Onset Of Sensorineural Hearing Loss Research Articles

Sudden Onset Sensorineural Hearing Loss Post Oral Contraception: A Case Report

Sudden onset hearing loss (SOHL) causes great concern to patients. It can be sensorineural or conductive hearing loss. Sudden onset sensorineural hearing loss (SSNHL) is considered an otological emergency. The incidence of SSNHL is 5-20 per 100,00. Although individuals of all ages can be affected, the peak incidence is between the fifth and sixth decade of life. Nearly all cases are unilateral; less than 2% of patients have bilateral involvement (Acipayam, Kocak and Elbistanli). This paper describes a case of a 43 years old female presented with sudden onset unilateral sensorineural hearing loss (SNHL) after oral contraceptive pill (OCP) use. The relation of OCP use with different pathophysiological theories of SSNHL is discussed.

Study of clinical profile of patients with sudden onset sensorineural hearing loss at a tertiary hospital

Study of clinical profile of patients with sudden onset sensorineural hearing loss at a tertiary hospital

Temporospatial aggregation of three individuals with idiopathic sudden onset low-tone sensorineural hearing loss

Objective To report temporospatial aggregation of low-tone sudden onset sensorineural hearing loss (SSNHL) and provide plausible aetiological explanations. Design Retrospective analysis of records of patients, including medical notes, clinic letters, audiology reports, blood test and imaging results after occurrence of low-tone SSNHL. Study sample Three patients working in the same department and presenting with low-frequency SSNHL between March and July 2019. Results There was complete symptom resolution in two of the three cases, one treated with oral and one with intratympanic steroid administration, whereas low-tone SSNHL persisted in the third case. Probability of events occurring randomly was calculated and it was suggested that the three cases were highly unlikely to be independent events. Conclusions This is the first report of temporospatial aggregation of acute low-tone idiopathic SSNHL. Our findings support that an environmental or transmissible agent could underlie aetiology in at least a proportion of such cases.

Cochlear Implantation From the Perspective of Genetic Background.

ABSTRACTWhile cochlear implantation (CI) technology has greatly improved over the past 40 years, one aspect of CI that continues to pose difficulties is the variability of outcomes due to numerous factors involved in postimplantation performance. The electric acoustic stimulation (EAS) system has expanded indications for CI to include patients with residual hearing, and is currently becoming a standard therapy for these patients. Genetic disorders are known to be the most common cause of congenital/early‐onset sensorineural hearing loss, and are also involved in a considerable proportion of cases of late‐onset hearing loss. There has been a great deal of progress in the identification of deafness genes over the last two decades. Currently, more than 100 genes have been reported to be associated with non‐syndromic hearing loss. Patients possessing a variety of deafness gene mutations have achieved satisfactory auditory performance after CI/EAS, suggesting that identification of the genetic background facilitates prediction of post‐CI/EAS performance. When the intra‐cochlear etiology is associated with a specific genetic background, there is a potential for good CI performance. Thus, it is essential to determine which region of the cochlea is affected by identifying the responsible genes. This review summarizes the genetic background of the patients receiving CI/EAS, and introduces detailed clinical data and CI/EAS outcomes in representative examples. Anat Rec, 303:563–593, 2020. © 2020 The Authors. The Anatomical Record published by Wiley Periodicals, Inc. on behalf of American Association of Anatomists.

Algorithm of hearing monitoring of children with congenital heart disease

Congenital heart disease (CHD) is one of the most common types of birth defect. Often newborn hearing screening is not performed for infants with congenital heart disease because of serious health problems at birth. Hearing loss in children with CHD may have late onset due to different stages of CHD treatment. The monitoring of the auditory function is required for early identification of hearing loss in children with CHD, however algorithm of follow-up assessment is not currently defined.
 Objective – estimation of prevalence, types and time of hearing loss onset in children with congenital heart disease and development a follow-up assessment for CHD children.
 Results. 148 children with congenital heart disease have been evaluated with auditory brainstem response testing, otoacoustic emissions, impedancemetry, behavioral hearing tests. The patients ranged in age from 7 days to 6 years. 55 children had hearing loss: 28 children with conductive hearing loss and 27 children with sensorineural hearing loss, 2 of them with auditory neuropathy. 6 children had late onset sensorineural hearing loss. The age of delayed hearing loss identification ranged from 4 months to 5 years.
 Conclusion. Children with CHD require long-term monitoring of the auditory function; an algorithm of follow-up assessment for children with congenital heart disease is proposed. Audiological assessment in children with CHD should include auditory brainstem response registration for auditory neuropathy identification.

Cortical Visual Impairment in Congenital Cytomegalovirus Infection.

To describe the presentation, evolution, and long-term outcome of cortical visual impairment (CVI) in patients with symptomatic congenital cytomegalovirus (CMV) infection, and to identify risk factors for the development of CVI in patients with symptomatic congenital CMV. Retrospective subanalysis of a long-term prospective cohort study with data gathered from 1982 to 2013. Eleven of 77 (14.3%) patients with symptomatic CMV, 0 of 109 with asymptomatic CMV, and 51 control patients had CVI. Overall, patients with symptomatic CMV had worse vision than patients with asymptomatic CMV, who in turn had worse vision than control patients. Microcephaly, intracranial calcification, dilatation of ventricles, encephalomalacia, seizure at birth, optic atrophy, chorioretinitis/retinal scars, strabismus, and neonatal onset of sensorineural hearing loss were risk factors associated with CVI. CVI may result from symptomatic congenital CMV infection. The relationship of CVI and its risk factors in patients with CMV suggests the potential to predict the development of CVI through predictive modeling in future research. Early screening of CVI in children born with symptomatic congenital CMV can facilitate educational, social, and developmental interventions. [J Pediatr Ophthalmol Strabismus. 2019;56(3):194-202.].

Possible involvement of cochlear macrophage in the onset of sensorineural hearing loss

Possible involvement of cochlear macrophage in the onset of sensorineural hearing loss

Convalescence of auditory brainstem response following idiopathic sudden onset sensorineural hearing loss

Convalescence of auditory brainstem response following idiopathic sudden onset sensorineural hearing loss

Mitigating noise-induced hearing loss after blast injury

Introduction Whilst hearing injuries are not life threatening they may have a profound impact on the victim’s ability to understand and interact with the world around them. Noise-induced hearing loss is a common occupational injury and hearing impairment as a consequence of noise and blast exposure remains the most common injury in both war and peace for military personnel. Health and Safety legislation has made an impact and in the future innovative approaches to mitigate against acoustic injury sustained in the work place will be fundamental. For the Armed Forces, noise exposure during conflict is unpredictable. Furthermore, recent events in the UK and elsewhere have highlighted the potential civilian impact of blast injuries on hearing in the acute setting. No well-established protocol for the management of acute, blast-induced hearing injury currently exists. Methods Narrative review is supported by electronic literature searches of PubMed, Embase and the Cochrane Library. Synthesis of published literature and production of flow charts for the acute setting are part of the Emergency Preparedness, Resilience and Response programme. Results Whilst there is a lack of high-quality randomised controlled trials, there are a number of studies that may inform our choice of acute management. Animal studies of acute acoustic trauma have shown the potential protective effects of corticosteroids. Human data may be extrapolated from sudden onset sensorineural hearing loss where again there is evidence for the use of corticosteroids. Less certainty exists around the use of other treatments including antioxidants. Intratympanic administration of corticosteroids may be superior to oral administration, particularly in the salvage setting. No evidence exists specifically pertaining to the paediatric population. Conclusion Prompt identification of any hearing deficit followed by administration of glucocorticoids either orally or via intratympanic preparations is the mainstay of management. Further research is needed to identify the optimum acute management.

Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic Review.

Background and Purpose: Cognitive impairment has been observed in patients with bilateral vestibular loss (BVL) and in patients with sensorineural hearing loss (SNHL). DFNA9 is an autosomal dominant disorder that causes a combination of both sensory deficits by the 3rd to 5th decade. We therefore hypothesize a combined detrimental effect on cognition. The aim of this systematic review was to identify studies related to DFNA9 in general and its relationship with cognitive impairment more specifically.Materials and Methods: Several databases including Medline, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, ISI Web of Knowledge, and Web of Science were searched to accumulate information about DFNA9-mutations, including phenotype, genotype, pathophysiology, quality of life (QOL), and imaging in general and cognitive function more specifically. A qualitative analysis was performed on the 55 articles that qualified.Results: The clinical features of DFNA9 are different along the 24 COCH mutations, described up to now. Vestibular symptoms generally present themselves a few years after SNHL onset in mutations associated with the vWFA-domain although they can precede SNHL onset in other mutations associated with the LCCL-domain. QoL has not been studied extensively in DFNA9, although scarce work is available on the positive impact of cochlear implantation to rehabilitate hearing. No studies were found evaluating cognition in DFNA9 patients.Conclusion: Although cognitive impairment has been demonstrated in patients with hearing loss as well as in patients with BVL, no studies have been reported on the combination of both sensory deficits, such as in DFNA9. Further research is warranted to correlate otovestibular status with cognition.

Late onset nonsyndromic hearing loss in a Dongxiang Chinese family is associated with the 593T > C variant in the mitochondrial tRNAPhe gene

We report here the clinical, genetic, molecular and biochemical characterization of a four-generation Dongxiang Chinese pedigree with suggestively maternally transmitted non-syndromic hearing loss. Five of 10 matrilineal relatives exhibited variable severity and age at onset of sensorineural hearing loss. The average ages at onset of hearing loss in matrilineal relatives of this family were 29years. Molecular analysis of their mitochondrial genomes identified the tRNAPhe 593T>C variant belonging to Asian haplogroup G2a2a. The m.593T>C variant resided at the position 17 of DHU-loop, where the position is important for the structure and function of tRNA. It was anticipated that the m.593T>C variant altered the structure and function of tRNAPhe. By using lymphoblastoid cell lines derived from the Chinese family, we showed a 46% decreases in the steady-state level of tRNAPhe in mutant cell lines. Western blotting analysis showed ∼35% reduction in the levels of mitochondrial translation in mutant cell lines carrying the m.593T>C variant. Impaired mitochondrial translation is apparently a primary contributor to the marked reduction in the rate of respiratory capacity. The respiratory deficiency lowed mitochondrial ATP production in the mutant cell lines. These data provide the evidence that mitochondrial dysfunctions caused by the m.593T>C variant lead to late-onset nonsyndromic hearing loss. Thus, our findings may provide the new insights into the understanding of pathophysiology and valuable information for management and treatment of maternally inherited hearing loss.

Mitochondrial mutations in maternally inherited hearing loss

BackgroundAlthough the mitochondrial DNA (mtDNA) mutations m.1555A > G and m.3243A > G are the primary causes of maternally inherited sensorineural hearing loss (SNHL), several other mtDNA mutations are also reported to be associated with SNHL.MethodsScreening of m.1555A > G and m.3243A > G mutations was performed for 145 probands. Nine probands fulfilled the following criteria: 1) bilateral and symmetric SNHL, 2) ≥ 4 family members with SNHL with a maternal trait of inheritance in ≥ 2 generations, 3) onset of SNHL before the age of 40 years, 4) high-frequency SNHL, and 5) no record of environmental factors related to SNHL. Sequencing of additional mtDNA regions was performed for five subjects meeting the clinical criteria, but the screening results were negative.ResultsAmong the nine cases meeting the five clinical criteria detailed above, three had the m.1555A > G mutation in MTRNR1, one had a m.3243A > G mutation in MTTL1, and one case had a m.7511T > C mutation in MTTS1. In the family with the m.7511T > C mutation, penetrance of SNHL among maternally related subjects was 9/17 (53%). The age at onset varied from birth (congenital) to adulthood. Hearing levels varied from normal to moderately impaired, unlike previously reported subjects with this mutation, where some maternal family members presented with profound SNHL. Family members with the m.7511T > C mutation and SNHL did not exhibit any specific clinical characteristics distinct from those of other individuals with SNHL and different mtDNA mutations. Among the 136 probands who did not meet the criteria detailed above, one case had the m.1555A > G mutation, and three cases had the m.3243A > G mutation.ConclusionsSince five of nine probands with the clinical criteria used in this study had mtDNA mutations, these criteria may be helpful for identification of candidate patients likely to have mtDNA mutations.

A Targeted Approach for Congenital Cytomegalovirus Screening Within Newborn Hearing Screening.

Congenital cytomegalovirus (cCMV) infection remains a leading cause of childhood hearing loss. Currently universal CMV screening at birth does not exist in the United States. An alternative approach could be testing infants who do not pass their newborn hearing screening (NHS) for cCMV. This study was undertaken to evaluate whether a targeted approach will identify infants with CMV-related sensorineural hearing loss (SNHL). Infants born at 7 US medical centers received NHS and were also screened for cCMV while in the newborn nursery. Infants who tested positive for CMV received further diagnostic audiologic evaluations to identify or confirm hearing loss. Between 2007 and 2012, 99 945 newborns were screened for both hearing impairment and cCMV. Overall, 7.0% of CMV-positive infants did not pass NHS compared with 0.9% of CMV-negative infants (P < .0001). Among the cCMV infants who failed NHS, diagnostic testing confirmed that 65% had SNHL. In addition, 3.6% of CMV-infected infants who passed their NHS had SNHL confirmed by further evaluation during early infancy. NHS in this cohort identified 57% of all CMV-related SNHL that occurred in the neonatal period. A targeted CMV approach that tests newborns who fail their NHS identified the majority of infants with CMV-related SNHL at birth. However, 43% of the infants with CMV-related SNHL in the neonatal period and cCMV infants who are at risk for late onset SNHL were not identified by NHS.

Comparison - effectiveness of oral steroid versus intratympanic Dexamethasone for sudden onset sensorineural hearing loss

BackgroundSudden sensory neural hearing loss is an ENT emergency, where 90% are idiopathic. Steroids are the mainstay of treatment, but the best mode of delivery need to be determined. AimThis study was carried out to determine the effectiveness of oral steroid versus intratympanic Dexamethasone for sudden onset sensory neural hearing loss MethodProspective, interventional, randomised, comparative study was carried out at the ENT unit at the National Hospital of Sri Lanka from September 2013 to August 2015. Results7 patients who met the inclusion criteria were randomly allocated in to two groups, and were treated with oral Prednisolone and Intratympanic Dexamethasone, which included 23 males and 14 females (n=37). Within the present study, 85% (17/20) patients who had oral Prednisolone and 94.10% (16/17) patients who had intratympanic Dexamethasone clinically improved after treatment. Overall improvement following ITD is significantly higher 94.10% (P&lt;0.01). There were no adverse effects reported. ConclusionIntratympanic dexamethasone (ITD) is an effective treatment modality for patients presenting with sudden onset sensorineural hearing loss, compared to high dose oral steroids. Hearing improvement in low and mid frequencies are 100% compared to high frequencies. Early diagnosis and treatment, gave best results.

Hearing Loss in Patients Who Received Cranial Radiation Therapy for Childhood Cancer.

Patients treated with cranial radiation therapy (RT) are at risk for sensorineural hearing loss (SNHL). Although SNHL is often characterized as a delayed consequence of anticancer therapy, longitudinal reports of SNHL in childhood cancer survivors treated with contemporary RT are limited. We report the incidence, onset, severity, and long-term trajectory of SNHL among children receiving RT. Potential risk factors for SNHL were also identified. Serial audiologic testing was conducted on 235 pediatric patients who were treated with conformal or intensity-modulated RT as part of an institutional phase II trial for localized primary brain tumors, including craniopharyngioma, ependymoma, and juvenile pilocytic astrocytoma. All but one patient had measurable cochlear radiation dose (CRD) greater than 0 Gy. The median follow-up from RT initiation to latest audiogram was 9 years with a median of 11 post-RT audiograms per patient. Audiograms were classified by the Chang Ototoxicity Grading Scale. Progression was defined by an increase in Chang grade from SNHL onset to the most recent evaluation. At last evaluation, SNHL was prevalent in 14% of patients: 2.1% had mild and 11.9% had significant SNHL requiring hearing aids. Median time from RT to SNHL onset was 3.6 years (range, 0.4 to 13.2 years). Among 29 patients with follow-up evaluations after SNHL onset, 65.5% experienced continued decline in hearing sensitivity in either ear and 34.5% had no change. Younger age at RT initiation (hazard ratio [HR], 2.32; 95% CI, 1.21 to 4.46), higher CRD (HR, 1.07; 95% CI, 1.03 to 1.11), and cerebrospinal fluid shunting (HR, 2.02; 95% CI, 1.07 to 3.78) were associated with SNHL. SNHL is a late effect of RT that likely worsens over time. Long-term audiologic follow-up for a minimum of 10 years post-RT is recommended.

Collagen type IV-related nephropathies in Portugal: pathogenic COL4A5 mutations and clinical characterization of 22 families.

Alport syndrome (AS) is caused by pathogenic mutations in the genes encoding α3, α4 or α5 chains of collagen IV (COL4A3/COL4A4/COL4A5), resulting in hematuria, chronic renal failure (CRF), sensorineural hearing loss (SNHL) and ocular abnormalities. Mutations in the X-linked COL4A5 gene have been identified in 85% of the families (XLAS). In this study, 22 of 60 probands (37%) of unrelated Portuguese families, with clinical diagnosis of AS and no evidence of autosomal inheritance, had pathogenic COL4A5 mutations detected by Sanger sequencing and/or multiplex-ligation probe amplification, of which 12 (57%) are novel. Males had more severe and earlier renal and extrarenal complications, but microscopic hematuria was a constant finding irrespective of gender. Nonsense and splice site mutations, as well as small and large deletions, were associated with younger age of onset of SNHL in males, and with higher risk of CRF and SNHL in females. Pathogenic COL4A3 or COL4A4 mutations were subsequently identified in more than half of the families without a pathogenic mutation in COL4A5. The lower than expected prevalence of XLAS in Portuguese families warrants the use of next-generation sequencing for simultaneous COL4A3/COL4A4/COL4A5 analysis, as first-tier approach to the genetic diagnosis of collagen type IV-related nephropathies.

Bilateral sudden sensorineural hearing loss: review

Unilateral and bilateral sudden sensorineural hearing loss represent different disease entities. The unilateral condition is more common and predominantly idiopathic, and up to 65 per cent of patients spontaneously recover hearing. Conversely, the bilateral condition is rare, mostly associated with serious systemic conditions, and has a higher prevalence of morbidity and mortality. A literature search using the PubMed database was conducted using the MeSH terms 'sudden', 'bilateral' and 'sensorineural hearing loss'. One hundred and three reported cases of bilateral sudden sensorineural hearing loss were identified. The condition is most often associated with toxic, autoimmune, neoplastic and vascular conditions. A younger age of onset, with a bimodal age distribution, was seen for bilateral sudden sensorineural hearing loss, compared with the unilateral condition. Patients with the bilateral condition had more profound hearing loss, with poorer recovery and a 35 per cent mortality rate. Vestibular symptoms were also less common than in the unilateral condition. The presentation of bilateral sudden onset sensorineural hearing loss is a medical emergency requiring thorough and urgent investigation to exclude life-threatening and reversible conditions.

A maternally inherited diabetes and deafness patient with the 12S rRNA m.1555A>G and the ND1 m.3308T>C mutations associated with multiple mitochondrial deletions

Maternally inherited diabetes and deafness (MIDD) is a mitochondrial syndrome characterized by the onset of sensorineural hearing loss and diabetes in adults. Some patients may have other additional clinical features common in mitochondrial disorders such as pigmentary retinopathy, ptosis, cardiomyopathy, myopathy and renal affections. We report a 40-year-old Tunisian patient presenting maternally inherited type 2 diabetes and deafness (MIDD). A molecular genetic analysis was conducted in the patient and his twin sister, but no reported mutations in the tRNALeu(UUR) and tRNAGlu genes were found, especially the two mitochondrial m.3243A>G and the m.14709T>C mutations in muscle and blood leukocytes. The results showed the presence of the mitochondrial NADH deshydrogenase 1 (ND1) homoplasmic m.3308T>C mutation the 2 tested tissues (blood leukocytes and skeletal muscle) of the proband and in the patient’s sister blood leukocytes. In addition, we identified the mitochondrial 12S rRNA m.1555A>G mutation in muscle and blood leukocytes. The Long-range PCR amplification revealed the presence of multiple deletions of the mitochondrial DNA extracted from the patient’s skeletal muscle removing several tRNA and protein-coding genes. Our study reported a Tunisian patient with clinical features of MIDD in whom we detected the 12S rRNA m.1555A>G and the ND1 m.3308T>C mutations with mitochondrial multiple deletions.

ステロイド精神病を呈した急性感音難聴の1例

A 36 y/o female presented with the chief complaint of diarrhea and vomiting which had lasted for four days, and with a family history of suicide. The first general examination showed severe dehydration with hyponatremia. After admission, she was diagnosed as having isolated adrenocorticotropic hormone (ACTH) deficiency and mixed connective tissue disease, and the steroid replacement therapy was started with the dose equivalent to 7.5 mg/day of prednisolone (PSL). Three days later, she had right sensorineural hearing loss (SNHL). She was given 40 mg/day PSL in addition to the steroid replacement therapy. On the next day, she developed a persecutory type of paranoid disorder, and then was given psychiatric medication. After tapering off PSL for SNHL, the delusion began to improve with psychiatric medication. Three weeks after the onset of SNHL, her hearing level had partially recovered. Ten months later, she did not show any psychic instability. A family history of psychosis and the present history of malnutrition and connective tissue disease are risk factors of steroid psychosis. It can develop even with 5 mg PSL if the patient has a risk factor. Careful medical history taking and knowledge about the steroid psychosis will prevent the severe side effects associated with steroid treatment.

難聴を合併したMPO-ANCA関連血管炎患者の3D-FLAIR MR画像評価

Patients with MPO-ANCA related angitis sometimes experience the onset of sonsorineural hearing loss and facial palsy. The main aim of this study was to investigate the relationship between the inflammation in the inner ear and sensorineural hearing loss, or between the inflammation of the facial nerve and facial nerve palsy in patients with MPO-ANCA related angitis. From 2007 to 2011, the inner ear was evaluated in 16 ears from 8 patients with MPO-ANCA related angitis with 3D-FLAIR MRI. In 12 ears of the 16 ears the onset of sensorineural hearing loss was noted, and the signal intensity ratio (SIR) in the cochlea was 0.62 +/- 0.15 on pre-enhancement 3D-FLAIR MRI, and 0.97 +/- 0.5 on post-enhancement. These SIR values were significantly higher than the SIR of non-sensorineural hearing loss patients. The onset of facial palsy was noted in 9 of 16 sides and the facial nerve SIR was 0.83 +/- 0.23 on post-contrast 3D-FLAIR MRI. The SIR in the facial nerve of facial nerve palsy patients was higher than non-facial palsy patients. The conclusion is that sensorineural hearing loss and facial palsy in patients with MPO-ANCA related angitis occurred due to strong inflammation in the cochlea or facial nerve. 3D-FLAIR MRI made this pathology clear.