Right-sided Hearing Loss Research Articles

Altered Contralateral Auditory Cortical Morphology in Unilateral Sudden Sensorineural Hearing Loss

Objective:To investigate the cerebral gray matter volume alterations in unilateral sudden sensorineural hearing loss patients within the acute period by the voxel-based morphometry method, and to determine if hearing impairment is associated with regional gray matter alterations in unilateral sudden sensorineural hearing loss patients.Study Design:Prospective case study.Setting:Tertiary class A teaching hospital.Patients:Thirty-nine patients with left-side unilateral sudden sensorineural hearing loss and 47 patients with right-side unilateral sudden sensorineural hearing loss.Intervention:Diagnostic.Main Outcome Measure:To compare the regional gray matter of unilateral sudden sensorineural hearing loss patients and healthy control participants.Results:Compared with control groups, patients with left side unilateral sudden sensorineural hearing loss had significant gray matter reductions in the right middle temporal gyrus and right superior temporal gyrus, whereas patients with right side unilateral sudden sensorineural hearing loss showed gray matter decreases in the left superior temporal gyrus and left middle temporal gyrus. A significant negative correlation with the duration of the sudden sensorineural hearing loss (R = −0.427, p = 0.012 for left-side unilateral SSNHL and R = −0.412, p = 0.013 for right-side unilateral SSNHL) was also found in these brain areas. There was no region with increased gray matter found in both groups of unilateral sudden sensorineural hearing loss patients.Conclusions:This study confirms that detectable decreased contralateral auditory cortical morphological changes have occurred in unilateral SSNHL patients within the acute period by voxel-based morphometry methods. The gray matter volumes of these brain areas also perform a negative correlation with the duration of the disease, which suggests a gradual brain structural impairment after the progression of the disease.

Aberrant topological properties of whole-brain functional network in chronic right-sided sensorineural hearing loss: a resting-state functional MRI study

To investigate the topological properties of the functional brain network in unilateral sensorineural hearing loss patients. In this study, we acquired resting-state BOLD- fMRI data from 19 right-sided SNHL patients and 31 healthy controls with normal hearing and constructed their whole brain functional networks. Two-sample two-tailed t-tests were performed to investigate group differences in topological parameters between the USNHL patients and the controls. Partial correlation analysis was conducted to determine the relationships between the network metrics and USNHL-related variables. Both USNHL patients and controls exhibited small-word architecture in their brain functional networks within the range 0. 1 - 0. 2 of sparsity. Compared to the controls, USNHL patients showed significant increase in characteristic path length and normalized characteristic path length, but significant decrease in global efficiency. Clustering coefficient, local efficiency and normalized clustering coefficient demonstrated no significant difference. Furthermore, USNHL patients exhibited no significant association between the altered network metrics and the duration of USNHL or the severity of hearing loss. Our results indicated the altered topological properties of whole brain functional networks in USNHL patients, which may help us to understand pathophysiologic mechanism of USNHL patients.

An unusual cause of sudden hearing loss. Probable neurosarcoidosis (NS).

AnAfricanAmericanman in his 40s initially presentedwith a sudden-onset unilateral hearing losswith associated vertigo and tinnitus. His medical history was unremarkable, and he specifically had nohistory of any auditory or vestibular symptoms. Results fromhis physicalexaminationwerealsounremarkableanddemonstratednormal otoscopic and neurologic findings. Audiometry results revealed amoderate right-sided sensorineural hearing loss across all frequencies (250-8000Hz). Prior audiogramswereunavailable for comparison. The patient was treated with tapered prednisone therapy and scheduled for magnetic resonance imaging (MRI) radiographic evaluation. Subsequent clinical and audiology evaluationsat 1weekand 1monthdemonstratedanalmost complete resolutionofhishearing lossandvertigosymptoms.HisMRIwasobtained 2 months after onset of his symptoms with the enhancement pattern shown (Figure). What is your diagnosis? A B

Sensorineural Hearing Loss: What Lies Beneath? Neurovascular Conflict Secondary to a Dural Arteriovenous Malformation

This middle-aged gentleman with no previous medical history presented to the local ENT outpatient clinic complaining of right-sided hearing loss. No history of trauma or previous head and neck surgery was elicited.
 Following clinical and auditory assessment a right sensorineural hearing loss was confirmed. A right-sided facial palsy was additionally identified on examination.
 A MRI of the internal auditory meati was performed (Figure 1a & 1b). Following radiologist review, MRI and MRA of the brain was undertaken.
 
 DISCUSSION
 
 Auditory impairment is a condition with a legion of potential causes. One of the routine aspects of the assessment process for those with sensorineural hearing loss is MR imaging (MRI) of the internal auditory meati (IAMS).
 
 The vast majority of MRI studies are normal, however one of the more commonly identified pathologies are cerebrovascular abnormalities. The most well recognised is neurovascular conflict of the vestibulocochlear nerve by a vascular loop at the root entry zone (REZ), however a broader range of potential responsible structural abnormalities are known. A wide range of processes for auditory dysfunction have been outlined.1 These include; cerebral ischaemia events, subarachnoid haemorrhage, cerebrovascular malformations and rarely dural arteriovenous fistulas (dAVFs).
 
 Dural AVF's are abnormal vascular communications between the dural venous sinuses and an arter(ies) - most frequently branches of the external carotid artery. Sensorineural hearing impairment is one of the rarer presenting symptoms. The mechanism for hearing impairment is believed to result from either direct vascular compression on the vestibulocochlear nerve from an enlarged aberrant draining vein or from a vascular steal phenomenon (Figures 2a & 2b). An engorged draining vein from the dAVF causing mechanical compression on the nerve is the most well recognized.2 A single prior case has been reported of compression from an intraossesous dAVF of the skull base.3
 
 The arteriovenous fistula may be directed identified (Figure 3) along with the associated signs of enlarged cerebral cortical veins and white matter change of venous hypertension (Figure 4).

P16.34 * BEVACIZUMAB TREATMENT FOR ACOUSTIC NEURINOMA IN NEUROFIBROMATOSIS TYPE 2: A CASE REPORT

Neurofibromatosis type 2 (NF2) is a dominantly inherited genetic condition. Unlike patients with sporadic tumours, NF2 patients often have multiple meningiomas, acoustic neurinomas and ependymomas. The multiplicity of tumours make surgery and radiation therapy for all tumours impracticable. In effect, bilateral acoustic neurinomas are the hallmark of this disease and these tumours cause progressive hearing loss in most patients. Neovascularization is necessary for tumour growth and is driven by tumour produced angiogenic factors such as vascular endothelial growth factor (VEGF). Bevacizumab is a humanized monoclonal antibody that neutralizes the activity of VEGF. Recent data showed that VEGF is produced by schwannoma tumour cells. Also, bevacizumab treatment, in patients with NF2 who were considered poor candidates for surgery and radiation therapy, was followed by clinically meaningful hearing improvement and tumour-volume reduction. We report the case of a 39 years old woman with sudden rightsided hearing loss. Magnetic resonance imaging (MRI) revealed multiple meningiomas and neurinomas (C2 and L5 lesions) and a right side acoustic neurinoma of 26x24 mm, confirming the diagnosis of NF2. Bevacizumab was given as an infusion every 2 weeks at a dose of 5.0 mg/kg body weight. Before the treatment, the patient has a thorough medical evaluation including blood pressure, urine analysis, liver and kidney function tests, coagulation status and history of thrombosis, hemoglobin, leukocyte count and thrombocyte count. Three months after the start of therapy the patient has reported an hearing response with progressive improvement in audiometry, word recognition and face-to-face conversation. Six months after the start of therapy, the right acoustic neurinoma had regressed and, also, mitigation of brain stem compression was clearly visible on the cranial MRI scans. The patient, after 12 months, continued to be treated with bevacizumab. In our experience no toxic effects drug-related has been seen; anyway, the absence of many common toxic effects of bevacizumab in our patient may have reflected her youth and the lack of other medical conditions. The exact mechanism of the action of bevacizumab on acoustic neurinomas remains to be elucidated. The drug may also suppress growth of tumour Schwann cells directly, a possibility that can be tested in vitro in future studies. The encouraging results with bevacizumab suggest consideration of a prospective clinical trial for progressive NF2-related acoustic neurinomas, especially for patients with imminent total hearing loss.

IL-1β inhibition in autoimmune inner ear disease: can you hear me now?

Clinical vignette: A 51-year-old man with right-sided sudden hearing loss presents to the otology clinic. He has a 4-year history of episodic vertigo of several hours' duration and fluctuating, progressive sensorineural hearing loss in his left ear. The vertigo attacks have not occurred for the last 18 months, and the left ear hearing is consistently poor. The patient's right ear hearing has dropped in the last 36 hours. MRI imaging of brain and temporal bone are normal. A 2-week "burst and taper" of oral prednisone is administered with no effect. Over the next 3 months, serial audiograms show rapidly progressive loss of threshold and word recognition scores on the right side. A trial of high-dose prednisone (60 mg/d for 30 days) results in full recovery of the right ear hearing and substantial improvement in the left ear. As the prednisone dose is slowly tapered over several months, the hearing drops again.

Ectopic pituitary adenoma associated with an empty sella presenting with hearing loss: case report with literature review

Ectopic pituitary adenomas are uncommon entities that may pose substantial diagnostic challenges. In the majority of these cases, patients present with endocrine and/or nasal obstruction symptoms. We report the case of an ectopic pituitary adenoma in a 76-year-old man with an empty sella who initially presented with right-sided hearing loss progressing to bilateral hearing loss over the next 4 years. Neuroimaging studies revealed a large, expansile central skull base mass replacing the clivus and sphenoid sinus, and invading the internal auditory canals and inner ear bilaterally. The tumor also involved the floor of the middle cranial fossae and bilateral medial temporal and occipital bones. Histopathologic examination, including immunohistochemical studies, revealed a sparsely granulated lactotroph adenoma. Hearing loss in a patient with ectopic pituitary adenoma constitutes an extremely unusual presentation. This case was further complicated by the presence of an empty sella and the absence of symptoms related to hyperprolactinemia.

Recurrent Temporal Bone Tenosynovial Giant Cell Tumor with Chondroid Metaplasia: The Use of Imaging to Assess Recurrence

Tenosynovial giant cell tumor (TGCT) is a benign proliferative lesion of unclear etiology. It is predominantly monoarticular and involves the synovium of the joint, tendon sheath, and bursa. TGCT of the temporomandibular joint (TMJ) is rare and aggressive resulting in destruction of surrounding structures. The diagnosis may be suggested by imaging, mainly by the MR features and PET/CT, and confirmed by histopathology. We describe the case of a 50-year-old man who presented with right-sided hearing loss, tinnitus and TMJ pain. Pathology revealed tenosynovial giant cell tumor with chondroid metaplasia. Six years later he developed a recurrence, which was documented to our knowledge for the first time with CT, MR and FDG PET/CT imaging.

An unusual cause of chronic otitis media with effusion

To describe an unusual cause for hearing loss in an adult. A 37-year-old man presented with a year's history of right-sided hearing loss. He had no history of trauma, or local or systemic infection. He was otherwise well, took no medication and had no allergies. He had a two-year history of low back pain. On examination, he had a retracted right tympanic membrane with no perforation, and a middle-ear effusion. Nasendoscopy was normal. Examination of other systems was unremarkable. Computed tomography of the temporal bones with contrast demonstrated a large, right, intra-cerebral internal carotid artery aneurysm compressing the eustachian tube. After a balloon occlusion test, he underwent endovascular occlusion of the parent vessel. He made a good post-operative recovery. A subsequent abdominal ultrasound excluded an abdominal aneurysm as a cause of his low back pain. His hearing had not improved three days post-operatively, and was to be formally assessed and monitored in the clinic. A carotid aneurysm is a rare cause of eustachian tube compression but must be considered in the differential diagnosis of conductive hearing loss. Aneurysms may have systemic causes, and their presence in other systems should be excluded as they may be multiple.

Vertigo and Sudden Hearing Loss at 35 000 Feet

Awoman in her 70s presented after experiencing a sudden rightsidedhearing loss duringdescent ona commercial air flight. She reportedright-sidedtinnitus,otalgia,andvertigowithnauseaandvomiting.Hermedical history includedeustachian tubedysfunctionand pressure-induced tympanicmembraneperforations; however, she deniedahistoryof vertigo. TheWeber test lateralized to the left ear using a 512-Hz tuning fork. Cranial nerves III toVII and IX toXIIwere normal. Thepatientwasnonambulatoryandcomplainedofmotioninduced vestibular symptoms. Audiometric test results revealed a right-sided profound mixed hearing loss with severe discrimination impairment, and the left ear hada severehigh-frequencyhearing losswith normalworddiscrimination. The right pure-tone average (PTA)was 85 dB and the left was 22 dB. Shewas treatedwith a prednisone taper, and a right ventilation tube was placed in anticipation of possible eustachian tube problems with future air travel. Vestibular and balance symptoms improved with vestibular rehabilitation and physical therapy over the next several months. Repeated audiometric tests 1 month after the incident revealed a PTA of 17 dB and 15 dB on the right and left sides, respectively, with normal bilateral word discrimination. Subjective right hearing had returned to its baseline status. Shown herein are a noncontrast computed tomographic (CT) image of the head (Figure, A), CT images of the right temporal bone in the axial plane (Figure, B and C), and a right coronal oblique CT image (Stenver projection) (Figure, D). What is your diagnosis? A B

Translabyrinthine resection of small intracanalicular acoustic tumor

This case is an example of a translabyrinthine resection of a small intracanalicular acoustic tumor. The patient is a 69-year-old right-handed woman with complaints of progressive incapacitating vertigo and right-sided hearing loss worsening over the past 3 years. She had normal facial nerve function with imaging demonstrating progressive increase in size of a small right-sided acoustic tumor. A translabyrinthine approach was performed, and the mass was resected completely. Facial nerve function remained normal immediately after surgery. The video can be found here: http://youtu.be/27ARlLLSbKE .

Combination of hearing screening and genetic screening for deafness-susceptibility genes in newborns

The aim of this study was to determine the clinical significance of the results of screening of newborn hearing and the incidence of deafness-susceptibility genes. One thousand newborn babies in the Handan Center Hospital (Handan, China) underwent screening of hearing and deafness-susceptibility genes. The first screening test was carried out using otoacoustic emissions (OAEs). Babies with hearing loss who failed to pass the initial screening were scheduled for rescreening at 42 days after birth. Cord blood was used for the screening of deafness-susceptibility genes, namely the GJB2, SLC26A4 and mitochondrial 12S rRNA (MTRNR1) genes. Among the 1,000 neonates that underwent the first hearing screening, 25 exhibited left-sided hearing loss, 21 exhibited right-sided hearing loss and 15 cases had binaural hearing loss. After rescreening 42 days later, only one of the initial 61 cases exhibited hearing loss under OAE testing. The neonatal deafness gene tests showed two cases with 1555A>G mutation and two cases with 1494C>T mutation of the MTRNR1 gene. In the SLC26A4 gene screening, four cases exhibited the heterozygous IVS7-2A>G mutation and one case exhibited heterozygous 1226G>A mutation. In the GJB2 gene screening, two cases exhibited the homozygous 427C>T mutation and 10 exhibited the heterozygous 235delC mutation. The genetic screening revealed 21 newborns with mutations in the three deafness-susceptibility genes. The overall carrier rate was 2.1% (21/1,000). The association of hearing and gene screening may be the promising screening strategy for the diagnosis of hearing loss.

See no evil, hear no evil…

A 32-year-old woman presented with right-sided visual loss, hearing loss and diffuse headache. Examination confirmed CN-II, V1/2, and VIII neuropathies. MRI showed an infiltrative skull-base process involving the pachymeninges. Serology and histopathology confirmed idiopathic hypertrophic pachymeningitis.

Lateralized Auditory Symptoms in Central Neuroaudiology Disorder

Many individuals with central auditory nervous system (CANS) pathology/disorder report a variety of auditory symptoms with difficulty hearing in noise being one of the most common complaints (Chermak and Musiek, 1992) Interestingly, there seems to be a paucity of information in the literature on lateralized hearing loss symptoms in patients with central auditory disorders. Reported here is a case where the patient reported auditory symptoms, specifically hearing loss that was lateralized distinctly to the right ear. This ear was contralateral to a stroke that involved the left hemisphere with neural compromise limited primarily to the left Heschl's gyrus. Although the patient reported other hearing difficulties, the lateralized symptom of right-sided hearing loss seemed to be the most noticed. While the pure-tone audiogram was totally disassociated from the right ear auditory symptoms, central auditory tests revealed a marked and consistent right ear deficit, which was consistent with the site of lesion. It is rare that a relatively small lesion in Heschl's gyrus results in the perception of hearing loss so specifically localized to the right ear. It is also of interest that a patient with definite complaints of hearing loss would yield a normal, highly symmetrical pure-tone audiogram. The triad of patient symptoms, anatomy of the lesion, and central auditory test findings in this case are discussed, and theories as to possible underlying mechanisms for the patient's auditory deficits are provided.

Giant cell reparative granuloma of the temporal bone successfully resected with preservation of hearing

To describe a case of giant cell reparative granuloma of the temporal bone which extended into the middle-ear cavity, and which was successfully treated surgically via a transmastoid approach, with hearing preservation. A 37-year-old man presented with a one-year history of right-sided hearing loss, complicated by a three-month history of otalgia and a sensation of aural fullness. Computed tomography and magnetic resonance imaging demonstrated an osteolytic tumour lesion in the right temporal bone. The diagnosis was confirmed by biopsy from the mastoid lesion. INVESTIGATION AND INTERVENTION: Pure-tone audiometry, computed tomography and magnetic resonance imaging were conducted, followed by total resection. The giant cell reparative granuloma of the temporal bone was completely resected, with preservation of hearing. Although this patient's giant cell reparative granuloma of the temporal bone extended into the middle-ear cavity, total resection was achieved, with preservation of hearing. To the best of our knowledge, hearing preservation following resection of giant cell reparative granuloma of the temporal bone has not previously been reported.

Adult-Onset Labyrinthine Fistula as a Sign of Undiagnosed Bilateral Congenital Cholesteatoma

Objective. Bilateral congenital cholesteatoma is a very rare condition. We describe the first case of an adult patient presenting with bilateral congenital cholesteatoma with labyrinthine fistula. Case report. We present a 34-year-old woman of bilateral congenital cholesteatoma with labyrinthine fistula, inducing left- sided mixed conductive-sensorineural hearing loss and vertigo, subsequently accompanied by right-sided conductive hearing loss. Otoscopic examination and computed tomography scan revealed bilateral congenital cholesteatoma with a fistula in the left lateral semicircular canal. Tympanomastoidectomy was performed, and both congenital cholesteatoma were completely removed, and membranous fistula of the left lateral semicircular canal was covered with a graft. Conclusion. Congenital cholesteatoma is usually detected in childhood not but in adulthood. With delay in diagnosis, the risk of cholesteatoma complications increases. Greater attention should be paid to complications such as labyrinthine fistula resulting from long-term exposure to congenital cholesteatoma in adults.

An Apparent Sporadic Endolymphatic Sac Tumor in a 14-Year-Old Boy

An Apparent Sporadic Endolymphatic Sac Tumor in a 14-Year-Old Boy Endolymphatic sac tumors (ELSTs) are rare, low-grade, histologically benign tumors arising from the epithelial lining of the endolymphatic duct or sac. They are slow growing but locally invasive and can be associated with von Hippel-Lindau disease. While there are cases in the medical literature documenting this type of tumor in adults, reports of ELSTs in the pediatric population are limited.The authors report the fifth reported case of a pediatric ELST in the English literature. A 14-year-old Jehovah’s Witness presented with a progressive two-year history of right-sided hearing loss, balance problems, gait difficulty, and bleeding behind the right tympanic membrane. Computed topography (CT) and magnetic resonance imaging (MRI) of the brain and temporal bone revealed a large lesion eroding into the petrous and mastoid bones with extension abutting the brain stem and invading the right transverse sinus.

Large Bilateral Internal Auditory Meatus Associated with Bilateral Superior Semicircular Canal Dehiscence

Superior semicircular canal dehiscence and an abnormally wide internal auditory meatus are clinical entities characterized by vestibular and cochlear symptoms. These symptoms are induced by hypersensitivity of labyrinthine receptors secondary to a bone defect of the otic capsule. We report the case of a 41-year-old man with congenital right-sided hearing loss who presented with bilateral superior semicircular canal dehiscence that was associated with wide, bulbous internal auditory meatus and a loss of the bony wall separating the lateral end of the meatus from the cochlea. The patient was experiencing vestibular and cochlear symptoms in the right ear and disabling tinnitus in the left ear. However, he refused all treatment and was lost to follow-up.

Freiburg Neuropathology Case Conference: Widespread Mass Lesions After Resection of a Glioblastoma Multiforme

an 80-year-old male patient presented with intermittent aphasia, focal seizures with speech arrest and gait disturbance. Magnetic resonance imaging (MRi) revealed a contrast-enhancing lesion in the left temporal lobe (Fig. 1). a left temporal craniotomy was performed and a complete microsurgical resection of the lesion was achieved as demonstrated by early postoperative MRi (not shown). neuropathological analysis revealed a glioblastoma multiforme WHO grade iV. The neurological symptoms resolved completely. The patient was treated with external beam radiation therapy (60 Gy). Four months after surgery, he presented with fatigue and reduced general condition. MRi revealed a contrast-enhancing rim around the resection cavity and a subdural hygroma with compression of the sulci of the left hemisphere (Fig. 2). He was treated with a subdural drainage for 3 days. although the symptoms improved, computed tomography (CT) showed no resolution of the subdural effusion. The patient received four cycles of standard temozolomide chemotherapy (200 mg/m2, 5/28 pattern). eight months after the surgery, the follow-up MRi revealed multiple intraand extraaxial contrast-enhancing mass lesions located supraand infratentorially (Fig. 3). Meanwhile, the patient had developed a right-sided facial paresis and hearing loss, dysmetria and severe ataxia. Open biopsy was performed and an intradural, extracerebral lesion in the right parietal area was excised. The patient was transferred into palliative care.

Cochlin-tomoprotein (CTP) detection test identified perilymph leakage preoperatively in revision stapes surgery

Perilymphatic fistula (PLF) is defined as an abnormal leakage between perilymph from the labyrinth to the middle ear. Symptoms include hearing loss, tinnitus, and vertigo. The standard mode of PLF detection is intraoperative visualization of perilymph leakage and fistula, which ostensibly confirms the existence of PLF. Other possible methods of diagnosis include confirmation of pneumolabyrinth via diagnostic imaging. Recently, a cochlin-tomoprotein (CTP) detection test has been developed that allows definitive diagnosis of PLF-related hearing loss. We report the case of a 45-year-old man who presented with right-sided tinnitus, hearing loss, and dizziness 30 years after stapes surgery. Middle ear lavage was performed after myringotomy. A preoperative diagnosis of PLF was reached using the CTP detection test. Intraoperative observations included a necrotic long process of the incus, displaced wire piston, and fibrous tissue in the oval window. Perilymph leakage was not evident. The oval window was closed with fascia, and vertigo disappeared within 2 weeks postoperatively. When PLF is suspected after stapes surgery, the CTP detection test can be a useful, highly sensitive, and less invasive method for preoperative diagnosis.