Sensorineural Deafness In Children Research Articles

Prognosis of 109 cases of sudden sensorineural hearing loss in children

Objective:To analyze the factors influencing the prognosis of sudden sensorineural deafness in children, and to provide theoretical basis for clinical prevention and treatment. Methods:The clinical data of 109 children with sudden deafness admitted to our hospital from 2016 to 2023 were retrospectively analyzed. The children were grouped according to eight related factors, including gender, age, climate, duration of hearing loss, concomitant symptoms, degree of hearing loss, sicken ear, and auditory curve. The chi-square test was used for univariate analysis, and logistic regression was employed to identify factors influencing prognosis. Results:After conventional treatment, 56 cases were ineffective（51.40%）, 30 cases were effective（27.5%）, 13 cases were effective（11.9%）, 10 cases were cured（9.2%）, and the total effective rate was 48.6%. Among concomitant symptoms, children with tinnitus had better treatment results（P<0.05）; In the degree of hearing loss, the effective rate of mild hearing loss was the highest（83.3%）, and the effective rate of very severe hearing loss was the lowest（40.0%）. The prognosis of low frequency decline and high frequency decline were better（P<0.05）; There was no significant correlation between gender, age, climate, duration of hearing loss, sicken ear and prognosis（P>0.05）. Conclusion:The auditory curve and the degree of hearing loss are the factors affecting the prognosis of children with sudden deafness. Additionally, children with tinnitus tend to have a better prognosis.

The Relationship between Prenatal Risk Factors and The Incident of Sensorineural Deafness in Children at Polyclinic ENT- Head and Neck Zainoel Abidin Hospital Banda Aceh *,

Background: Sensorineural deafness is one of congenital disorders that caused by prenatal, natal, and postnatal factors. Sensorineural deafness is a public health problem in the world due to its high prevalence and highly negative impact on child development. Objective: To determine the relation between prenatal risk factors (family history of hearing loss, ototoxic drugs consumption, history of infection, history of Diabetes Mellitus, history of gestational hypertension) with sensorineural hearing loss in children at ENT- Head and Neck Polyclinic Dr. Zainoel Abidin Hospital. Methods: This study was analytic retrospective with cross sectional design. The sampling technique used was simple random sampling. The total samples are 47 children at the ENT-Head and Neck Polyclinic of RSUDZA who has been examined with OAE, BERA and ASSR from January 2020 to December 2021 and diagnosed with sensorineural hearing loss. Data were analyzed using SPSS which bivariate analysis using Chi- square and Fisher’s exact. Results: 47 children who had been diagnosed with sensorineural hearing loss included in the inclusion criteria. The mean age of children in this study was 3.44 years with male predominance of 55.3%. The most severe degree of hearing loss was found with the percentage of weight (93.6%) in right ear and (89.4%) in left ear. A history of maternal infection during pregnancy was the most common risk factor found in this study with profound hearing loss in both ears with p value = 0.05. Conclusion: No relation between prenatal risk factors and sensorineural hearing loss in children.

Genomic analysis of childhood hearing loss in the Yoruba population of Nigeria

Although variant alleles of hundreds of genes are associated with sensorineural deafness in children, the genes and alleles involved remain largely unknown in the Sub-Saharan regions of Africa. We ascertained 56 small families mainly of Yoruba ethno-lingual ancestry in or near Ibadan, Nigeria, that had at least one individual with nonsyndromic, severe-to-profound, prelingual-onset, bilateral hearing loss not attributed to nongenetic factors. We performed a combination of exome and Sanger sequencing analyses to evaluate both nuclear and mitochondrial genomes. No biallelic pathogenic variants were identified in GJB2, a common cause of deafness in many populations. Potential causative variants were identified in genes associated with nonsyndromic hearing loss (CIB2, COL11A1, ILDR1, MYO15A, TMPRSS3, and WFS1), nonsyndromic hearing loss or Usher syndrome (CDH23, MYO7A, PCDH15, and USH2A), and other syndromic forms of hearing loss (CHD7, OPA1, and SPTLC1). Several rare mitochondrial variants, including m.1555A>G, were detected in the gene MT-RNR1 but not in control Yoruba samples. Overall, 20 (33%) of 60 independent cases of hearing loss in this cohort of families were associated with likely causal variants in genes reported to underlie deafness in other populations. None of these likely causal variants were present in more than one family, most were detected as compound heterozygotes, and 77% had not been previously associated with hearing loss. These results indicate an unusually high level of genetic heterogeneity of hearing loss in Ibadan, Nigeria and point to challenges for molecular genetic screening, counseling, and early intervention in this population.

Determine the Frequency of Sensorineural Deafness in Children with Cerebral Palsy

Aim: To determine the frequency of sensorineural deafness in child with cerebral palsy. Study Design: Descriptive/cross-sectional Place and Duration of Study: Department of Neurology, Chandka Medical College Hospital, Larkana from 1st October 2020 to 31st March 2021. Methodology: One hundred and seventy patients of both genders age between 1-14 years were enrolled. Children diagnosed as having cerebral palsy and fulfilling the inclusion criteria was included. Hearing assessment was done with audiometry and degree of hearing loss was recorded in both ears in the form of mild, moderate, severe and profound degree of hearing loss. Results: Most of the patients 90 (52.94%) were between 1-5 years old followed by 51(30%) patients between 5-10 years with mean age of 4.8±7.8 years. 115 (67.65%) cases were males and 55 (32.35%) females. 54 (31.8%) cases had microcephaly and116 (68.2) cases had normal head circumference. Among 170 cases of cerebral palsy 48 (28.23%) cases had hearing loss and 122 (71.77%) of patients of CP had no hearing deficit. Among 48 cases of CP with hearing loss 15 (31.25%) cases had mild, 14 (29.17) cases had moderate, 10 (20.83%) had severe and 11 (22.92%) cases had profound hearing loss. Conclusion: The frequency of sensorineural deficit is high and significantly associated with cerebral palsy patients. Keywords: Degree of hearing loss, Sensorineural hearing loss, Cerebral palsy

Sensorineural hearing loss after bacterial meningitis in children

Sensorineural hearing loss – form of hearing loss up to its total lossat which any of the sites of the sound-receiving part can be damaged. The most important problem of patients with sensorineural hearing loss is lack of effective treatment and inability to restore hearing because 80% of patients who suffered from bacterial meningitis loss of hearing is accompanied by ossification of internal ear structures. However, bacterial meningitis is still registered in Kazakhstan and is a reason of development of sensorineural hearing loss and often accompanied byossification of internal ear structures in children and adults leading to longterm neurological lesions, such as hearing impairments or hearing loss. For early diagnosis of hearing loss in children with bacterial meningitis, an audiological examination should be performed in the first 48 hours from the onset of the diseaseand after discharge from the hospital for 1 year. To date, the only effective method of hearing restoration is surgery – cochlear implantation. This review of the literature is devoted to one of the actual complications of bacterial meningitis – sensorineural deafness in children.

Prevalence of Severe and Profound Sensorineural Hearing Loss in School Children in West Bengal, India.

There is pressing need for reliable information about prevalence of sensorineural hearing loss in children in India. Without this basic information it is difficult to plan services on a rational basis. In this stratified cross sectional study, we aimed to determine the prevalence of severe and profound sensorineural hearing loss in school children in West Bengal, India. We hypothesized that all children with severe or profound hearing loss in elementary schools (age 6-14years) would be among those identified as 'children with special need' because of their marked problems with hearing, speech or communication. First stratum of our study included all 6-14years old mainstream elementary school 'children with special need', with known or suspected hearing problem. Second stratum covered all 6-14years old hearing impaired children attending special schools. Comprehensive audiological assessment of 10,763 'children with special need' in mainstream elementary schools identified 3984 children showing severe or profound SNHL (≥71dB). A further 1022 children were identified with similar loss in special schools, giving a total of 5006 children with severe or profound sensorineural loss among a school population of 8,654,057, with estimated prevalence of 0.58 (CI 0.57-0.59) per 1000 children. This is the first large scale study of the epidemiology of severe and profound sensorineural hearing loss in school children in India. Although further studies are needed from other parts of India, in the interim, findings of this study can form the basis for planning resource allocation, service provision, investigation into etiology and prevention of sensorineural deafness in children in India.

INNOVATIONS IN THE MANAGEMENT OF CHILDREN’S NEUROINFECTIONS

The article describes some topical problems of neuroinfections in children and the ways of their solutions on the basis of the experience of a leading institution — Pediatric Research and Clinical Center for Infectious Diseases. There are introduced some innovation approaches of diagnostics and therapy of bacterial purulent meningitis tested at the institution. There are described diagnostic markers of meningitis in children, method of ultrasound evaluation monitoring of the condition of kidneys, renal blood flow, adrenal glands in the patients with a fulminant course of generalized form of meningococcal infection, and innovation therapeutic approach by recombinant interleukin-2 — roncoleukin introduction to the complex therapy. There is reflected an innovation method of differential diagnostics by the determination of acute-phase protein haptoglobin in cerebrospinal fluid. There are presented the tested methods to diagnose sensorineural deafness in children with bacterial purulent meningitis, critical condition polyneuropathy, and inflammatory polyneuropathy by the technique of rehabilitation potential evaluation.

Neuroprotective Effects of Brain-Derived Neurotrophic Factor (BDNF) on Hearing in Experimental Pneumococcal Meningitis

Bacterial meningitis is still one of the most common causes of acquired profound sensorineural deafness in children despite antibiotic treatment. We investigated the neuroprotective effects of brain-derived neurotrophic factor on hearing function in experimental bacterial meningitis. We implanted stainless steel tubes into both cerebral ventricles of Sprague-Dawley rats aged 21 days. Bacterial meningitis was induced by inoculating a strain of serotype III Streptococcus pneumoniae into the cisterna magna. Six micrograms per day of brain-derived neurotrophic factor (groups 1 and 3) or albumin (groups 2 and 4) was injected into the cerebral ventricles 24 hours after or before infection, respectively, for a duration of 7 days. Additionally, all rats received antibiotic subcutaneous treatment starting 24 hours after infection for 7 days. Brainstem auditory evoked potentials were recorded 24 hours before and 24 hours after infection and after 7 days of treatment with brain-derived neurotrophic factor or placebo and antibiotics, respectively, to determine hearing threshold. Our results showed that the hearing thresholds of animals in each group increased significantly 24 hours after infection compared with the results recorded 24 hours before infection (P < .01). After 7 days of treatment with brain-derived neurotrophic factor, brainstem auditory evoked potential responses recurred in 16 ears when stimulated at 75 dB hearing level in groups 1 and 3. Their hearing thresholds significantly decreased compared with the control group 2 (P < .05) and group 4 (P < .01). However, 13 of 14 ears absent brainstem auditory evoked potential responses could still not be identified at 75 dB hearing level in control groups 2 and 4. The improvement of the hearing thresholds in group 3 (treated before infection) was greater than that of group 1 (treated after infection) (P < .05), but there was no significant difference found between the control groups before and after infection (P > .05). Our study supports the hypothesis that the administration of exogenous brain-derived neurotrophic factor can be effective in preventing or treating hearing loss following bacterial meningitis.

Electron Microscopic Temporal Bone Histopathology in Experimental Pneumococcal Meningitis

Bacterial meningitis is one of the most common causes of acquired profound sensorineural deafness in children. Measurement of hearing and examination of the cochlea is limited in patients suffering from acute meningitis. A rabbit model of pneumococcal meningitis was developed to identify the temporal bone histopathologic changes that occur in meningogenic labyrinthitis caused by Streptococcus pneumoniae. Light microscopy was previously performed on temporal bones from acutely meningitic rabbits with profound hearing loss as determined electrophysiologically. Extensive inflammation of the cochlea with endolymphatic hydrops was observed. The organ of Corti, however, showed preserved architecture in the majority of these animals. In order to further investigate these findings, a protocol was used to create meningitic rabbits with hearing loss ranging from early high-frequency loss to profound deafness. The temporal bones from 7 rabbits were examined by transmission electron microscopy. In cases of mild hearing loss, partial degeneration of the inner row of outer hair cells, as well as edema of efferent cochlear nerve endings and marginal cells of the stria vascularis, was seen. With increasing degrees of hearing loss, the remainder of the organ of Corti and intermediate cells of the stria showed ultrastructural abnormalities. Spiral ganglion cells and basal cells of the stria vascularis remained intact in all subjects. This study provides unique information regarding the histology and pathophysiology of meningogenic deafness. The clinical implications of these findings are discussed, with an emphasis on potentially reversible changes and therapeutic intervention.

The impact of dexamethasone on hearing loss in experimental pneumococcal meningitis.

Bacterial meningitis, particularly that resulting from Streptococcus pneumoniae, is a common cause of acquired profound sensorineural deafness in children. The pathogenesis of meningogenic hearing loss has been investigated in an experimental rabbit model. In this study significant deafness was documented within the first 15 hours of infection. Initiation of antibiotic therapy at this time diminished the severity of hearing loss in most animals. The addition of dexamethasone to antibiotic therapy prevented the development of profound deafness. These results suggest this model will be useful in developing antiinflammatory strategies to improve the outcome of bacterial meningitis.

Invasion of the Inner Ear by Haemophilus influenzae Type b in Experimental Meningitis

Bacterial interstrain variation for cochlear invasion was studied by intraperitoneal inoculation of infant rats with Haemophilus influenzae type b. Eight pairs of CSF isolates from children with or without deafness due to meningitis were injected into half of each litter in separate experiments. At 48 h, quantitative CSF culture results and CSF white blood cell counts were equivalent for the two groups. Organisms within the cochlea were detected in four of eight animals in each group. There was no difference between the deaf and nondeaf isolates in the degree or frequency of inner ear inflammation in formalin-fixed sections. In separate experiments, animals were inoculated with H. influenzae type b and 24 h later treated with ampicillin, or ampicillin plus dexamethasone. At 48 h, although CSF white blood cell counts were significantly reduced in the steroid group, no difference was noted in the degree of cochlear inflammation between the two groups. The ability of H. influenzae type b to invade the inner ear of infant rats does not correlate with the development of sensorineural deafness in children following H. influenzae type b meningitis. Steroid administration does not appear to diminish the inflammatory reaction within the cochlea more than antibiotics alone in this model, but may delay CSF sterilization by ampicillin.

Two-Level Screening Audiometry in Public School

The two-level screening audiometry has been performed every year since 1980 in Kobe City. The method is as follows. When a child fails to respond to 30 dB at 1 kHz or 25 dB at 4 kHz, he is tested at a level 20 dB higher. Thus one can obtain more information as to the grade and type of the hearing impairment of a child than by the usual one-level screening method. According to the two-level test the hearing status of each child was classified into 9 types, i.e., 0 (normal) to 8. Thus the hearing types of both ears of each child are described in two digits, for example 3--6. This method may be useful for statistical purposes as well as for individual care of hearing impaired school children, especially for children who need educational care but cannot consult the otological clinics for some reason. From the otological point of view, our method offers valuable information about the unawared sensorineural deafness in children of school age. (The children themselves, school teachers, and parents do not aware of its presence.)

Aetiology of bilateral sensorineural deafness in children.

The study was carried out in children born during the four year period 1981-1984 in the Greater Manchester County. The children were investigated for possible congenital infections. Perinatal assessment was carried out for adverse aetiological factors. Family histories were obtained and parents and siblings were examined for any hearing impairment. A total of 164 cases were investigated. Various aetiological groups were formed which showed the following distribution: cause unknown 36.5 per cent; genetic group 20.1 per cent; adverse perinatal factors 14.6 per cent; congenital infections 9.8 per cent; meningitis 6.1 per cent; chromosomal abnormalities 3.7 per cent; syndromal group 3.7 per cent; and miscellaneous 5.5 per cent. The findings are discussed in the light of previous studies.

小児感音難聴におけるステロイド療法について

小児感音難聴におけるステロイド療法について

Deafness Following Mumps: The Possible Pathogenesis and Incidence of Deafness

Mumps is thought to be the most common cause of unilateral acquired sensorineural deafness in children. Mumps deafness is usually sudden in onset, profound or complete, and may be associated with vestibular symptoms. The authors' clinical survey of 55 patients with unilateral deafness which could reasonably be ascribed to mumps indicates that the hearing loss is exclusively unilateral, severe or total and permanent, and that approximately 45% of the patients experienced dysequilibrium of vestibular origin. An analysis of the present series of mumps deafness also suggests that the primary route of invasion of the virus is hematogenous, and thus the term "viral endolymphatic labyrinthitis" is proposed as the possible pathogenesis of the deafness, since both tympanogenic and meningogenic routes of viral invasion to the labyrinth can be excluded on the basis of the clinical and cerebrospinal fluid studies. This view of the pathogenesis, particularly that mumps meningitis is not associated with deafness, is supported by several reports including those of Vuori et al. (1962), Azimi et al., (1969), Lindsay (1973), Nadol (1978), etc. The incidence of deafness following mumps appears to be extremely low, approximately 1:20,000, as estimated by Everberg (1957).

Congenital rubella in the United Kingdom before 1970: the prevaccine era.

Congenital rubella in England and Wales before the introduction of rubella vaccine is described. Data are derived from selected hospital studies of children with congenital rubella and on prospective follow-up studies of women infected with rubella during pregnancy. Estimates based on epidemiologic studies suggest that rubella accounted for 15% of the cases of sensorineural deafness in children and for 2% of congenital heart defects. From this information it can be calculated that in England and Wales 200-300 babies would have been born with serious defects due to congenital rubella infection each year, in the absence of an epidemic, if rubella vaccination had not been introduced. The importance of continued surveillance of congenital rubella defects and terminations of pregnancy because of rubella infection is stressed.

Unilateral Severe Sensori-neural Deafness

Twenty-nine patients with unilateral severe sensori-neural hearing loss were studied in the light of preservation of semicircular canal function. Most ears were incidentally found to be deaf at the age of 3 to 13, but 4 adult ears became deaf after mumps infection. Twenty-five out of 29 were total deafness and other 4 had residual hearing at 254 and 500Hz.Twenty-four ears had good response to 2ml ice water, but three ears revealed vestibular hypofunction.Serological studies using complement fixation and hemagglutination inhibition tests were done to clarify the etiologies of the deafness with preserved semicircular canal function, and these tests revealed no specific cause of the unilateral deafness.The conclusion of this study was that pathology of the severe unilateral sensori-neural deafness in children was in general in the cochlea and that most of the cases preserved the semicircular canal function.

Acupuncture as a treatment for sensori-neural deafness in children

Acupuncture as a treatment for sensori-neural deafness in children

Feasibility of acupuncture as a treatment for sensori-neural deafness in children.

AbstractPurpose of this study is to test the hypothesis that treatment by acupuncture can decrease the level of hearing loss in profoundly sensori‐neural deafness in children. Acupuncture treatments at sites recommended by Chinese colleagues were given five days per week for three weeks a month for six months. Thirty children received regular acupuncture, to a depth of 13 mm and 10 shallow acupucture, only 1–2 mm. Audiometric tests were performed weekly on each child at 8 points (125–8,000 Hz) plus SAT. At intervals bone conduction, SRT, and other audiometric tests were made. Treatments adhered closely to the recommendations of the Chinese colleagues; however, we set a six‐month pilot study rather than the extensive period (one to four years) which they follow.Comparisons were made of the average level of hearing loss in the two groups at the beginning and at the end of the study at four key frequencies. Although there is a small improvement in seven of the eight averages, this does not represent any practical improvement in hearing. Detailed comparison for each of the 40 subjects fails to reveal any consistently maintained improvement.