Sensorineural hearing loss

Miliary tuberculosis is a severe form of tuberculosis resulting from dissemination of Mycobacterium tuberculosis bacilli. Since symptoms appearing in patients due to miliary TB are diverse and atypical, depending on the site of invasion, early diagnosis and treatment are important. A paradoxical response of tuberculosis is a rare phenomenon and it can be a clinical difficulty to treatment especially when involving the central nervous system. We present a case report with a review of related literature about the patient who developed sudden hearing loss due to tuberculosis infection in vestibulocochlear area...

OBJECTIVE: To present a case of sudden sensorineural hearing loss (SSNHL) and the related diagnostic work-up. CLINICAL PRESENTATION AND INTERVENTION: A 54-year-old man presented with unilateral SSNHL episode and vertigo. A severe patent foramen ovale (PFO) that included a complete ear nose and throat, audiological and neurological examination was discovered during the diagnostic work-up. Audiometry, blood and serological tests were performed. Magnetic resonance imaging (MRI), supra-aortic trunks and lower limbs color Doppler echography were done...

Objectives: Local administration of 3-nitropropionic acid (3-NP) to the inner ear induces sensorineural hearing loss. Several studies have shown the otoprotective effects of ginkgo biloba extract EGb 761. Moreover, EGb 761 has been reported to activate Sirtuin 1 (SIRT1). The present study was designed to investigate whether EGb 761 prevents 3-NPinduced sensorineural hearing loss and determine its effects on the expression of SIRT1. Methods: Sprague Dawley rats were divided into four experimental groups: control group receiving vehicle of 3-NP, EGb group receiving EGb 761, 3-NP group receiving 3-NP, and EGb+3-NP group receiving EGb 761 and 3-NP...

To combine the benefits of perimodiolar stimulation with minimal insertion trauma, a thin, pre-curved electrode (CI532) was recently developed by Cochlear Ltd. (Sidney). This array is held straight prior to insertion by an external polymer reloadable sheath that is removed after full electrode insertion. Sixty-seven patients suffering from severe-to-profound sensorineural hearing loss (mean age 42.2 years; mean duration of the hearing loss 19.6 years; mean PTA thresholds at 250-2000 Hz 92.4 dB HL) were implanted with the CI532...

AIM OF THE STUDY: Recent advances in understanding the underlying molecular mechanism for distal renal tubular acidosis (dRTA), led to an increased attention towards the primary and the familial forms of the disease. Mutations in ATP6V1B1 and ATP6V0A4 are usually responsible for the recessive form of the disease. Mutations in gene AE1 encoding the Cl-/HCO3- exchanger, usually present as dominant dRTA, but a recessive pattern has been recently described. Our objective is to identify the mutational spectrum responsible of dRTA in a consanguineous Libyan family...

Among various forms of hearing loss, there are acute (within 72 hours) or sub-acute (weeks to months) presentations that may be reversible with early pharmacological intervention. The workup of a patient presenting with hypoacusia includes the usual history and physical examination in conjunction with an audiometric assessment in order to categorize the hearing loss as conductive, sensorineural, or mixed. Sudden sensorineural hearing loss (SSNHL) and autoimmune inner ear disease (AIED) are acute and subacute forms of sensorineural hypoacusia most likely to be reversed with prompt pharmacological intervention...

A previously healthy 77-year-old Japanese man presented with a 2-week history of daily fevers peaking at 38°C, chills, hearing loss, and almost 10 kg of unintentional weight loss over 2 months. Pure tone audiometry showed mixed conductive-sensorineural hearing loss: right, 63.6 dB, left, 80.0 dB. Blood tests after admission showed a high myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) level (>300 U/mL), so we suspected ANCA-related vasculitis. The Japanese Otorhinolaryngology Society has recently been advocating the concept of otitis media with ANCA-associated vasculitis (OMAAV)...

Light-gated ion channels and transporters have been applied to a broad array of excitable cells including neurons, cardiac myocytes, skeletal muscle cells and pancreatic β-cells in an organism to clarify their physiological and pathological roles. Nonetheless, among nonexcitable cells, only glial cells have been studied in vivo by this approach. Here, by optogenetic stimulation of a different nonexcitable cell type in the cochlea of the inner ear, we induce and control hearing loss. To our knowledge, deafness animal models using optogenetics have not yet been established...

Vestibular schwannoma (VS) is the most common tumor of the cerebellopontine angle, and it typically presents with sensorineural hearing loss. The genomic landscape of schwannoma is complex and many of the molecules implicated in VS pathogenesis represent targets not amenable to antibody-based or small molecule therapeutics. Tumor-targeted delivery of small interfering RNA (siRNA) therapeutics provides a direct and effective means to interrogate targets while minimizing off-target effects. To establish a preclinical model for therapeutic inhibition of putative targets in VS, archived tumor specimens, fresh tumor cells derived from patients with sporadic VS, and an established schwannoma cell line were screened...

INTRODUCTION: The consequence of an exposure to intense sounds can be a temporary or permanent hearing loss and even with a rapid therapeutic management, severe sensorineural sequelae may persist. METHODS: the authors report a retrospective study about 64 patients followed for an acute acoustic trauma during a period of 8 years (2006 to 2013). For all the cases, a clinical examination associated to a pure-tone audiometry was conducted. Hearing levels were measured at the frequencies 500, 1000, 2000 and 3000 Hertz...

Distal renal tubular acidosis (dRTA) is a tubular disorder with a primary defect of urinary acidification and acid excretion in the collecting duct system. Consequently, patients develop hyperchloremic metabolic acidosis with an inappropriately alkaline urine. Inherited forms of dRTA are due to mutations in at least three distinct genes: SLC4A1, ATP6V1B1, ATP6V0A4. Mutations in SLC4A1-(AE1) are inherited either in an autosomal dominant manner or in a recessive one. ATP6V1B and ATP6V0A4 mutations affect two different subunits of the vacuolar H(+)-ATPase proton-pump, the B1 and a4 subunits, and are inherited in an autosomal recessive manner...

OBJECTIVE: Early prediction of therapeutic outcomes could reduce exposure to ineffective treatments and optimize clinical outcomes. However, none of the known otologic predictors is amenable to therapeutic intervention for idiopathic sudden sensorineural hearing loss (ISSNHL). The aims of this study were to investigate psychological stress as a potential predictor to discriminate outcomes in ISSNHL. METHODS: Various psychological measures were conducted including structured interview assessment tools in patients with recently diagnosed ISSNHL before initiating treatment...

OBJECTIVE: To examine the value of a subjective numerical rating scale (NRS) in the initial evaluation of patients suspected of suffering from unilateral sudden sensorineural hearing loss (SSNHL) until a formal audiogram is available. STUDY DESIGN: Prospective noncontrolled clinical study. METHODS: Thirty-one consecutive patients referred to the emergency department due to suspected unilateral SSNHL and with no other aural pathology by history or physical examination were enrolled...

AIM: This study aimed to estimate long-term impairment attributable to congenital cytomegalovirus infection (cCMV). METHOD: This nationwide cohort study retrospectively assessed cCMV in children born in 2008 in the Netherlands, testing 31 484 stored neonatal dried blood spots. Extensive medical data of cCMV-positive children (n=133) and matched cCMV-negative comparison children (n=274) up to 6 years of age were analysed. RESULTS: Moderate to severe long-term impairment was diagnosed in 24...

Enlarged vestibular aqueduct (EVA)‑associated hearing loss is frequently detected in individuals carrying the SLC26A4 mutation in the Chinese population. The present study aimed to identify the causative SLC26A4 coding mutations in a patient group with nonsyndromic hearing loss (NSHL) and EVA. Genomic DNA was extracted from blood samples obtained from 52 NSHL patients with EVA and from 60 normal controls. The mutation analysis for 20 coding exons of SLC26A4 was performed by direct sequencing. The results of the mutational analysis showed that there were two probands from two separate families suffering from bilateral sensorineural hearing loss with EVA, carrying the same novel compound heterozygous mutation of SLC26A4 (c...

Although the tonotopic organisation of the human primary auditory cortex (PAC) has already been studied, the question how its responses are affected in sensorineural hearing loss remains open. Twenty six patients (aged 38.1 ± 9.1 years; 12 men) with symmetrical sloping sensorineural hearing loss (SNHL) and 32 age- and gender-matched controls (NH) participated in an fMRI study using a sparse protocol. The stimuli were binaural 8s complex tones with central frequencies of 400 HzCF, 800 HzCF, 1600 HzCF, 3200 HzCF, or 6400 HzCF, presented at 80 dB(C)...

OBJECTIVE: This study aimed to clarify the molecular epidemiology of hearing loss by identifying the responsible genes in patients without GJB2 mutations. STUDY DESIGN: Prospective genetic study. SETTING: Tertiary referral hospital. PATIENTS: Fifty one patients with bilateral sensorineural hearing loss, 20 men, and 31 women, mean age 24.9 years, range 3 to 64 years, from 49 families. GJB2 and deltaGJB6-D13S1830 mutations were excluded previously...

HYPOTHESIS: Cochlear dysfunction is not common in human meningioma of the internal auditory canal. BACKGROUND: Meningiomas arising from the cerebellopontine angle and internal auditory canal typically cause hearing loss. Cochlear dysfunction is known to contribute to sensorineural hearing loss induced by vestibular schwannoma, the most common tumor of the internal auditory canal. Detailed cochlear histopathology in meningioma has not been reported. METHODS: Retrospective analysis of cochlear histopathology in five unoperated and five operated meningiomas of the internal auditory canal identified after screening human temporal bone collections from three academic medical centers...

BACKGROUND AND PURPOSE: Focal low-attenuation outpouching or diverticulum at the anterolateral internal auditory canal is an uncommon finding on CT of the temporal bone. This finding has been described as cavitary otosclerosis in small case reports and histology series. The purpose of this study was to establish the prevalence of internal auditory canal diverticulum and its association with classic imaging findings of otosclerosis and/or hearing loss. MATERIALS AND METHODS: Temporal bone CT scans of 807 patients, obtained between January 2013 and January 2016, were retrospectively reviewed to identify internal auditory canal diverticula and/or classic imaging findings of otosclerosis...

The emergence of Zika-virus-associated congenital microcephaly has engendered renewed interest in the pathogenesis of microcephaly induced by infectious agents. Three of the original "TORCH" agents are associated with an appreciable incidence of congenital microcephaly: cytomegalovirus, rubella virus, and Toxoplasma gondii. The pathology of congenital microcephaly is characterized by neurotropic infectious agents that involve the fetal nervous system, leading to brain destruction with calcifications, microcephaly, sensorineural hearing loss, and ophthalmologic abnormalities...