Sudden Hearing Impairment Research Articles

Bilateral Sudden Irreversible Hearing Loss in a Case of Chronic Myeloid Leukaemia: A Case Report.

Chronic myeloid leukemia is a type of blood cancer that affects the bone marrow and results in an overproduction of immature WBCs. The genetic mutation that causes CML is the BCR-ABL fusion gene. Adolescents are rarely affected. The case study aims to discuss a rare case of chronic myeloid leukemia causing bilateral hearing impairment, tinnitus, and vertigo. A 30-year-old female presented to the hospital in November, 2021, with sudden hearing impairment and other symptoms, leading to a CML diagnosis. Blood tests revealed hyperleukocytosis with marked neutrophilia, mild basophilia, and eosinophilia, and a BCR-ABL quantitation of 85%. Bone marrow aspiration showed granulocytic hyperplasia, mild left-shifted maturation, and less than 1% blasts. The patient was started with options including tyrosine kinase inhibitors (TKIs) such as Imatinib, which target the BCR-ABL fusion gene, reducing the number of leukemia cells and improving her white blood cell count. However, her deafness persisted, and she became dependent on hearing aids. CML presenting with hearing loss is rare, believed to be related to the infiltration of leukemic cells in the inner ear or microvascular complications. Treatment with tyrosine kinase inhibitors such as Imatinib can improve hematologic parameters, but the effect on hearing loss is uncertaint.

Chronic Myeloid Leukemia (CML) with Permanent Hearing Loss

Objective: The present case study aims to discuss a rare case of chronic myeloid leukemia causing bilateral hearing impairment, tinnitus, and vertigo. Introduction: Chronic Myeloid Leukemia (CML) or chronic granulocytic leukemia is a type of blood cancer that affects the bone marrow. It results in an overproduction of immature White Blood Cells (WBCs) that typically happens throughout or after middle age, and adolescents are rarely affected. The genetic mutation that causes CML is known as the BCR-ABL fusion gene. Case Presentation: In this case report, a 30-year-old female presented to the hospital of Jazan, Saudi Arabia, with sudden hearing impairment and other symptoms, leading to a CML diagnosis. The patient had no prior hearing impairment background, and the otoscopic scanning was normal. The Pure Tone Audiometry (PTA) showed profound sensorineural hearing loss on both sides. Blood tests revealed hyperleukocytosis with marked neutrophilia, mild basophilia, and eosinophilia and a BCR-ABL quantitation of 85%. Bone marrow aspiration showed granulocytic hyperplasia, mild left-shifted maturation, and less than 1% blasts. Results: The patient was started with options including Tyrosine Kinase Inhibitors (TKIs) such as Imatinib, which target the BCR-ABL fusion gene, reducing the number of leukemia cells and improving her white blood cell count. However, her deafness persisted, and she became dependent on hearing aids. Discussion: CML presenting with hearing loss is rare, with only a few reported cases in the literature. It is believed to be related to the infiltration of leukemic cells in the inner ear or the microvascular complications associated with the disease. Hearing loss can be reversible or permanent, depending on the severity and duration of the disease. Treatment of CML with tyrosine kinase inhibitors such as Imatinib can improve the hematologic parameters, but the effect on hearing loss is uncertain.

The moderating effect of personality traits on acute tinnitus sensation in idiopathic sudden sensorineural hearing loss.

People may experience tinnitus after sudden hearing impairment. The details of the relationship between tinnitus improvement and hearing recovery are still unclear. Personality traits may play a role in the modulation of tinnitus sensation. We investigated the moderating effect of personality traits on pretreatment and posttreatment tinnitus sensation in patients with idiopathic sudden sensorineural hearing loss (ISSHL). This prospective longitudinal study enrolled 33 patients diagnosed with unilateral ISSHL and acute tinnitus in 2018-2019 at one institute. Clinical data were collected before and after treatment, including results of pure-tone audiometry (PTA), the Clinical Tinnitus Questionnaire (CTQ), the Tinnitus Handicap Inventory (THI), tinnitus loudness and annoyance (Visual Analog Scale; VAS), the Hospital Anxiety and Depression Scale (HADS), and the Big Five Inventory (BFI)-44. Eighteen men and 15 women with an average age of 48.3 ± 15.8 years were enrolled. The pretreatment hearing threshold and THI score were 58.0 ± 27.4 dB HL and 42.9 ± 26.2 (range: 0-88), respectively; the posttreatment values were 39.8 ± 22.4 dB HL and 20.7 ± 22.5 (range: 0-64), respectively. There were significant differences between pretreatment and posttreatment hearing thresholds as well as THI, VAS, and HADS scores (p < 0.001) but not BFI-44 results. Neuroticism and openness had significant positive and negative correlations, respectively, with acute tinnitus stress before treatment (r = 0.561, p = 0.001; r = -0.359, p = 0.040). After 3 months of follow-up, all patients were analyzed separately by dividing them into recovery (n = 16) and nonrecovery groups (n = 14) according to their hearing improvement status by Siegel's criteria. Neuroticism showed a significant moderating effect on acute tinnitus sensation in the ISSHL recovery group (p < 0.001) but not in the nonrecovery group (p = 0.106). Neuroticism and openness may affect acute tinnitus stress in ISSHL patients. Practitioners should consider personality traits when managing ISSHL patients with bothersome tinnitus.

Односторонняя внезапная сенсоневральная тугоухость при COVID-19. Клинические наблюдения и обзор литературы

At the end of 2019, an outbreak of a new coronavirus infection began in China, Wuhan, which is the first pandemic of the 21st century. The International Committee on Taxonomy of Viruses has classified the causative agent of the new disease as SARS-CoV-2, and on February 11, 2020, the World Health Organization (WHO) announced the official name of the infection – COVID-19. According to statistics for August 2021, the number of people infected with SARS-CoV-2 in the world is 216 million, and the number of deaths is 4.5 million. COVID-19 can be asymptomatic or manifest with various clinical manifestations, including neurological and otorhinolaryngological. The article describes 3 clinical cases of unilateral sudden hearing impairment in patients with confirmed COVID-19. The diagnosis of unilateral sensorineural hearing loss was established using acumetry, tone threshold audiometry, acoustic impedance measurement, and recording of otoacoustic emissions. In most cases, COVID-19 is characterized by a certain clinical picture, however, with an asymptomatic and mild course, an atypical manifestation of the disease with the development of sudden sensorineural hearing loss is possible.

Cerebral Inflow and Outflow Discrepancies in Severe Sudden Sensorineural Hearing Loss.

The aim of this study is to evaluate whether cerebral inflow and outflow abnormalities, assessed by the means of a validated ultrasound model, could be associated with Sudden Sensorineural Hearing Loss (SSNHL). According to Clark, a total of 42 patients affected by severe SSNHL and 19 healthy volunteers matched by gender without any history of sudden hearing impairment have been included in this study. Patients and controls underwent EchocolorDoppler assessment of brain hemodynamics. All subjects affected by SSNHL were also assessed with Auditory Brainstem Responses (ABR) and Magnetic Resonance Imaging (MRI) in order to exclude retrocochlear pathology. The head inflow through the common carotid artery was practically equivalent between groups, but at the level of the carotid bifurcation, the external carotid artery showed a highly significant flow rate in SSNHL 5.4±2 vs 3.9±1.1 ml/s in controls (p=0.01). The brain inflow was similar between patients and controls, but interestingly the flow rate of the vertebral artery was significantly reduced in SSNHL 1.6±0.8 vs 2.8±0.9 ml/s (p=0.01). The brain outflow was found significantly restricted at the level of the jugular outlet 6.6±6 vs 9.9±6 ml/s (p=0.002); consequently, the collateral flow index was significantly increased in SSNHL (p=0.001). The present study shows a discrepant distribution of the brain inflow which seems to penalize the posterior segments of the Willis polygon in patients affected by severe SSNHL. In addition, our study confirms the presence of chronic cerebrospinal venous insufficiency in SSNHL with significant activation of venous collateral circulation.

Coping strategies and social environment of patients with sudden hearing loss

BackgroundHearing impairment has a significant physiological, psychological, and social impact on one’s life. The primary aim of this study was to explore which coping strategies are most frequently used by patients with a sudden hearing impairment within the period of just a few days after the first symptoms appear.Participants and procedureSixty-four patients were asked to complete the coping inventory COPE between the first and the fifth day following the diagnosis of hearing loss.ResultsThe start of hearing impairment acts as a trigger for the use of a problem-solving coping strategy. Social support from the family represents an important source of help for patients with sudden hearing loss. Humour as a coping strategy was found to be used as a means of easing the current unfavourable situation and concealing one´s emotions from others.ConclusionsActive coping and seeking social support were the most frequently used coping strategies.

Incidence of otolaryngological symptoms in patients with temporomandibular joint dysfunctions.

The functional disorders of the masticatory organ are the third stomatological disease to be considered a populational disease due to its chronicity and widespread prevalence. Otolaryngological symptoms are a less common group of dysfunction symptoms, including sudden hearing impairment or loss, ear plugging sensation and earache, sore and burning throat, difficulties in swallowing, tinnitus, and vertigo. The diagnostic and therapeutic problems encountered in patients with the functional disorders of the masticatory organ triggered our interest in conducting retrospective studies with the objective of assessing the incidence of otolaryngological symptoms in patients subjected to prosthetic treatment of the functional disorders of masticatory organ on the basis of the analysis of medical documentation containing data collected in medical interviews. Material and Methods. Retrospective study was conducted by analyzing the results of medical interviews of 1208 patients, who had reported for prosthetic treatment at the Functional Disorders Clinic of the Department of Dental Prosthetics of Jagiellonian University Medical College in Cracow between 2008 and March 14, 2014. Results. Otolaryngological symptoms were observed in 141 patients. The most common symptoms in the study group were earache and sudden hearing impairment; no cases of sudden hearing loss were experienced.

Sudden hearing impairment combined with diabetes mellitus or hyperlipidemia

The objective of this study was to delineate whether the presence of the risk factors of microvascular disorder (MD) contribute to the prognostic outcome in patients with idiopathic sudden deafness (ISD). Retrospective clinical record review was conducted with 106 patients of ISD. Pure tone hearing thresholds were compared among the groups with or without hyperglycemia and hypercholesterolemia. Significantly better hearing recoveries were observed in cases with hyperglycemia as compared with controls. There was a tendency that the magnitude of hearing recovery in hypercholesterolemia was greater than that of controls although there was no statistical difference. The presence of the risks in MD could be an indicator of better hearing prognosis of ISD if we choose appropriate treatments. Heterogeneity of the ISD makes the establishment of the relationship between the risk factors of MD and etiology of some cases of ISD. We may have to choose the treatments for ISD considering whether the patient has any risk factor of MD.

両側突発難聴で判明した白血病例

A 30-year old man was admitted to Shizuoka City Hospital because of bilateral sudden hearing impairment. He was pale and manifested hepato-splenomegaly. A blood examination revealed chronic myelocytic leukemia in blastic crisis with DIC. He died of multiple cerebral hemorrhages 4 days after his initial visit.

聴力障害を主訴とした腎癌小脳橋角部転移例

We experienced a rare case of metastatic cerebellopontine (CP) angle tumor, originating from a renal cell carcinoma with hearing impairment. A 61-year-old male was admitted to our hospital complaining of sudden hearing impairment of the left ear. He had been well for six years, after a diagnosis of left renal cell carcinoma two years previously, with lung metastasis and brain metastases in the frontal lobe. Brain tumors had been removed twice. Magnetic resonance imaging (MRI) of the head showed a new lesion at the CP angle with recurrence of the left metastatic tumors.Hearing impairment was achieved by the administration of corticosteroid. The patient showed loss of II -V waves on left ABR, and optokinetic nystagmus test depression on the counterclockwise. Hearing impairment and dizziness progressively worsened during the subsequent two year period, but he was managed as an out-patient until March in 1998.

ＩＦＮ療法中に発症した一側性高度難聴

A 43-year-old male was admitted to our clinic because of sudden hearing impairment in his left ear. The patient had received intravenous injection of interferon beta for the treatment of HCV hepatitis. On the second day of interferon administration, he complained of ear fullness and tinnitus in his left ear. Pure tone audiometry revealed up-sloping hearing impairment.An initial high-dose steroid therapy was successful for recovery of hearing loss. Interferon alpha was then administered for HCV hepatitis and hearing loss worsened 10 weeks after secondary interferon therapy. The same steroid regimen was unsuccessful and severe hearing loss developed. Hyperbaric oxygen therapy was then conducted and was the only effective treatment for this progression. One week after the recovery, left hearing impairment thrice occurred and no treatment was effective.Audiological side effects were reported in 40% of the patients who received interferon administration. The side effects were usually temporary and recovered soon after termination of interferon therapy. The present case revealed that hearing loss found during interferon therapy does not always show a fair prognosis. Further study on the audiological side effects during interferon therapy should be performed.

Physiologischer Ansatz zur Durchblutungsförderung bei der akuten Labyrinth-Ischämie*

One of the main factors of sudden hearing impairment, acute vestibular disturbance, and acute tinnitus is generally thought to be an acute labyrinthine ischemia of varying degrees. However, the scientific basis for this assumption has not yet been proven, and there is a great variety of treatment modalities. Recent circulation research and fundamental physiological considerations led to the development of a new concept of treatment of these diseases. In order to enhance the labyrinthine blood circulation, physiological and physical methods were tested in 42 patients, especially with regard to patient compliance and in addition to conventional therapy. The methods tested included active circulation training. Finnish bath observing certain precautions, sequentially increased temperature bathing, massage, and fango therapy of the neck. The compliance of the patients to the new concept was good, in some cases even enthusiastic, either to the "total body" methods or to the local neck treatment. The principle of active and/ or passive stimulation of blood circulation in acute labyrinthine ischemia has been well accepted by all patients. A study including functional results is in preparation.

Side effects of high-dose interferon therapy for chronic hepatitis C

Background/Aims: Various side effects have been reported in patients treated with alpha interferon, but their incidence and prognosis remain unknown. Methods: Nine hundred and eighty-seven patients with chronic active hepatitis C received 6 to 10 MU of alpha interferon per day for 2 weeks and 3 times per week for 22 weeks. Autoantibodies, thyroid function tests, and fasting plasma glucose concentrations were evaluated prior to alpha interferon therapy. Results: Of the 987 patients, 310 were required reduction in the dose of alpha interferon to 3 MU/day or cessation of alpha interferon therapy because of adverse reactions such as flu-liked symptoms, leukopenia, and thrombocytopenia. Of the remaining 677, five developed diabetes mellitus, 12 had hyperthyroidism, and six acquired hypothyroidism. Of the 18 with thyroid disorders, five demonstrated antimicrosomal antibodies before therapy. Forty-four patients revealed high or low concentrations of thyroid stimulating hormone at the end of alpha interferon therapy. Three patients developed interstitial pneumonia, one acquired systemic lupus erythematosus-like syndrome, two had autoimmune hepatitis, two developed rheumatoid arthritis, and one developed autoimmune thrombocytopenic purpura. No patients had a history of an autoimmune disorder. One patient experienced sudden hearing impairment and one had retinal detachment. Melena was seen in three patients; two of these cases were compatible with ischemic colitis. Symptoms of depression were seen in 23 patients, and one patient manifested memory loss. Conclusion: High-dose alpha interferon therapy induces various adverse effects. Most of the side effects cannot be predicted, but are reversible.

Probational Treatment of Sudden Deafness with Prostacyclin: A Pilot Study

Sudden idiopathic hearing loss has occasionally been supposed to be caused by a disturbed microcirculation in the inner ear of unknown origin. Little is known about the regulation of cochlear blood flow and the effectiveness of drugs in cochlear microcirculation. Because animal experiments gave evidence that prostacyclin (PGI2) might be one biochemical substratum of local regulators in the flow of blood in the stria vascularis, 11 patients with sudden idiopathic hearing loss were treated once for 6 h with prostacyclin (10 ng/kg body weight/min) in a first open clinical trial. In most cases prostacyclin increased hearing level (mean value: 7.4 dB/frequency/day) more than a standard therapy with pentoxifylline. The substitution of PGI2 could be another indication of a rheologic disorder--whether per se or within a larger context of inflammation-like interaction--in the inner ear of patients with sudden hearing impairment.

ムンプスによる両側聾の一症例

A case of bilateral total deafness after mumps infection is repotred. A 6-year-old boy was referred to us because of sudden hearing impairment in the course of mumps parotitis. Physical examination showed no abnormalities except bilateral parotid swellings. Neurological examination revealed no signs of meningitis or disepuilibrium. The audiogram showed marked bilateral hearing loses at allfrequencies. A diagnosis of sensorineural deafness following mumps was made. High dose of steroid and vasodilators was administered for ten days. However, he did not recover.

糖尿病に合併した両側同時発症突発性難聴

A rare case of simultaneous bilateral sudden deafness in a 58-year-old female with diabetes mellitus is presented.She visited Hokusetsu General Hospital with complaints of sudden bilateral hearing disturbances and right tinnitus. She had had poorly controlled diabetes mellitus three years. Several treatments, including medications for diabetes mellitus led to for very good recovery of hearing improvement in the right ear, but only slight improvement in the left ear.Our experience with this case and a review of the literature indicate that in sudden deafness secondary to diabetes mellitus there are various etiologic mechanisms, such as metabolic dysfunction of the stria vascularis, changes of endolymphatic osmotic pressure, sludging of blood cells, vasospasm and abnormalities of the vessels in the cochlea.Further clinical and experimental studies are needed to elucidate the pathophysiology of sudden hearing impairment in patients with diabetes mellitus.