Aetiological investigation of sensorineural hearing loss in children.
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ABSTRACT:
SUBMITTER: Wilson C
PROVIDER: S-EPMC1720289 | biostudies-literature | 2005 Mar
REPOSITORIES: biostudies-literature
Publications
Aetiological investigation of sensorineural hearing loss in children.
Wilson C C Roberts A A Stephens D D
Archives of disease in childhood 20050301 3
A survey of aetiological investigations arranged on children with severe/profound bilateral sensorineural hearing impairment by ENT consultants and community paediatricians working in audiology is described. Greater routine use of ECG, urine analysis, and ophthalmological assessment is needed, with a more selective approach to other investigations as recommended by the British Association of Audiological Physicians (BAAP) and the British Association of Community Doctors in Audiology (BACDA). ...[more]
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Project description:This study aims to evaluate the etiology of pediatric sensorineural hearing loss (SNHL). A total of 423 children with SNHL were evaluated, with the focus on the determination of causative genetic and acquired etiologies of uni- and bilateral SNHL in relation to age at diagnosis and severity of the hearing loss. We found that a stepwise diagnostic approach comprising of imaging, genetic, and/or pediatric evaluation identified a cause for SNHL in 67% of the children. The most common causative finding in children with bilateral SNHL was causative gene variants (26%), and in children with unilateral SNHL, a structural anomaly of the temporal bone (27%). The probability of finding an etiologic diagnosis is significantly higher in children under the age of 1 year and children with profound SNHL.Conclusions: With our stepwise diagnostic approach, we found a diagnostic yield of 67%. Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor. The diagnostic yield is associated with the age at detection and severity of hearing loss: the highest proportion of causative abnormalities is found in children with a young age at detection or a profound hearing loss. What is Known: • Congenital sensorineural hearing loss is one of the most common congenital disorders • Determination of the cause is important for adequate management and prognosis and may include radiology, serology, and DNA analysis What is New: • Using a stepwise diagnostic approach, causative abnormalities are found in 67% both in uni- and bilateral SNHL, with the highest diagnostic yield in very young children and those suffering from profound hearing loss • Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor.
| S-EPMC6647487 | biostudies-literature
Project description:IntroductionSensorineural hearing loss (SNHL) has been reported to occur at increased frequency in the pediatric sickle cell disease (SCD) population, likely secondary to ototoxic medication regimens and repeat sickling events that lead to end organ damage. Risk and protective factors of SNHL in this population are not fully characterized. The objective of this study was to describe audiology results in children with SCD and the prevalence and sequelae of SNHL.MethodsA comprehensive clinical database of 2600 pediatric SCD patients treated at 1 institution from 2010-16 was retrospectively reviewed to identify all patients who were referred for audiologic testing. Audiologic test results, patient characteristics, and SCD treatments were reviewed.Results181 SCD children (97 male, 153 HbSS) underwent audiologic testing, with 276 total audiology encounters, ranging 1-9 per patient. Mean age at first audiogram was 8.9 ± 5.2 years. 29.8% had prior cerebrovascular infarct and an additional 25.4% had prior abnormal transcranial Doppler screens documented at time of first audiogram. Overall, 13.3% had documented hearing loss, with 6.6% SNHL. Mean pure tone average (PTA) among patients with SNHL ranged from mild to profound hearing loss (Right: 43.3 ± 28.9, Left: 40.8 ± 29.7), sloping to more severe hearing loss at higher frequencies.ConclusionsHearing loss was identified in a significant subset of children with SCD and the hearing loss ranged from normal to profound. Though the overall prevalence of SNHL in SCD patients was low, baseline audiology screening should be considered.
| S-EPMC6443092 | biostudies-literature
Project description:OBJECTIVE:This study aimed to investigate the presence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in patients presenting with only sudden sensorineural hearing loss (SSHNL) during the COVID-19 pandemic. METHODS:The study included five male patients who presented with the sole complaint of unilateral SSNHL to the otolaryngology outpatient clinic between 03-12 April 2020. The patients were referred to the infectious diseases clinic to be evaluated for SARS-CoV-2 by real time polymerase chain reaction (RT-PCR) testing. RESULTS:RT-PCR testing for SARS-CoV-2 was positive in one of the patients and negative in the other four patients. A positive response to COVID-19-specific treatment in the SARS-CoV-2 positive SSNHL patient was noted. CONCLUSION:It should be remembered that non-specific symptoms such as SSNHL could be the only sign with which to recognize a COVID-19 case. Awareness of such a non-specific presentation of COVID-19 patients is crucial during this pandemic period for preventing infectious spread through isolation and early initiation of COVID-19 targeted treatment.
| S-EPMC7289736 | biostudies-literature
Project description:IntroductionTo compare inpatient treated patients with idiopathic (ISSNHL) and non-idiopathic sudden sensorineural hearing loss (NISSNHL) regarding frequency, hearing loss, treatment and outcome.MethodsAll 574 inpatient patients (51% male, median age: 60 years) with ISSNHL and NISSNHL, who were treated in federal state Thuringia in 2011 and 2012, were included retrospectively. Univariate and multivariate statistical analyses were performed.ResultsISSNHL was diagnosed in 490 patients (85%), NISSNHL in 84 patients (15%). 49% of these cases had hearing loss due to acute otitis media, 37% through varicella-zoster infection or Lyme disease, 10% through Menière disease and 7% due to other reasons. Patients with ISSNHL and NISSNHL showed no difference between age, gender, side of hearing loss, presence of tinnitus or vertigo and their comorbidities. 45% of patients with ISSNHL and 62% with NISSNHL had an outpatient treatment prior to inpatient treatment (p < 0.001). The mean interval between onset of hearing loss to inpatient treatment was shorter in ISSNHL (7.7 days) than in NISSNHL (8.9 days; p = 0.02). The initial hearing loss of the three most affected frequencies in pure-tone average (3PTAmax) scaled 72.9 dBHL ± 31.3 dBHL in ISSNHL and 67.4 dBHL ± 30.5 dBHL in NISSNHL. In the case of acute otitis media, 3PTAmax (59.7 dBHL ± 24.6 dBHL) was lower than in the case of varicella-zoster infection or Lyme disease (80.11 dBHL ± 34.19 dBHL; p = 0.015). Mean absolute hearing gain (Δ3PTAmaxabs) was 8.1 dB ± 18.8 dB in patients with ISSNHL, and not different in NISSNHL patients with 10.2 dB ± 17.6 dB. A Δ3PTAmaxabs ≥ 10 dB was reached in 34.3% of the patients with ISSNHL and to a significantly higher rate of 48.8% in NISSNHL patients (p = 0.011).ConclusionsISSNHL and NISSNHL show no relevant baseline differences. ISSNHL tends to have a higher initial hearing loss. NISSHNL shows a better outcome than ISSNHL.
| S-EPMC8794920 | biostudies-literature
Project description:To critically review and evaluate the proposed mechanisms and documented results of the therapeutics currently in active clinical drug trials for the treatment of sensorineural hearing loss.US National Institutes of Health (NIH) Clinical Trials registry, MEDLINE/PubMed.A review of the NIH Clinical Trials registry identified candidate hearing loss therapies, and supporting publications were acquired from MEDLINE/PubMed. Proof-of-concept, therapeutic mechanisms, and clinical outcomes were critically appraised.Twenty-two active clinical drug trials registered in the United States were identified, and six potentially therapeutic molecules were reviewed. Of the six molecules reviewed, four comprised mechanisms pertaining to mitigating oxidative stress pathways that presumably lead to inner ear cell death. One remaining therapy sought to manipulate the cell death cascade, and the last remaining therapy was a novel cell replacement therapy approach to introduce a transcription factor that promotes hair cell regeneration.A common theme in recent clinical trials registered in the United States appears to be the targeting of cell death pathways and influence of oxidant stressors on cochlear sensory neuroepithelium. In addition, a virus-delivered cell replacement therapy would be the first of its kind should it prove safe and efficacious. Significant challenges for bringing these bench-to-bedside therapies to market remain. It is never assured that results in non-human animal models translate to effective therapies in the setting of human biology. Moreover, as additional processes are described in association with hearing loss, such as an immune response and loss of synaptic contacts, additional pathways for targeting become available.
| S-EPMC5465007 | biostudies-other
Project description:Profound sensorineural hearing loss (SNHL) is an auditory disability associated with auditory and cognitive dysfunction. Due to distinct pathogenesis, some associated structural and functional changes within the brain have been investigated in previous studies, but whole-brain structural alterations are incompletely understood. We extended the exploration of neuroanatomic differences in whole-brain structure in children with profound SNHL who are primarily users of Chinese sign language (CSL). We employed surface-based morphometry (SBM) and subcortical analyses. T1-weighted magnetic resonance images of 26 children with profound SNHL and 27 age- and sex-matched children with normal hearing were analyzed. Compared with the normal control (NC) group, children with profound SNHL showed diverse structural changes in surface-based and subcortical analyses, including decreased cortical thickness in the left postcentral gyrus, superior parietal lobule, paracentral lobule, precuneus, the right transverse temporal gyri, and the middle temporal gyrus; a noticeable increase in the Local Gyrification Index (LGI) in the left precuneus and superior parietal lobule; and diverse changes in gray-matter volume (GMV) in different brain regions. Surface-based vertex analyses revealed regional contractions in the right thalamus, putamen, pallidum, and the brainstem of children with profound SNHL when compared with those in the NC group. Volumetric analyses showed decreased volumes of the right thalamus and pallidum in children with profound SNHL. Our data suggest that children with profound SNHL are associated with diffuse cerebral dysfunction to cortical and subcortical nuclei, and revealed neuroplastic reorganization in the precuneus, superior parietal lobule, and temporal gyrus. Our study provides robust evidence for changes in connectivity and structure in the brain associated with hearing loss.
| S-EPMC7756106 | biostudies-literature
Project description:Hearing loss is the most common sensory impairment in humans and currently disables 466 million people across the world. Congenital deafness affects at least 1 in 500 newborns, and over 50% are hereditary in nature. To date, existing pharmacologic therapies for genetic and acquired etiologies of deafness are severely limited. With the advent of modern sequencing technologies, there is a vast compendium of growing genetic alterations that underlie human hearing loss, which can be targeted by therapeutics such as gene therapy. Recently, there has been tremendous progress in the development of gene therapy vectors to treat sensorineural hearing loss (SNHL) in animal models in vivo. Nevertheless, significant hurdles remain before such technologies can be translated toward clinical use. These include addressing the blood-labyrinth barrier, engineering more specific and effective delivery vehicles, improving surgical access, and validating novel targets. In this review, we both highlight recent progress and outline challenges associated with in vivo gene therapy for human SNHL.
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Project description: Not available
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Project description:Patients with sensorineural hearing loss (SNHL) tend to show language delay, executive functioning deficits, and visual cognitive impairment, even after intervention with hearing amplification and cochlear implants, which suggest altered brain structures and functions in SNHL patients. In this study, we investigated structural brain MRI in 30 children with SNHL (18 mild to moderate [M-M] SNHL and 12 moderately severe to profound [M-P] SNHL) by comparing gender- and age-matched normal controls (NC). Region-based analyses did not show statistically significant differences in volumes of the cerebrum, basal ganglia, cerebellum, and the ventricles between SNHL and NC. On surface-based analyses, the global and lobar cortical surface area, thickness, and volumes were not statistically significantly different between SNHL and NC participants. Regional surface areas, cortical thicknesses, and cortical volumes were statistically significantly smaller in M-P SNHL compared to NC in the left middle occipital cortex, and left inferior occipital cortex after a correction for multiple comparisons using random field theory (p?<?0.02). These regions were identified as areas known to be related to high level visual cognition including the human middle temporal area, lateral occipital area, occipital face area, and V8. The observed regional decreased thickness in M-P SNHL may be associated with dysfunctions of visual cognition in SNHL detectable in a clinical setting.
| S-EPMC6698225 | biostudies-literature
Project description:Background and Introduction: Idiopathic sudden sensorineural hearing loss (ISSNHL) is characterized by rapid onset, typically unilateral presentation, and variable recovery. This case-control observational study aimed to improve patient counseling by objectively characterizing long-term hearing loss progression following ISSNHL, using sequential audiometry in the largest-to-date cohort of patients with ISSNHL. Methods: Patients diagnosed with ISSNHL at a tertiary referral hospital from 1994 through 2018 with sequential audiometry were studied. Case controls with sensorineural hearing loss (SNHL) were matched by age, sex, baseline hearing status, and frequency of sequential audiometry. Hearing loss progression was quantified using Kaplan-Meier (K-M) analysis to account for variable follow-up duration. A subgroup analysis was performed by age, sex, preexisting comorbidities, ISSNHL-associated symptoms, ISSNHL treatment, and degree of post-ISSNHL hearing recovery. Results: A total of 660 patients were identified with ISSNHL. In patients with post-ISSNHL recovery to good hearing [pure tone average (PTA) <30 dB and word recognition score (WRS) > 70%], median time to progression to non-serviceable (PTA > 50 dB or WRS <50%) SNHL was 16.4 years. In patients with incomplete post-ISSNHL hearing recovery, contralateral ears were also at significantly higher risk of SNHL progression over the following 12-year period. Male sex was associated with increased risk of SNHL progression [odds ratio (OR) 3.45 male vs. female] at 5-year follow up. No other subgroup factors influenced the likelihood of SNHL progression. Discussion and Conclusion: Patients should be counseled on continued risk to long-term hearing after stabilization of hearing post-ISSNHL, with particular emphasis on greater risk to the contralateral ear in those with incomplete ipsilateral recovery.
| S-EPMC8693444 | biostudies-literature