Sensorineural hearing loss

AIM: Very preterm infants are at risk of neonatal hearing loss. However, it is unknown whether infants with a normal neonatal hearing screening result risk sensorineural hearing loss (SNHL) at a later age. METHODS: This cohort study was conducted at the Erasmus Medical University Center Rotterdam, the Netherlands, on 77 very preterm infants born between October 2005 and September 2008. All infants underwent auditory brainstem response audiometry during neonatal hearing screening and at two years of corrected age...

Mutations in the mitochondrial DNA have been certified to be one of the most important causes of maternally inherited sensorineural hearing loss. Among these, mitochondrial 12S rRNA1555A>G, 1494C>T and other mutations are associated with both nonsyndromic and drug induced hearing loss caused by aminoglycosides. Individuals carrying 1555A>G or 1494C>T mutation have a variety of clinical manifestations, which implies that the 1555A>G or 1494C>T mutation is a chief factor underlying the development of deafness but insufficient to produce the clinical phenotype...

The objective of the study was to discuss the findings of intraoperative electrically evoked auditory brainstem response (eABR) test results with a recently designed intracochlear test electrode (ITE) in terms of their relation to decisions of cochlear or auditory brainstem implantation. This clinical study was conducted in Hacettepe University, Department of Otolaryngology, Head and Neck Surgery and Department of Audiology. Subjects were selected from inner ear malformation (IEM) database. Eleven subjects with profound sensorineural hearing loss were included in the current study with age range from 1 year 3 months to 4 years 3 months for children with prelingual hearing loss...

Cochlear implants (CIs) are surgically implanted medical devices used to treat individuals with severe-to-profound sensorineural hearing loss. Although these devices have been remarkably successful at restoring audibility, many patients experience poor outcomes. Our group has developed the first image-guided CI programming technique where the electrode positions are found in CT images and used to estimate neural activation patterns, which is unique information that audiologists can use to define patient-specific processor settings...

PURPOSE: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL). However, accurate diagnosis of cCMV as the etiology of SNHL is problematic beyond the neonatal period. This study therefore examined whether cCMV infection could be identified retrospectively in children presenting with unexplained SNHL to a multidisciplinary diagnostic outpatient otolaryngology clinic at an academic medical center in Minnesota. METHODS: Over a 4-year period, 57 patients with an age range of 3months to 10years with unexplained SNHL were recruited to participate in this study...

We herein report a unique case of a lateral pontine demyelinating lesion presenting with unilateral sensorineural hearing loss and paradoxical ipsilateral hyperacusis. The association of unilateral hearing loss and ipsilateral hyperacusis is a rare manifestation of a central nervous system lesion. The paradoxical combination of these symptoms strongly suggests pontine dysfunction and prompts urgent neurological evaluation.

PURPOSE: We aimed to determine if a non-contrast screening MRI is cost-effective compared to a full MRI protocol with contrast for the evaluation of vestibular schwannomas. METHODS: A decision tree was constructed to evaluate full MRI and screening MRI strategies for patients with asymmetric sensorineural hearing loss. If a patient were to have a positive screening MRI, s/he received a full MRI. Vestibular schwannoma prevalence, MRI specificity and sensitivity, and gadolinium anaphylaxis incidence were obtained through literature review...

PurposeIn 2012 we reported in six individuals a clinical condition almost indistinguishable from PLOD1-kyphoscoliotic Ehlers-Danlos syndrome (PLOD1-kEDS), caused by biallelic mutations in FKBP14, and characterized by progressive kyphoscoliosis, myopathy, and hearing loss in addition to connective tissue abnormalities such as joint hypermobility and hyperelastic skin. FKBP14 is an ER-resident protein belonging to the family of FK506-binding peptidyl-prolyl cis-trans isomerases (PPIases); it catalyzes the folding of type III collagen and interacts with type III, type VI, and type X collagens...

We report about a patient, who in 2005, as a 37-year-old, experienced a sudden, mild to moderate, mid-frequency sensorineural hearing loss in the right ear, along with tinnitus and mild dizziness. MRI of the temporal bone revealed a very small (1 mm) enhancing lesion in the second turn of the right cochlea after injection of contrast medium. Hearing gradually deteriorated, with complete hearing loss in 2015. At this time, an MRI scan showed a lesion completely filling the cochlea.The tumor was removed through a subtotal cochleoectomy...

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OBJECTIVES: Sudden neurosensory deafness is the second debilitating disease in China. In fact, the current treatment is limited to hearing aids, assistive devices and cochlear implants. However, some patients might not be suitable for surgery or even hearing aids. METHODS: Hereby, we report a pediatric case of sudden sensorineural hearing loss accompanied by otalgia and blocked ear sensation. RESULTS: He firstly failed with conventional treatment but later his hearing symptoms were improved with our electro-acupuncture therapy according to pure tone audiometry findings and clinical responses...

SGNs are the primary auditory neurons, and damage or loss of SGNs leads to sensorineural hearing loss. BMP4 is a growth factor that belongs to the TGF-β superfamily and has been shown to play a key role during development, but little is known about its effect on postnatal cochlear SGNs in mice. In this study, we used the P3 Bhlhb5-cre/tdTomato transgenic mouse model and FACS to isolate a pure population of Bhlhb5+ SGNs. We found that BMP4 significantly promoted SGN survival after 7 days of culture. We observed fewer apoptotic cells and decreased expression of pro-apoptotic marker genes after BMP4 treatment...

Impairments of the inner ear result in sensorineural hearing loss and vestibular dysfunction in humans. A large proportion of these disorders are congenital, and involve both auditory and vestibular systems. Therefore, genetic interventions to correct deficits must be administered during early developmental stages. In this study, we evaluated inner ear gene transfer in neonatal mice by canalostomy using an adeno-associated virus serotype 8 (AAV8) vector. AAV8 with the green fluorescence protein (GFP) gene was inoculated into the inner ear of the neonatal mice through the posterior semicircular canal (canalostomy)...

Near-threshold tones (targets) in noise that are preceded by cues of the same frequency or occur with a high probability are detected better than tones of other frequencies that may occur with a lower probability (probes); the better detection of targets than probes defines the attentional filter. We measured attentional filters using a cued probe-signal procedure with a two-interval forced-choice (2IFC) method in normal-hearing subjects (N = 15) and subjects with sensorineural hearing loss (SNHL; N = 14) with a range of hearing levels...

To identify vestibular dysfunction in children after cochlear implant surgery and to study the utility of static posturography in evaluating vestibular function in children. A prospective study was carried out on 25 children between 2 and 7 years of age with sensorineural hearing loss with no overt vestibular dysfunction. All children underwent static posturography using Synapsis Posturographic System (SPS) software (Version 3.0, REV C) using a static platform with foam. The centre of pressure (COP) shift was recorded as statokinesiogram on the software and the mean vestibular, visual and somesthetic scores were obtained...

Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images...

OBJECTIVES: (1) Report a rare case of translabyrinthine resection of a sporadic vestibular schwannoma (VS) and concurrent cochlear implantation (CI). (2) Discuss pre-, intra-, and post-operative considerations in this unique patient population. (3) Describe surgical and audiologic outcomes reported in this population. METHODS: Case report and review of the literature. PubMed search 'Cochlear Implantation'[Mesh] AND ('Neuroma, Acoustic'[Mesh] OR 'VESTIBULAR SCHWANNOMA'[All Fields] OR 'SCHWANNOMA'[All Fields]) limited to humans and English language...

PURPOSE: NDP-related retinopathies are a group of X-linked disorders characterized by degenerative and proliferative changes of the neuroretina, occasionally accompanied with varying degrees of mental retardation and sensorineural hearing loss. NDP is the predominant gene associated with NDP-related retinopathies. The purpose of this study was to report the clinical and genetic findings in three unrelated patients diagnosed with NDP-related retinopathies. METHODS: The patients underwent complete ophthalmic examination followed by genetic analyses...

BACKGROUND: Several studies have demonstrated that children with sensorineural hearing loss (SNHL) may exhibit balance disorders, which can compromise the gait performance of this population. OBJECTIVE: Compare the gait performance of normal hearing (NH) children and those with SNHL, considering the sex and age range of the sample, and analyze gait performance according to degrees of hearing loss and etiological factors in the latter group. METHOD: This is a cross-sectional study that assessed 96 students, 48 NH and 48 with SNHL, aged between 7 and 18 years...

A single reflection is the simplest simulation of reverberation and provides insights into more complex scenarios of listening in rooms. This paper presents an analysis of the effects of a single reflection as its delay and intensity are systematically varied. The changes to the envelope modulations are analyzed using not only the traditional within-auditory-band analysis approach but also an across-band spectro-temporal analysis using cepstral correlation coefficients. The use of an auditory model allowed the extension of the simulations to include sensorineural hearing loss...