she should be closely monitored in terms of having co-infections and appropriate diagnostic methods including blood cultures taken in febrile episodes should be performed.

A Case of Relapsing Polychondritis with Sequentially Bilateral Sudden Sensory Neural Hearing Loss

Relapsing polychondritis is a relatively rare systemic disease of unknown etiology. The disease is considered as an autoimmune disorder and characterized by episodic recurrent inflamma tion of cartilage and connective tissue, most often of the ears, nose, eyes, joints, respiratory tract, cardiovascular system and audiovestibular system. Treatment plans involve suppression of the immune system with corticosteroid. However, steroid therapy is associated with a number of adverse effects, and non-steroidal anti-inflammatory agents and immunosuppressive agents are helpful in treating the disease. Authors have recently experienced a case of relapsing polychondritis in a 24-year-old man who had both auricular chondritis and bilateral sudden hearing loss with vertigo. The patient showed a significant hearing improvement with the treatment using steroid and immunosuppressive agents. We report this case with a review of the literature.

Breast Cancer Presenting as Sudden Bilateral Hearing Loss: Case Report

Sudden sensorineural hearing loss is defined as a rapid onset hearing impairment occurring over a 72-h period, equal or greater than 30 dB in three consecutive frequencies. The annual incidence is 5-20 cases per 100,000, most being unilateral. Simultaneous bilateral involvement is rare, occurring in 1-2% of all cases. This is the first report of bilateral involvement as the presenting symptom of breast cancer diagnosed 1 year later. The authors present a case of a female patient, 63-year-old, who appeared in the emergency department with sudden bilateral deafness as the only symptom. The examination showed no further alterations. Additional studies were performed, and the patient was admitted in the hospital for monitoring, therapy and etiologic investigation. After investigation, we concluded this was a rare case of severe bilateral sensorineural hearing loss associated with occult carcinoma of breast, diagnosed later. This is of relevance not only to otolaryngologists, but also to clinicians who may deal with similar complaints, because only a third will have an underlying etiology that is diagnosed along the way. Sudden hearing loss is an ear emergence, in which fast evaluation by a specialist or someone who may recognize the complaints significantly improves the prognosis for hearing recovery. J Med Cases. 2014;5(4):220-223 doi: http://dx.doi.org/ 10.14740 / jmc1688w

Sudden hearing loss in a patient of Hodgkin's lymphoma following vinblastine chemotherapy: a rare case report and review of the literature

Ototoxicity is a well-known complication of certain chemotherapeutic agents. There have been few reports of ototoxicity following administration of vincristine. However, vinblastine has seldom been implicated causing ototoxicity. We report a case of sudden bilateral hearing loss in a 32-year-old male patient of classical Hodgkin’s lymphoma following standard adriamycin, bleomycin, vinblastine, dacarbazine chemotherapy.

Sudden Hearing Loss Associated With Tacrolimus After Pediatric Renal Transplant

Hearing impairment is a frequent problem that can easily influence the quality of life for the individual. It may affect one's social and academic life. Knowledge regarding hearing impairment after renal transplant is sparse. It has been suggested that renal transplant improves hearing function. Potential ototoxic side effects may be related to immunosuppression with calcineurin inhibitors. In pediatric renal transplanted patients, we do not have enough information about this subject. We report 2 cases that developed sudden hearing loss after a renal transplant that was associated with high serum levels of tacrolimus. Two renal transplanted children (a 15-year-old boy and a 17-year-old girl), in the fourth year of their follow-up after transplant, developed symptomatic bilateral sudden hearing loss. There was a marked hearing impairment for the higher frequencies between 4000 and 8000 Hz in pure-tone audiometry evaluation. Also, a decrease of speech understanding was found, but the patients were not conscious of this problem. Hearing loss in these patients was not associated with any known risk factors such as chronic renal disease, ototoxic drugs, or acoustic trauma. Sudden hearing loss occurred under high serum levels of tacrolimus, and after dosage correction of tacrolimus pure-tone audiometry ruled out hearing loss progression for each patient. Awareness of this potential complication of tacrolimus may be helpful for early recognition and treatment.

Bilateral Internal Acoustic Canal Mass

We reported a case of bilateral internal acoustic canal mass. A 42-year-old man patient was previously treated for colon cancer. After surgery during chemotherapy signs as severe vertigo and bilateral sudden hearing loss occurred. Temporal bone magnetic resonance imaging (MRI) had bilateral internal acoustic canal masses.

THU0324 Inner Ear Dysfunction as an Expression of Atherosclerosis in Primary Antiphospholipid Syndrome

BackgroundMicrovascular oclussion may occur as part of the manifestations of the antiphospholipid syndrome (APS). One of these manifestations is Sensorineural hearing loss (SHL).ObjectivesTo determine the prevalence of inner ear dysfunction...

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

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. 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. The patient underwent a cochlear implantation with a modest outcome. 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.

Vertebrobasilar Occlusion Presenting as Sudden Isolated Bilateral Sensorineural Hearing Loss: Case Report

Isolated bilateral deafness is a rare but possible symptom of vertebrobasilar ischemia, primarily due to occlusion of the anterior inferior cerebellar arteries or their branch, the internal auditory artery. We reported on uncommon case of sudden bilateral sensorineural hearing loss without typical neurological symptoms resulting from vertebrobasilar ischemia. We performed the available examinations, including otoscopy, laboratory tests, and pure tone audiogram, however we were not able to identify the cause of bilateral sensorineural hearing loss. Brain magnetic resonance image showed the cerebellar infarction of the posterior inferior cerebellar artery territory. Brain magnetic resonance angiography showed bilateral vertebral and basilar artery occlusion. We suggest vertebrobasilar ischemia as a cause of sudden isolated deafness.

Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype

Sudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease). The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date specific diagnostic tests are not available. We report a case of bilateral sudden hearing loss, tinnitus, intense rotatory vertigo, and nausea in a female patient in which the clinical manifestations, in addition to raised levels of circulating immune complexes, antithyroglobulin antibodies, and the presence of the HLA A1-B8-DR3 haplotype, allowed us to hypothesize an autoimmune inner ear disease. Cyclosporine-A immunosuppressive treatment in addition to steroids helped in hearing recovery that occurred progressively with normalization of the hearing function after a five-month treatment. Cyclosporine-A could be proposed as a therapeutic option in case of autoimmune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.

A Case of Bilateral Sudden Hearing Loss and Tinnitus after Salicylate Intoxication

Salicylate, the active ingredient of aspirin can cause sensorineural hearing loss and tinnitus when plasma concentrations reach a critical level. The ototoxic mechanisms of salicylate remain unclear but hearing and tinnitus usually recovers a few days after intoxication. There have been few reports of salicylate-induced ototoxicity in Korea, and the majority is caused by a low dose of aspirin. Herein, we report a case of sudden hearing loss and tinnitus after acute salicylate intoxication and review recent updates on salicylate ototoxicity.

Bilateral sudden sensorineural hearing loss as an initial presentation of myelodysplastic syndrome

This study reports an unusual case in which myelodysplastic syndrome presented bilateral sudden sensorineural hearing loss as the first symptom of the disease. The aural symptoms and signs such as tinnitus, dizziness, and hearing impairment of a hematologic disease are common. However, sudden hearing loss as the first manifestation of a hematologic disease is extremely rare. A 76-year-old woman presented with bilateral sudden hearing loss. The patient was found to have myelodysplastic syndrome during a workup for her hearing loss. Unfortunately, the patient's hearing loss did not improve after the medical treatment.

Sudden bilateral sensorineural deafness in a 5 years old girl with blue sclerae, keratoconus, hypermobility of the joints and hyperelasticity of the skin with a mutation in the ZNF469 gene encoding for Brittle-Cornea-Syndrome

Aims: The Brittle-Cornea-Syndrome is a rare autosomal recessive hereditary generalised connective tissue disease with typical symptoms like high corneal fragility, keratoconus, blue sclerae, hypermobility of the joints and hyperelasticity of the skin. Progressive hearing disorders up to deafness were mentioned as well. Hitherto two mutations are identified concerning the ZNF469 gene and the PRDM5 gene. Methods: A 5 years old girl was presented with a sudden complete sensorineural bilateral hearing loss. Through a BERA-investigation right-sided no responses were found up to 103 dB, sinistral only a V-wave on 90 dB was found and through an electrocochleography even no responses were found bilaterally. Bilateral arched demineralisations around the cochlea were found in the petrosal thin-layer CT. The cerebral MR-imaging was normal. No improvement was achieved under a therapy with steroids for 10 days. After occurrence of a headache, subfebrile temperatures and vomiting a lumbal puncture was completed. The liquor-analysis was normal with normal Reiber-Diagram and no hints of a lyme-disease or a viral infection were found. Results: Based on the blue sclerae, the keratoconus, the hypermobility of the joints and the hyperelasticity of the skin we suggested a Brittle-Cornea-Syndrome. This suspected diagnosis could be confirmed genetically. A so far not found new homozygous frameshift mutation in exon2 in the ZNF469 gene could be identified in our patient. Meanwhile a bilateral cochlear implant operation was done. Summary: The confirmation of our suspected diagnosis of a Brittle-Cornea-Syndrome with typical blue sclerae, a keratoconus, hypermobility of the joints and hyperelasticity of the skin succeded with the help of a genetic analysis. Progressive hearing disorders up to deafness were mentioned before as well, though a sudden complete bilateral sensorineural deafness has not been described before. Infectious, space-consuming lesions or other causes of the sudden bilateral sensorineural deafness could be excluded, in order that a relation to the identified genetic disease could be seen.

Bilateral intracranial vertebral artery dissection presenting as sudden bilateral hearing loss

Although sudden unilateral hearing loss is relatively common, espite clinical and radiological investigations, most cases remain diopathic. The central disorders causing sudden hearing loss are escribed in cases with vertebrobasilar insufficiency; multiple scleosis; sarcoidosis; meningitis; forms of vasculitis, such as Behc et isease; human immunodeficiency virus or cytomegalovirus infecion; and Susac syndrome [1]. Sudden hearing loss is usually nilateral; bilateral hearing loss is rare. Among various etioloies affecting auditory pathways, the incidence of bilateral hearing oss is estimated to be 0.5–1.2% in patients with vertebrobasilar schemia or occlusive disorders [1,2], and hearing loss resulting rom vertebral artery dissection is extremely rare. We report a case f bilateral intracranial vertebral artery dissection presenting as udden bilateral hearing loss with no abnormality in brainstem and erebellar structures on magnetic resonance imaging (MRI).

Clinical Practice Guideline

Sudden hearing loss (SHL) is a frightening symptom that often prompts an urgent or emergent visit to a physician. This guideline provides evidence-based recommendations for the diagnosis, management, and follow-up of patients who present with SHL. The guideline primarily focuses on sudden sensorineural hearing loss (SSNHL) in adult patients (aged 18 and older). Prompt recognition and management of SSNHL may improve hearing recovery and patient quality of life (QOL). Sudden sensorineural hearing loss affects 5 to 20 per 100,000 population, with about 4000 new cases per year in the United States. This guideline is intended for all clinicians who diagnose or manage adult patients who present with SHL. The purpose of this guideline is to provide clinicians with evidence-based recommendations in evaluating patients with SHL, with particular emphasis on managing SSNHL. The panel recognized that patients enter the health care system with SHL as a nonspecific, primary complaint. Therefore, the initial recommendations of the guideline deal with efficiently distinguishing SSNHL from other causes of SHL at the time of presentation. By focusing on opportunities for quality improvement, the guideline should improve diagnostic accuracy, facilitate prompt intervention, decrease variations in management, reduce unnecessary tests and imaging procedures, and improve hearing and rehabilitative outcomes for affected patients. The panel made strong recommendations that clinicians should (1) distinguish sensorineural hearing loss from conductive hearing loss in a patient presenting with SHL; (2) educate patients with idiopathic sudden sensorineural hearing loss (ISSNHL) about the natural history of the condition, the benefits and risks of medical interventions, and the limitations of existing evidence regarding efficacy; and (3) counsel patients with incomplete recovery of hearing about the possible benefits of amplification and hearing-assistive technology and other supportive measures. The panel made recommendations that clinicians should (1) assess patients with presumptive SSNHL for bilateral SHL, recurrent episodes of SHL, or focal neurologic findings; (2) diagnose presumptive ISSNHL if audiometry confirms a 30-dB hearing loss at 3 consecutive frequencies and an underlying condition cannot be identified by history and physical examination; (3) evaluate patients with ISSNHL for retrocochlear pathology by obtaining magnetic resonance imaging, auditory brainstem response, or audiometric follow-up; (4) offer intratympanic steroid perfusion when patients have incomplete recovery from ISSNHL after failure of initial management; and (5) obtain follow-up audiometric evaluation within 6 months of diagnosis for patients with ISSNHL. The panel offered as options that clinicians may offer (1) corticosteroids as initial therapy to patients with ISSNHL and (2) hyperbaric oxygen therapy within 3 months of diagnosis of ISSNHL. The panel made a recommendation against clinicians routinely prescribing antivirals, thrombolytics, vasodilators, vasoactive substances, or antioxidants to patients with ISSNHL. The panel made strong recommendations against clinicians (1) ordering computerized tomography of the head/brain in the initial evaluation of a patient with presumptive SSNHL and (2) obtaining routine laboratory tests in patients with ISSNHL.