Sensorineural hearing loss

Objective: To investigate the correlation between the proliferation inhibition effect of glucocorticoid (GC) on peripheral blood mononuclear cell (PBMC) and the pure tone average (PTA) improvement in SSNHL patients. Methods: Sixty inpatients with SSNHL were included from July 2013 to October 2015 in Nanjing Drum Tower Hospital, Medical School of Nanjing University. Peripheral venous blood was collected before receiving treatment, then the PBMC was isolated for GC proliferation inhibition. PBMCs of each patient were cultivated into 4 groups: Group A: PBMCs+ Medium; Group B: PBMCs+ Medium+ lipopolysaccharide (LPS, 1 μmol/L); Group C: PBMCs+ Medium+ LPS+ Dexamethasone; Group D: Medium...

Bi-allelic variations in the gap junction protein beta-2 (GJB2) gene cause up to 50% of cases of newborn hearing loss. Heterozygous pathogenic GJB2 variations are also fivefold overrepresented in idiopathic patient groups compared to the normal-hearing population. Whether hearing loss in this group is due to unidentified additional variations within GJB2 or variations in other deafness genes is unknown in most cases. Whole-exome sequencing offers an effective approach in the search for causative variations in patients with Mendelian diseases...

Sudden sensorineural hearing loss is an otologic emergency. Many etiological factors can lead to this pathology. Honey bee (Apis mellifera) sting may lead to local and systemic reactions due to sensitization of the patient. In this paper we described a sudden sensorineural hearing loss occurred after honey bee sting.

We reported the recovery of sensorineural hearing loss and tinnitus in a 22-year-old man after complete removal of intracranial portion of jugular foramen schwannoma via the retrosigmoid approach. The aim of this case report was to present the excision of a large jugular foramen schwannoma via the retrosigmoid approach and to describe the improvement of sensorineural hearing loss related to arachnoid inflammations due to chronic arachnoiditis after suboccipital craniectomy. The recovery of sensorineural hearing loss and tinnitus after release of arachnoid adhesions may indicate the clinical significance of these adhesions or arachnoiditis, which should also be considered and investigated in the etiology of other neurotological diseases...

Intralabyrinthine schwannoma (ILS) is a rare benign tumor that affects the ends of cochlear and vestibular nerves. In a majority of the cases, it occurs with unilateral progressive sensorineural hearing loss. Less frequent symptoms include tinnitus, imbalance, vertigo, or fullness. The advent of magnetic resonance imaging allows early diagnosis and enables an appropriate therapeutic protocol. This report describes a case of intravestibular schwannoma, with fluctuating hearing loss and intractable vertigo, treated with intratympanic gentamicin...

OBJECTIVE: In this study, we evaluated the prognostic value of the Dizziness Handicap Inventory (DHI) and the Tinnitus Handicap Inventory (THI) in patients with unilateral sudden sensorineural hearing loss (SSNHL). MATERIALS AND METHODS: In total, 101 patients with unilateral SSNHL (44 women, 57 men), who were admitted and treated at our institution between December 2012 and June 2014, were included in the study. All patients completed the questionnaires for DHI and THI during their admission and were treated with bed rest and oral methylprednisolone (1 mg/kg, which was eventually tapered)...

CONTEXT: Deep band modulation (DBM) improves speech perception in individuals with learning disability and older adults, who had temporal impairment in them. However, it is unclear on perception of DBM phrases at quiet and noise conditions in individuals with auditory neuropathy spectrum disorder (ANSD) and sensorineural hearing loss (SNHL), as these individuals suffer from temporal impairment. AIM: The aim is to study the effect of DBM and noise on phrase perception in individuals with normal hearing, SNHL, and ANSD...

In this study, we describe a Japanese family with progressive hearing loss and macrothrombocytopenia. Using next-generation and Sanger sequencing analyses, we identified a heterozygous variant in exon 27 of the DIAPH1 gene (NM_005219), c.3637C>T, p.R1213X. All patients in the family had sensorineural hearing loss and macrothrombocytopenia. None of the patients exhibited a tendency to bleed. No pathogenic variants were found in the MYH9 gene. Hearing loss began with high-frequency loss during early childhood and progressed to severe hearing loss involving all frequencies...

The most common disease-causing mitochondrial DNA (mtDNA) mutation in mitochondrial encephalomyopathy (ME) with lactic acidosis and stroke-like episodes (MELAS) is m.3243A>G. In the future, the incidence of patients with cerebral infarction and diabetes mellitus is expected to increase tremendously. Additionally, the A3243G mutation typical of diabetes is estimated to be present in approximately 2% of all diabetes patients, which suggests that the potential disease population with a mitochondrial disorder is greater than previously thought, and there may have been many cases among the elderly that were misdiagnosed...

OBJECTIVE: Rheumatoid arthritis is thought to induce conductive hearing loss and/or sensorineural hearing loss. This study evaluated the function of the middle ear and cochlea, and the related factors. METHODS: Pure tone audiometry, speech reception thresholds, speech discrimination scores, tympanometry, acoustic reflexes, and distortion product otoacoustic emissions were assessed in rheumatoid arthritis patients and healthy volunteers. RESULTS: Pure tone audiometry results revealed a higher bone conduction threshold in the rheumatoid arthritis group, but there was no significant difference when evaluated according to the sensorineural hearing loss definition...

OBJECTIVE: To evaluate the presence of cardiovascular risk factors and recovery of idiopathic sudden sensorineural hearing loss in hospitalised patients. METHODS: A single-centre retrospective study of 80 patients hospitalised for idiopathic sudden sensorineural hearing loss was conducted over a 6-year period. Mean pure tone hearing thresholds were assessed by pure tone audiometry. RESULTS: Twenty-three of 80 patients (28.75 per cent) initially had no cardiovascular risk factors...

OBJECTIVE: The study investigated improvements in maximal speech intelligibility after Vibrant Soundbridge (VSB) implantation and analyzed the effect of the hearing loss pattern on maximal speech intelligibility represented by a phonetically balanced word score (PBmax). The effect of middle ear implants on PBmax has not been evaluated yet. STUDY DESIGN: Study. SETTING: Tertiary academic medical center. PATIENTS/INTERVENTIONS: Sixty patients who underwent VSB from December 2011 to January 2016 were retrospectively reviewed...

OBJECTIVES: The purpose of this laboratory-based study was to compare the efficacy of two hearing aid fittings with and without nonlinear frequency compression, implemented within commercially available hearing aids. Previous research regarding the utility of nonlinear frequency compression has revealed conflicting results for speech recognition, marked by high individual variability. Individual differences in auditory function and cognitive abilities, specifically hearing loss slope and working memory, may contribute to aided performance...

OBJECTIVE: Autosomal Dominant Non-Syndromic Hearing Loss (ADNSHL) is extremely heterogeneous in nature. More than 60 loci with 30 different genes have been identified linked to ADNSHL. Mutation in KCNQ4 is considered as one of the most common causative factor responsible for ADNSHL. No study focused on the genetic alteration of KCNQ4 gene among hearing loss patients in India. The present study for the first time was carried out to determine the mutation spectrum of KCNQ4 gene in ADNSHL patients of West Bengal state, India...

We report the first sporadic case of nonsyndromic autosomal dominant hearing loss (DFNA11). The patient was a 5-year-old boy with moderate bilateral hearing loss. Targeted next-generation sequencing analysis of patient DNA identified a known heterozygous DFNA11 mutation, c.689C > T, in MYO7A, encoding p.Ala230Val. The mutation was not detected in the parents of the patient and is considered to be de novo. This mutation is identical to the one reported previously in an Italian family. Accumulation of mutation data increases the feasibility of identifying autosomal dominant mutations in sporadic sensorineural hearing loss...

OBJECTIVE: The purpose of this study is to highlight the relationship between obstructive hydrocephalus, changes in intracranial pressure, and sensorineural hearing loss. METHODS: A case of a 10-month old infant with sensorineural hearing loss secondary to obstructive hydrocephalus is reported. A literature review, with a focus on sensorineural hearing loss in the setting of changes in intracranial pressure, was performed. RESULTS: The authors report the case of a 10-month old infant with metopic and bicoronal craniosynostosis who presented with bilateral moderately severe sensorineural hearing loss after failing newborn hearing screening...

OBJECTIVE: Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. METHODS: Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database...

OBJECTIVE: Autosomal dominant non-syndromic low-frequency sensorineural hearing loss (LFSNHL) DFNA6/14/38 is an uncommon type of hearing loss that classically affects low frequencies of 2000 Hz and below, demonstrating an ascending configuration. The current study aimed to investigate the cause of LFSNHL in a five-generation Chinese family. METHODS: The phenotype of the Chinese family was characterized using audiologic testing and pedigree analysis. The combined approach of array screening and whole-exome sequencing was used to identify the disease-causing gene in this family...

OBJECTIVE: The aim of this study is to determine the prevalence of jugular bulb-vestibular aqueduct dehiscence (JBVAD) in patients undergoing temporal bone multidetector computed tomography (MDCT) and assess the relationship between JBVAD and hearing loss with the findings of audiometry. METHODS: About 1503 temporal bone MDCT scans were evaluated for the prevalence of JBVAD. Correlation of the imaging findings and audiometric data was performed. Patients were divided into two groups, those with and those without hearing loss, and were statistically compared...

OBJECTIVE: Evaluate the effects of therapy with citalopram on the central auditory processing in the elderly measured by central auditory tests. STUDY DESIGN: Prospective, randomized, double-blind, placebo-controlled study. SETTING: Tertiary referral center. PATIENTS: Thirty-nine patients older than 60 years with normal hearing thresholds or symmetrical sensorineural hearing loss up to 70 dBHL, word-recognition score equal to or better than 70%, and diagnosed with central auditory processing disorders completed the study...