Right-sided Hearing Loss Research Articles

Multifocal metachronous occurence of different hystologic sinonasal-type papilloma

Introduction: Sinonasal-type papilloma is a very rare tumor, most commonly connected with the sinonasal space, and very rarely with the middle ear. Primary tumors of the temporal bone are extremely rare and only 28 cases have been described in literature, with additional 29 cases of tumor spreading from the sinonasal tract to the temporal bone. Case presentation: We discuss the case of a 49-year-old woman who had a primary right-sided exophytic form of the sinonasal papilloma of the middle ear, which led to right-sided hearing loss, aural fullness, and otorrhea. During postoperative CT and MRI follow-up one year after surgery, a sinonasal oncocytic-type papilloma was discovered in the sphenoid sinus. To our knowledge, this is the first described case of histologically two different primary sinonasal-types of papilloma in a patient. Common presenting symptoms associated with sinonasal papilloma of the middle ear can be easily misdiagnosed with chronic otitis media or Eustachian tube dysfunction. Although primarily benign, sinonasal papillomas are locally aggressive and pose a risk of recurrence and malignant transformation. Therefore, surgery remains the treatment of choice with necessary long-term follow-up, to detect relapse or even a completely new tumor in that area.

ASYMETRIE W POZNANIU I POMIARACH INTELIGENCJI U DZIECI Z UBYTKIEM SŁUCHU W PRAWYM LUB LEWYM UCHU

IntroductionHemispheric asymmetry of the central nervous system affects various features of the brain involved in cognitive ability. Functional asymmetry, such as different hearing ability in the left or right ear, will also affect cognitive processes.Material and methodsThe aim of this study was to assess how intelligence measures and cognitive abilities in children and adolescents might have been affected by hearing deficits in the left or right ear. The study involved 208 children, 126 who were in an experimental group and 82 in a control group. In the experimental group, there were 26 children who were diagnosed with right-sided hearing loss, 34 with leftsided hearing loss, and 66 with bilateral hearing loss; all children in this group had used hearing devices since diagnosis. We assessed hearing unilaterally and bilaterally and looked for asymmetries in terms of intelligence measures and visual and spatial functioning.ResultsChildren with bilateral hearing impairment had lower intelligence compared to those without impairment. Children with unilateral hearing impairment had similar intelligence level compared to well hearing children. Children with left-sided hearing impairment had higher intelligence compared to those with right-sided hearing impairment and lower nonverbal intelligence compared to well-hearing children. Children with right-sided hearing impairment had lower verbal intelligence.ConclusionsHearing impairment has an impact on various measures of intelligence, as well as on the organisation and performance of cognitive processes.

Localised sinonasal amyloidosis presenting with unilateral hearing loss: a case report and review of the literature

Localised sinonasal amyloidosis is a rare occurrence with only twenty-nine documented cases in literature. This report follows the case of a 79-year-old gentleman with an atypical presentation of headaches and unilateral (right-sided) hearing loss. The patient initially underwent magnetic resonance imaging of his internal auditory meatus which was normal. Flexible nasoendoscopy was performed which identified a right middle meatus discharging possible polypoidal lesion. A computed tomography scan of his sinuses was performed which identified a large soft tissue lesion projecting into the upper nasal airway arising from the nasal recess, with the appearance suspicious for a polyp. Subsequently, the patient underwent functional endoscopic sinus surgery to manage right maxillary sinusitis and further examine the right-sided polypoidal mass lesion that was obstructing the maxillary antrum. Maxillary sinus biopsy revealed a diagnosis of sinonasal amyloidosis while tests for systemic amyloidosis were negative.

A Case of Patient with Bilateral Cochleovestibular Function Loss due to Infratentorial Superficial Siderosis

Superficial siderosis (SS) is a rare neurodegenerative condition characterized by hemosiderin deposition in the central nervous system, which sometimes leads to sensorineural hearing loss and vestibular dysfunction. This case report details the diagnosis and treatment of a 63-year-old female patient presenting with a yearlong history of postural instability and recent symptoms of sudden right-sided hearing loss and imbalance. Physical examination and imaging revealed bilateral deafness and infratentorial SS. Treatment, including medication, vestibular exercises, high-dose steroids, and hyperbaric oxygen therapy, led to a significant alleviation of dizziness but no improvement in hearing. This case emphasizes the need for clinicians to consider SS in patients presenting with sudden-onset hearing loss and dizziness, even without prior any medical history or trauma, to accurately identify the underlying cause.

Vestibular Dysfunction Secondary to Reactivation of the Neurotropic Virus VZV After COVID-19: A Case Study.

BACKGROUND The novel coronavirus COVID-19 infection, caused by the SARS-CoV-2 virus, was declared a global pandemic by the World Health Organization (WHO) in March 2020. As of April 14, 2021, the virus has impacted the lives of over 138 million lives globally, with hundreds of thousands more impacted each day. COVID-19 has resulted in a multitude of clinical manifestations such as respiratory pathology, cardiovascular complications, and neurological dysfunction, and recent evidence points to potential changes in vestibular function secondary to COVID-19 infection. CASE REPORT We present the case of a 67-year-old female patient presenting with vertigo, hearing loss, tinnitus, aural dullness, dizziness, and imbalance following a diagnosis of shingles after contracting COVID-19. Results of a comprehensive balance evaluation were suggestive of a right peripheral vestibulopathy, otolith dysfunction, and a right-sided sensorineural hearing loss. The patient's subjective and objective data may support the claim that COVID-19 can lead to an increase in cases of vestibular dysfunction after reactivation of neurotropic viruses. CONCLUSIONS Our study and others suggest the possibility of vestibular deficits following the reactivation of VZV following a period of immunosuppression in conjunction with the lowered cell-mediated immunity caused by COVID-19. Additionally, vestibular interventions such as advanced vestibular treatment could be used to improve vestibular function in a linear manner.

Temporal bone manifestation of primary extranodal Rosai–Dorfman disease: a case report

BackgroundRosai–Dorfman disease is a rare benign histiocytic disorder characterized in most cases by painless cervical adenopathy. Less than 10% of extranodal cases involve bony lesions. Primary bone Rosai–Dorfman disease in the absence of nodal disease is extremely rare.Case presentationA 48 year-old Caucasian male presented with progressive right-sided otalgia, tinnitus, vertigo, and hearing loss. A right temporal bone lytic lesion was detected on diagnostic imaging. Resection of the lesion and histopathological examination revealed Rosai–Dorfman disease.ConclusionsRosai–Dorfman disease primary bone lesions are an atypical presentation of a rare disease. This is the second reported case of Rosai–Dorfman disease arising within the temporal bone. This case study reveals that Rosai–Dorfman disease should be considered for patients presenting with inflammatory/lytic lesions of the temporal bone, in cases where infection and malignancy have been excluded.

Emergent Revascularization After Transposition of an Unexpected Intraosseous Anomalous Subarcuate Loop During Vestibular Schwannoma Surgery: A Case Report

BACKGROUND AND IMPORTANCE: The presence of an anomalous anteroinferior cerebellar artery (AICA) embedded within the subarcuate fossa increases the difficulty of cerebellopontine angle (CPA) tumor surgery. Iatrogenic injury of posterior fossa arteries can result in serious morbidity. CLINICAL PRESENTATION: A 70-year-old man presented with right-sided hearing loss and facial dysesthesia. Magnetic resonance imaging showed a tumor with solid and cystic components and 35-mm maximum diameter in the right CPA. The AICA traveled just dorsal to the tumor and was well-developed because the ipsilateral vertebral artery and posteroinferior cerebellar artery (PICA) were aplastic. During surgery, we unexpectedly encountered an anomalous loop of the AICA-PICA which was embedded in the subarcuate fossa. This loop was mobilized using an ultrasonic bone curette to enable further tumor resection. However, it occluded immediately after mobilization and required open thrombectomy and end-to-end anastomosis. After revascularization, near-complete tumor resection was achieved without causing facial nerve dysfunction or brainstem/cerebellar infarction. Pathological examination of the resected anomalous loop showed abnormal focal hypertrophy of the adventitia and the presence of external elastic lamina. CONCLUSION: Mobilization of an anomalous AICA-PICA loop embedded within the subarcuate fossa during VS resection can result in arterial occlusion which requires thrombectomy and revascularization. Surgeons should be aware of this vascular anomaly and be prepared to deal with its ramifications.

Hearing loss classification via AlexNet and Support Vector Machine

This paper presents a new method for detecting hearing loss. Our approach is first to use AlexNet to extract the features. Then, we use the Support Vector Machine as a classifier to classify the images. 10-fold cross-validation results showed that the sensitivities of the healthy control group, the left-sided hearing loss group, and the right-sided hearing loss group in this method were 94.67%, 94.00%, and 95.17%, respectively, achieving a very good effect compared with other hearing loss detection methods. In conclusion, our method is effective for the identification of hearing loss.

Symptoms: Sudden Hearing Loss and Aural Fullness

Symptoms: Sudden Hearing Loss and Aural Fullness

Symptom: Runny Nose Six Years After Acoustic Neuroma Surgery

Symptom: Runny Nose Six Years After Acoustic Neuroma Surgery

Pilocytic astrocytoma of the cerebellopontine angle: a rare case

BackgroundPilocytic astrocytomas are the most common primary brain tumors in children. Most of the lesions occur in or near the midline. They are rare in extra-axial locations.Case presentationWe report a 23-year-old lady who was found to have progressive right-sided sensorineural hearing loss and features of raised intracranial pressure due to a pilocytic astrocytoma of the right cerebellopontine angle. After an extensive Medline search, we could find only three cases of primary extra-axial pilocytic astrocytoma of the cerebellopontine angle (CPA) reported till now.ConclusionEven a rare occurrence, primary pilocytic astrocytoma, and in general gliomas, should be kept in mind in the differential diagnosis of CPA lesions.

Altered topological properties of the intrinsic functional brain network in patients with right-sided unilateral hearing loss caused by acoustic neuroma.

Neuroimaging studies have identified alterations in functional connectivity between specific brain regions in patients with unilateral hearing loss (UHL) and different influence of the side of UHL on neural plasticity. However, little is known about changes of whole-brain functional networks in patients with UHL and whether differences exist in topological organization between right-sided UHL (RUHL) and left-sided UHL (LUHL). To address this issue, we employed resting-state fMRI (rs-fMRI) and graph-theoretical approaches to investigate the topological alterations of brain functional connectomes in patients with RUHL and LUHL. Data from 44 patients with UHL (including 22 RUHL patients and 22 LUHL patients) and 37 healthy control subjects (HCs) were collected. Functional brain networks were constructed for each participant, following by graph-theoretical network analyses at connectional and global (e.g., small-worldness) levels. The correlations between brain network topologies and clinical variables were further studied. Using network-based analysis, we found a subnetwork in the visual cortex which had significantly lower connectivity strength in patients with RUHL as compared to HCs. At global level, all participants showed small-world architecture in functional brain networks, however, significantly lower normalized clustering coefficient and small-worldness were observed in patients with RUHL than in HCs. Moreover, these abnormal network metrics were demonstrated to be correlated with the clinical variables and cognitive performance of patients with RUHL. Notably, no significant alterations in the functional brain networks were found in patients with LUHL. Our findings demonstrate that RUHL (rather than LUHL) is accompanied with aberrant topological organization of the functional brain connectome, indicating different pathophysiological mechanisms between RUHL and LUHL from a viewpoint of network topology.

Primary nasopharyngeal amyloidosis with nasal polyposis: Case report of a diagnostic challenge.

BackgroundPrimary nasopharyngeal amyloidosis is a rare entity of localised amyloidosis. Patients usually present with symptoms that mimic other common nasal diseases. We report an unusual case of nasopharyngeal amyloidosis that co-exist with nasal polyposis at the same time.Case presentationWe described a 72-year-old gentleman who presented with left-sided nasal obstruction, rhinorrhoea and hyposmia and right-sided hearing loss. Examination revealed bilateral intranasal polyposis with right lobular swelling at torus tubarius and right sided middle ear effusion. Biopsy revealed inflammatory nasal polyps with nasopharyngeal amyloidosis. Patient was treated successfully with functional endoscopic sinus surgery (FESS) for nasal polyposis and an en bloc wide local excision of the torus tubarius with no signs of recurrence at one year follow-up.ConclusionClinicians should have raised index of suspicion of a possible primary nasopharyngeal amyloidosis in patients presenting with nasopharyngeal mass with co-existing nasal polyposis to avoid delay in diagnosis and treatment.

Rhabdomyosarcoma of Middle Ear

An eight-year-old girl presented to the outpatient department with a history of decreased hearing from the right ear, bloody ear discharge, and facial deviation to the left side. On clinical examination, she was afebrile with conductive right-sided hearing loss. With a primary diagnosis of unsafe type of chronic suppurative otitis media in mind, computed tomography scan of the temporal bone was performed which showed an aggressive destructive lesion in the petrous temporal bone. Magnetic resonance imaging was then performed to look for soft tissue extension and vascular involvement. Based on the clinical and imaging findings, she was diagnosed as a high-grade neoplasm of the right temporal bone, such as rhabdomyosarcoma. Histopathology finally confirmed the diagnosis of an embryonal type of rhabdomyosarcoma of the middle ear cleft.

Teaching Video NeuroImage: An Uncommon Cause of Hearing Loss.

A 66-year-old woman presented with right-sided hearing loss, insidious in onset. Otoscopic examination showed pulsation of the tympanic membrane in a seated position (Video 1) which diminished on lying down. A CT venogram of the auditory canal showed a dehiscent right jugular bulb along the hypotympanic surface (Figures 1 and 2). A dehiscent jugular bulb develops because of the absence of sigmoid plate separating the bulb from the middle ear. It appears as blue mass behind the tympanic membrane which may distend with Valsalva or internal jugular vein compression. Affected individuals, while often asymptomatic, can experience conductive/sensorineural hearing loss, tinnitus, or vestibular dysfunction.1 The patient is followed with imaging periodically for disease progression. Over 10 years, her hearing has been stable. Neurologists should be familiar with the otoscopic appearance of auditory canal dehiscence as tinnitus, and vestibular dysfunction is a common presentation. Treatment involves reassurance and follow-up with serial imaging. Surgical or endovascular intervention is reserved for intolerable symptoms.2

Wolf-Hirschhorn Syndrome with Hyperparathyroidism: A Case Report and a Narrative Review of the Literature.

Wolf-Hirschhorn syndrome (WHS) is a contiguous gene deletion condition. The WHS core phenotype includes developmental delays, intellectual disabilities, seizures, and distinctive facial features. Various other comorbidities have also been reported, such as hearing loss, heart defects, as well as eye problems and kidney problems. In this report, we present a case of WHS accompanied by hyperparathyroidism and hypercalcemia, which has not been previously reported. A girl was born at 37 weeks of gestation by vaginal delivery. She was small for the gestational age (2,045 g) and admitted to neonatal intensive care unit. She had typical WHS facial features and was found to have bilateral small kidneys associated with transient metabolic acidosis and renal insufficiency. She had right-sided sensorineural hearing loss, a small atrial septal defect, and colpocephaly and hypoplasia of corpus callosum. She had a single seizure which was well controlled with an oral antiepileptic medication. Cytogenetic studies demonstrated a large terminal chromosome 4p deletion (21.4 Mb) and 4p duplication (2.1 Mb) adjacent to the deletion. A unique finding in this patient is her consistently elevated levels of parathyroid hormone and serum calcium, suggesting hyperparathyroidism. We present this rare case along with a review of the literature and hope to draw an attention to a potential relationship between WHS and hyperparathyroidism.

POS-427 WHIPPLE DISEASE IN A PATIENT WITH MEMBRANOUS GLOMERULONEPHRITIS: FIRST CASE IN LITERATURE

The Whipple Disease (WD) is an extremely rare infective condition with an incidence of one individual in a million people1, mainly attributed to the Tropherima Whipplei (TW). It is described as a gram-positive belonging to the family of Actinomycetes.Along with other factors, the genetic plays its role through the immunological hyperactivity towards TW. Here we report an atypical presentation of this disease, in a patient admitted to our Department several times. Male patient, 46 years old, affected by essential epilepsy, treated with Carbamazepine, and a serum-negative rheumatoid arthritis treated with NSAID. The patient reported a right-sided hearing loss due to suspected viral infection and had a history of papillary thyroid carcinoma, treated with a total thyroidectomy and radiometabolic therapy. The patient was hospitalized to our Nephrology Unit for the onset of a nephrotic syndrome (24h-proteinuria 13640 mg/day) and US-guided renal biopsy was performed, where leading to a histological diagnosis of Membranous Nephropathy (MN) with high titre of serum PLA2R antibodies and normal kidney function (Tab.1). Because of his personal history of neoplasia, we could not follow KDIGO guidelines recommendations for MN, which indicate a first-line treatment with an alkylating agent alternated with cortisone for six months2.Therefore we opted for a treatment with mycophenolate of 2g/die. Due to the failed remission, we started a treatment with Rituximab.The patient has been hospitalized three more times for the onset of dyspepsia, anaemia, joint pain, weight loss, diarrhea and fever, which have been studied by the following examinations (Tab. 2). In particular, a diffuse lymphangiectasia was highlighted at the second esophagogastroduodenoscopy (EGD) performed (Fig. 1). The last EGD revealed a flocculating appearance of the second duodenal portion mucosa, resembling grain of millet. The histological examination noted the presence of massive macrophage infiltration and a slight shortening of the villi. The pictures were perfectly compatible with the diagnosis of WD (Fig 2.). The patient undertook antibiotic therapy with doxycycline 200 mg/day in combination with hydroxychloroquine 600 mg/day with a maintenance dosage of prednisone 5 mg/day. After two months, he reached the complete remission of joint and gastrointestinal manifestations with improvement of renal parameters: albuminemia raised of 13% (2.75 mg/dl), and 24h-proteinuria reduced of 60% (3596 mg). The WD diagnosis and the response to the therapy clarified the pathogenesis of other symptoms previously attributed to other idiopathic different diseases. Therefore, we assume that these manifestations may be caused by the overlapping action of the autoimmune renal pathology and WD.

What's Your Diagnosis? Symptoms: Middle Ear Mass and Unilateral Hearing Loss

What's Your Diagnosis? Symptoms: Middle Ear Mass and Unilateral Hearing Loss

Diagnosis of GATA2 Deficiency in a Young Woman with Hemophagocytic Lymphohistiocytosis Triggered by Acute Systemic Cytomegalovirus Infection.

Patient: Female, 22-year-oldFinal Diagnosis: GATA2 deficiency • hemophagocytic lymphohistiocytosisSymptoms: Chest pain • cough • fever • malaise • shortness of breathMedication: —Clinical Procedure: Bone marrow biopsy • bronchoscopySpecialty: ImmunologyObjective:Rare diseaseBackground:Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease characterized by an intense immunologic response that results in multiorgan dysfunction. It typically manifests as a result of a familial genetic immunodeficiency disorder or secondary to a trigger such as an infection, malignancy, or autoimmune disease. The major factors involved in the development of the disease are an individual’s genetic propensity to develop HLH, such as rare associated mutations, or inflammatory processes that trigger the immune system to go haywire.Case Report:Before the COVID-19 pandemic, a 22-year-old woman with a history of congenital absence of the right kidney, right-sided hearing loss, and leukopenia presented with a 3-week history of generalized malaise, fever, chest pain, cough, and shortness of breath. She developed an acute systemic cytomegalovirus infection further complicated by HLH. Based on her history and clinical course, an underlying primary immunodeficiency was suspected. An immunodeficiency gene panel revealed a monoallelic mutation in GATA2, a gene that encodes zinc-transcription factors responsible for the regulation of hematopoiesis.Conclusions:GATA2 deficiency encompasses a large variety of mutations in the GATA2 gene and leads to disorders associated with hematologic and immunologic manifestations of monocytopenia and B-, and natural killer-cell deficiency. Over time, affected individuals are at high risk of developing life-threatening infections and serious hematologic complications, such as myelodysplastic syndromes and/or leukemias. We aimed to illustrate the importance of identifying an underlying genetic disorder associated with secondary HLH to help guide acute and long-term management.

Compensation capacities of brain in unilateral and bilateral auditory system damage in children

Neuroplasticity is a basic feature of neuronal system which provides function rebuilt or developmental dysfunction repair. Hearing impairment can have an impact on intelligence levels, organization and structure of cognitive processes. Cognitive capacities profiles of deaf people present neuropsychological results of functional reorganization of brain. 208 children were accepted for this study. Experimental group consisted of 126 children suffering from sensorineural hearing insufficiency. 26 children were diagnosed with right-sided hearing loss, 34 children were diagnosed with left-sided hearing loss and 66 children were diagnosed with bilateral hearing loss. Control group consisted of 82 normally hearing children, confirmed in audiometric assessment. In order to establish levels of cognitive abilities and intelligence in patients enrolled for the study polish adaptation of Wechsler Intelligence Scale for Children (WISC-R) was administered. Children with bilateral hearing loss obtained worse results in verbal scale compared to well hearing controls. Verbal intelligence in control group was statistically higher than in right-sided auditory dysfunction group. Control group average result in nonverbal scale was statistically significantly higher compared to left-sided hearing loss patients. Children with right-sided auditory dysfunction obtained higher results in nonverbal scale compared to children with left-sided auditory dysfunction. Left-sided hearing impairment group had higher scores in almost all subtests of verbal scale than right-sided hearing impairment group. Left-sided auditory disability enhances linguistic capacity, which indicates compensation abilities of left hemisphere responsible for verbal processes. Right-sided auditory disability enhances analytic processes, visual synthesis and image reasoning, which indicates compensation abilities of right hemisphere responsible for nonverbal processes.