Absence Of Otoacoustic Emissions Research Articles

Late-onset of Labyrinthine Dysfunction in Chronic Brucella-induced Encephalitis

Brucellosis, caused by Brucella, dominates in the Middle East. Acute or chronic brucellosis can involve any organ system, and the involvement of the central nervous system leads to encephalitis, called neurobrucellosis (NB). We report a rare case from north India where a variant of Brucella caused NB led to progressive labyrinthine dysfunction; who developed vertigo, disequilibrium, and gradual progressive sensorineural (SN) hearing loss. Audiological tests pure-tone audiometry (PTA), otoacoustic emissions (OAE), stapedial muscle reflexes, auditory evoked brainstem responses (ABR), and vestibular evoked myogenic potentials (VEMPs) were conducted on the patients. PTA showed a bilateral profound SN hearing loss, an absent OAE, and ABR suggestive of cochlear dysfunction. The cervical and ocular VEMP showed delayed n1 and p1 latencies, suggesting superior and inferior nerve branch involvement. Vertigo or disequilibrium and SN hearing loss can be the late-onset manifestations of labyrinthine dysfunction in chronic Brucella-induced encephalitis.

Role of Otoacoustic Emissions in Hearing Assessment of Children with Autism

Evaluation of hearing is essential in the diagnostic process of children suspected having autism spectrum disorder (ASD). The aim of this study was to examine the efficiency and feasibility of utilizing otoacoustic emissions as an objective test to assess hearing in children diagnosed with ASD. A pilot study was conducted over a period of 3 years (from September 2019 to November 2022) in the Audiology Department at the Center for Specialty Surgeries, Benghazi, Libya. A total of 61 children with autism aged between 2-15 years referred for hearing evaluation were enrolled in the study. The clinical procedure consisted of otoscopic ear examination, and Otoacoustic Emission (OAE) recording. OAE testing was attempted on the study sample (n 51) who met the inclusion criteria. And successfully completed for 44 participants (86%): 40 cases (91%) from the first attempt while 4 cases (9%) needed a second or more visit. Four autistic children (9%) had absent otoacoustic emissions in one ear. Testing couldn't be completed for 7 children (14%) due to behavioral difficulties. Results of this study showed the feasibility of using Distortion Product Otoacoustic Emissions (DPOAEs) in assessment of hearing for children with autism for whom obtaining reliable behavioral responses can be challenging.

High Frequency Tympanometry in Early Infancy and Its Effect on Oto Acoustic Emissions.

The present study was conducted to study the benefit of high frequency tympanometry (HFT) in detecting middle ear pathologies in infants. This study also aims to find the effect of middle ear diseases on absent otoacoustic emissions (OAE). Our study was a prospective study which included 123 healthy neonates below 1 month of age. The babies were referred for the new born hearing screening programme. They were subjected to DPOAE followed by tympanometry with a 1000z probe tone (HFT). The study was approved by the institutions ethical committee. "Present" OAE result was obtained for 202 ears, while "absent" OAE result was obtained for 44 ears. High frequency tympanometry was also performed on all the participants, where a normal tympanogram was obtained in 170 ears and 76 ears showed an abnormal tympanogram. The following parameters were studied: peak pressure, ear canal volume and compliance. Descriptive statistics were obtained for the same. Association of OAE with HFT was studied. Out of the 44 ears with refer OAE, 34 ears had an abnormal tympanogram and among 202 ears with a present OAE, 160 ears had a normal tympanometric peak indicating a sensitivity and specificity of 77.3% and 79.2% respectively. High frequency tympanometry not only helps in early detection of middle ear pathologies but also to find the effect of middle ear diseases on absent otoacoustic emissions.

Noise-induced Hearing Loss among Non-Medical Workers in a Healthcare Institution

Introduction: The effects of prolonged and excessive noise exposure on healthcare workers have not been studied. This study aims to estimate the prevalence of hearing loss among such workers and correlate the degree of hearing loss with age, duration of exposure, and coexisting illnesses. Methods: A retrospective review of 117 clinical records was conducted on 179 employees working in areas of excessive noise in a tertiary care hospital. Correlations between categorical variables were made using the Chi-square and Fisher's exact tests, while an independent samples t-test was used to compare means for continuous variables. Binary logistic regression was used to quantify the strength of correlations through an odds ratio and 95% confidence interval. Results: A clinical evaluation was conducted to assess hearing loss on employees from various departments, including Boiler Section, Central Sterile Supply Department (CSSD), Laundry, Prosthetics and Orthotics Laboratory, and Dietary. The median age of the participants was 37 years. Out of the 116 participants who underwent by audiological tests, 63.8% had sensorineural hearing loss and 68.5% had absent otoacoustic emissions in at least one frequency. The incidence of hearing loss was found to increase with age (p = 0.037). A significant correlation was noted between hearing loss and the duration of noise exposure of more than 10 years (p = 0.0013). Considering all areas together, a significantly higher proportion of employees with hearing loss was observer (p = 0.044), with 69.5% in the CSSD. Conclusion: Noise-induced hearing loss is a prevalent occupational health hazard in healthcare settings. The risk of developing hearing loss increases with the duration of work in a noise-generating area.

Infantile Onset Encephalomyopathy, Heart Block, and Sensorineural Hearing Loss: RMND1-Associated Mitochondrial Disease

AbstractMutations in RMND1 (required for mitotic division-1) has been associated with infantile onset mitochondrial disease and combined oxidation phosphorylation deficiency. This report describes a girl child of Indian origin with RMND1-associated mitochondrial disease. This 13-month-old girl, born to consanguineous parents presented with gradual loss of acquired milestones and recurrent vomiting from 5 months of age. She experienced failure to thrive, profound hypotonia, areflexia, and sensorineural deafness. Evaluation showed elevated serum lactate and complete heart block. Audiological evaluation done at 6 and 13 months of age revealed bilateral A type tympanogram, bilateral absent stapedial reflexes, absent otoacoustic emissions (OAE), and absent brainstem auditory evoked responses suggestive of bilateral profound sensorineural hearing loss. Muscle biopsy revealed evidence of ragged red fibers, ragged blue fibers, and Cytochrome coxidase (COX) deficient fibers on histochemistry and multiple complex deficiency on spectrophotometry. Exome sequencing revealed homozygous stop-loss variation, c.1349G > C, in exon 12 of RMDN1 resulting in substitution of amino acid serine for stop codon at position 450 and subsequent elongation of the protein by 31 amino acids (p.Ter450SerextTer31) which was verified by Sanger's sequencing. This report further strengthens the phenotype genotype correlations in RMND1-associated mitochondrial disease, especially the occurrence of the reported variation in South Asian patients. In addition, familiarity with the phenotype might help the physician to do targeted metabolic testing and facilitate appropriate early interventions.

A Hearing Screening Pilot Study With Students With Significant Cognitive Disabilities.

Purpose Many children with severe intellectual and developmental disabilities are at a higher risk for hearing loss than their peers who are typically developing. Unfortunately, they do not consistently participate in routine school-based hearing screenings. The current study investigated the feasibility of increasing their participation using an otoacoustic emissions protocol and documented results relative to student educational profiles. Method A total of 43 students with significant cognitive disabilities enrolled at a public school exclusively serving this population participated in the study. All but 9, who were excluded because of known hearing loss, were screened by a licensed audiologist assisted by audiology doctoral students. The protocol included otoscopy, tympanometry, distortion product otoacoustic emissions (DPOAEs), and teacher surveys. Results DPOAE screening was attempted on 33 students without previously diagnosed hearing loss and successfully completed for 26 (78.8%). Two students (4.6%) with absent otoacoustic emissions and normal tympanograms were referred for further assessment due to concerns about possible sensorineural hearing loss in one or both ears, and 10 (23.3%) had abnormal tympanograms in one or both ears. Conclusions Considering the high risk of sensorineural hearing loss for children with significant disabilities, it is important for them to be included in school hearing screenings. The results of this study demonstrate the feasibility of using DPOAEs for school-based hearing screenings with this population with an interprofessional team of licensed audiologists, educators, and speech-language pathologists. The results further suggest that students with significant disabilities and hearing loss may be unidentified and underserved. Given the complex needs of this population, an interprofessional practice model for hearing screenings and intervention services is recommended.

Clinical and epidemiological aspects of microcephaly in the state of Piauí, northeastern Brazil, 2015‐2016

ObjectivesTo describe aspects of the microcephaly epidemic in the state of Piauí. MethodsAll cases of congenital microcephaly confirmed in the state between 2015 and 2016 were included (n=100). Investigation forms of the Regional Reference Center for Microcephaly were reviewed. Discarded cases (n=63) were used as a comparison group. ResultsIn October, November, and December 2015 incidence rates reached 4.46, 6.33 and 3.86/1000 live births, respectively; 44 cases were reported in the state capital. Among the mothers of confirmed and discarded cases, the frequency of skin rash during pregnancy was 50/97 (51.5%) and 8/51 (15.7%), respectively (p<0.001); 33 confirmed cases (35.9%) had a head circumference z‐score between −2 and −3, 23 (25%) between −3 and −4, and 8 (8.7%) had a z‐score of less than −4. Head computer tomography scans revealed calcifications in 78/95 (82.1%) cases. Lissencephaly, hydrocephalus and agenesis of the corpus callosum were also frequently observed. Ophthalmic findings included retinal pigment epithelium rarefaction and atrophy. Absence of otoacoustic emissions was observed in 21/70 cases. One newborn also presented lower limb muscle atrophy. There were no significant differences in vaccination rates for influenza, diphtheria‐tetanus‐acellular pertussis, and hepatitis B in either group. ConclusionsThe state of Piauí, like others in the northeastern region, faced an epidemic of congenital microcephaly between 2015 and 2016, presumably related to congenital Zika virus infection, more intense in the capital. Current challenges include the improvement of vector control, basic research, scaling‐up of diagnostic tools for pre‐natal screening of Zika virus, vaccines, and health care for affected children.

Phenotypic Characterization of DFNB16-associated Hearing Loss.

We hypothesized that patients with DFNB16 caused hearing loss show characteristical audiological findings depending on genetic results. Hearing loss belongs to the most frequent congenital diseases. In 50-70% of individuals, hearing loss is caused by genetic defects. DFNB1 (deafness, neurosensory, autosomal-recessive) is the most frequently affected locus. Despite its great genetic heterogeneity, comprehensive analysis of genes like STRC, encoding stereocilin (DFNB16) is possible. The genetic architecture of the DFNB16 locus is challenging and requires a unique molecular genetic testing assay. The aim of the study is a systematic characterization of the audiological phenotype in DFNB16-positive patients. Since 2011, 290 patients with suspicion of inherited hearing loss received a human genetic exploration. Eighty two DFNB1-negative patients advanced to further testing in the DFNB16 locus. STRC-positive patients obtained complete audiological diagnostic workup. Additionally, epidemiological data was collected. Nine of 82 (11%) of the examined patients (mean age 5 yr) showed mutations in the STRC (3 homozygous, 6 compound heterozygous). Aside from a moderate hearing loss in the pure tone audiogram, auditory brainstem response thresholds were 40-50 dB nHL. Otoacoustic emissions were detectable in only one patient. Examination of the DFNB16-locus should be a standard diagnostic test after negative DFNB1-gene screening result. Notably, DFNB16-associated hearing loss can be audiologically characterized as moderate sensorineural hearing loss in the main speech field with absent otoacoustic emissions. Our study is the first to correlate audiological findings with genetic results in patients with hearing loss due to STRC.

Characteristics of auditory evaluation in Williams syndrome: a systematic review.

Identify the characteristics of the clinical audiological evaluation of individuals with Williams syndrome by means of a systematic literature review. The following research question was initially determined: "What are the characteristics of clinical auditory assessment in individuals with Williams syndrome?". From this, a bibliographic search was conducted in four databases using the descriptors: Williams syndrome, Hearing loss, and Audiology. Only full articles with evidence levels 1 or 2, published in Brazilian Portuguese or English, were selected. Results obtained in the auditory tests used in the clinical routine, namely: immittance test, pure-tone audiometry, otoacoustic emissions, and brainstem auditory evoked potential were analyzed. Two hundred nine studies were found, but only 12 met the inclusion criteria for the study. It was possible to observe prevalence of type A tympanometry curve, which may occur with absence of acoustic reflexes, mild to moderate sensorineural hearing loss, affecting mainly the high frequencies, absent or less amplified otoacoustic emissions, and brainstem auditory evoked potential without retrocochlear alteration. Cochlear impairment is common in individuals with Williams syndrome and the main disorders found in the hearing assessment in this population are absence of otoacoustic emissions and acoustic reflexes, as well as presence of mild to moderate sensorineural hearing loss, mainly in the high-frequency range, observed by audiometry.

Association between hearing organ and renal function in young adult type 1 diabetic patients: A cross-sectional study

Type 1 diabetes can lead to impaired function of many organs and tissues. The aim of this study was to evaluate associations between hearing and kidney function in young adult type 1 diabetic patients. 31 patients (9 women) with type 1 diabetes, aged <45, with disease duration <10 years were included. Blood and urine samples for laboratory tests and urinary albumin excretion (UAE) assessment were obtained. eGFR was calculated with CKD-EPI formula. In all patients pure-tone audiometry, transient evoked otoacoustic emissions and auditory brainstem responses were evaluated, also eye fundus was examined. Mean patients’ age was 29.5 ± 7.0 years and disease duration 4.6 ± 2.6 years. All patients had eGFR > 60.0 ml/min/1.73 m2. In one case microalbuminuria and in 3 patients early retinopathy were revealed. Linear correlation between eGFR and hearing threshold at 4, 6, 8 and 12 kHz was found. Patients with hearing impairment (n = 7) had lower eGFR 108.8 vs. 121.7 ml/min/1.73 m2, p = 0.047 compared to normal-hearing subjects. Also patients with absence of otoacoustic emissions in at least one ear had lower eGFR, 103.1 vs. 123.3 ml/min/1.73 m2, p < 0.001, compared to the remaining group. In auditory brainstem responses we found significant linear correlation between eGFR and wave III and interval I-III latencies, and between UAE and waves III, V and interval I-III latencies. This study suggests existence of relationship between hearing and kidney function in type 1 diabetic patients. Pathways directly linking hearing and renal function are unknown. Larger studies are necessary to further analyze these relationships.

Clinical and epidemiological aspects of microcephaly in the state of Piauí, northeastern Brazil, 2015–2016

ObjectivesTo describe aspects of the microcephaly epidemic in the state of Piauí. MethodsAll cases of congenital microcephaly confirmed in the state between 2015 and 2016 were included (n=100). Investigation forms of the Regional Reference Center for Microcephaly were reviewed. Discarded cases (n=63) were used as a comparison group. ResultsIn October, November, and December 2015 incidence rates reached 4.46, 6.33 and 3.86/1000 live births, respectively; 44 cases were reported in the state capital. Among the mothers of confirmed and discarded cases, the frequency of skin rash during pregnancy was 50/97 (51.5%) and 8/51 (15.7%), respectively (p<0.001); 33 confirmed cases (35.9%) had a head circumference z-score between −2 and −3, 23 (25%) between −3 and −4, and 8 (8.7%) had a z-score of less than −4. Head computer tomography scans revealed calcifications in 78/95 (82.1%) cases. Lissencephaly, hydrocephalus and agenesis of the corpus callosum were also frequently observed. Ophthalmic findings included retinal pigment epithelium rarefaction and atrophy. Absence of otoacoustic emissions was observed in 21/70 cases. One newborn also presented lower limb muscle atrophy. There were no significant differences in vaccination rates for influenza, diphtheria-tetanus-acellular pertussis, and hepatitis B in either group. ConclusionsThe state of Piauí, like others in the northeastern region, faced an epidemic of congenital microcephaly between 2015 and 2016, presumably related to congenital Zika virus infection, more intense in the capital. Current challenges include the improvement of vector control, basic research, scaling-up of diagnostic tools for pre-natal screening of Zika virus, vaccines, and health care for affected children.

Hearing in Williams Syndrome: From Input to Processing

Williams Syndrome (WS) is a rare genetic disease recurrent from a microdeletion of 1.5 ~ 1.8 Mb on the long arm of the chromosome 7q11.23, involving approximately 26 to 28 genes. Although these individuals present a number of physical malformations and varying cognitive deficits, an intriguing aspect of this syndrome is high auditory sensitivity and musical appreciation, which can occur concurrently with hearing loss. Several studies in the literature have used auditory tests in order to characterize the audiological profile of these patients and, thus, to seek indications that allow us to better understand how the auditory information processing in this syndrome occurs. Thus, the present study aimed to assess and describe the main characteristics of the auditory phenotype in individuals with Williams syndrome (WS) related to sound conduction and processing by the auditory system and to search for scientific evidence of genetic factors that may affect the auditory characteristics of this population. Some genes frequently absent in WS individuals may be responsible for changes in the structures and functionality of the auditory system and the brain and may be related to some auditory characteristics observed in this population. The absence of acoustic reflexes has been reported, as well as, cochlear impairment with absence of Otoacoustic Emissions and progressive sensorineural hearing loss, mainly in the high frequencies. As for auditory processing, more studies are needed in order to better understand how sound processing occurs in these patients. Some genes that are frequently absent in WS, including ELN, GTF21 and LIMK1, may account for changes in the structures and functions of the auditory system and brain as a whole in these individuals and may be related to certain auditory characteristics observed in this population. Further studies on auditory processing are needed to better understand how sound processing occurs in these individuals.

Role of otoacoustic emissions in the early detection and prevention of ototoxicity

Introduction: Evaluating Otoacoustic Emissions (OAEs) is one of the audiological methods for ototoxicity monitoring. The absence of OAEs indicates cochlear damage. The aim of this study was to study the usefulness of OAEs in early detection and prevention of ototoxicity. Materials and Methods: A prospective, comparative study was conducted to evaluate Transient-Evoked OAEs (TEOAEs) and Distortion-Product OAEs (DPOAEs) as they relate to Pure Tone Audiometry (PTA) in cases of ototoxicity. Hearing assessments of study group of 55 individuals exposed to ototoxic drugs were evaluated and compared at regular intervals, within the group and with control group of 25 individuals using PTA, TEOAEs, and DPOAEs. The abnormal OAE responses were calculated as predictive of significant changes in PTA thresholds. Student's t-test (independent samples, two-tailed) and ANOVA used for analysis. Results: PTA thresholds in the study group remained unaffected in the first three follow-ups whereas TPOAEs and DPOAEs were both found affected at the third follow-up, in significant number of ears in the study group. Significantly lower PTA thresholds were found in study group only at the fourth follow-up. Conclusions: Changes in TEOAEs and DPOAEs precede changes in PTA thresholds in ototoxicity and have high sensitivity and specificity in predicting hearing loss in such cases.

Investigating the Possible Audiological Effects of Hypothyroidism

Objective: We investigated audiological function in patients diagnosed with primary hypothyroidism secondary to Hashimoto’s thyroiditis. Methods: Forty-one patients diagnosed with primary hypothyroidism secondary to Hashimoto’s thyroiditis and 20 control subjects participated in this study. Otoscopic examination, immittance testing, pure tone audiometry, and otoacoustic emission testing were completed on all participants. In addition, all participants were screened for the presence of tinnitus. Results: All participants’ demonstrated normal otoscopic examination and 0.226-kHz tympanometry test findings. Pure tone audiometric (PTA) testing revealed mild sensorineural hearing loss (SNHL) in 16 patients diagnosed with primary hypothyroidism (39%) compared to only one participant in the control group. Eight patients with primary hypothyroidism reported bothersome tinnitus (19.5%), and transient evoked otoacoustic emissions (TEOAE) were absent in 8 left ears (19.5%) and 4 right ears (9.75%). Conclusion: Hypothyroidism secondary to Hashimoto’s thyroiditis may be a cause of mild SNHL, particularly at high frequencies, in addition to bothersome tinnitus. Absent otoacoustic emissions suggest cochlear outer hair cells as the possible lesion site in patients with hypothyroidism.

Auditory and otologic profile of Alström syndrome: Comprehensive single center data on 38 patients.

Alström syndrome (AS) is a rare autosomal recessive ciliopathy caused by mutations in the ALMS1 gene. Hallmark characteristics include childhood onset of severe retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, and cardiomyopathy. Here we comprehensively characterize the auditory and otologic manifestations in a prospective case series of 38 individuals, aged 1.7-37.9 years, with genetically confirmed AS. Hearing loss was preceded by retinal dystrophy in all cases, and had an average age of detection of 7.45 years (range 1.5-15). Audiometric assessments showed mean pure tone averages (0.5, 1, 2, 4 kHz) of 48.6 and 47.5 dB HL in the right and left ears, respectively. Hearing was within normal limits for only 8/74 ears (11%). For the 66 ears with hearing loss, the degree was mild (12%), moderate (54%), or severe (8%). Type of hearing loss was predominantly sensorineural (77%), while three ears had mixed loss, no ears had conductive loss, and type of hearing loss was indeterminate for the remaining 12 ears. Serial audiograms available for 33 patients showed hearing loss progression of approximately 10-15 dB/decade. Our data show that hearing loss associated with AS begins in childhood and is a predominantly symmetric, sensory hearing loss that may progress to a severe degree. Absent otoacoustic emissions, intact speech discrimination, and disproportionately normal auditory brainstem responses suggest an outer hair cell site of lesion. These findings indicate that individuals with AS would benefit from sound amplification and if necessary, cochlear implantation.

Otitis media with effusion in children younger than 1 year

ObjectiveTo determine the prevalence of otitis media with effusion in children younger than 1 year and its association with the season of the year, artificial feeding, environmental and perinatal factors. MethodsRetrospective study of 184 randomly included medical records from a total of 982 healthy infants evaluated for hearing screening tests. Diagnosis of otitis media with effusion was based on otoscopy (amber-gold color, fluid level, handle of malleus position), type B tympanometric curves and absence of otoacoustic emissions. Incomplete medical records or those describing acute otitis media, upper respiratory tract infections on the assessment day or in the last 3 months, neuropathies and craniofacial anomalies were excluded. Data such as gestational age, birth weight, Apgar score, type of feeding and day care attendance were compared between children with and without otitis media with effusion through likelihood tests and multivariate analysis. Results25.3% of 184 infants had otitis media with bilateral effusion; 9.2% had unilateral. In infants with otitis media, the following were observed: chronological age of 9.6±1.7 months; gestational age >38 weeks in 43.4% and birth weight >2500g in 48.4%. Otitis media with effusion was associated with winter/fall, artificial feeding, Apgar score <7 and day care attendance. The multivariate analysis showed that artificial feeding is the factor most often associated to otitis media with effusion. ConclusionsOtitis media with effusion was found in about one third of children younger than 1 year and was mainly associated with artificial feeding.

Auditory phenotype in Stickler syndrome: results of audiometric analysis in 20 patients.

Hearing loss in Stickler syndrome has received little attention due to the often more disabling ocular, orofacial and skeletal manifestations. Estimates suggest a global prevalence of sensorineural hearing loss (SNHL) ranging from 50% to about 100% though, depending on the underlying Stickler genotype. By performing extensive audiometric analysis in Stickler patients, we aimed to further elucidate the auditory phenotype. Twenty molecularly confirmed Stickler patients (age 10-62year), of whom sixteen with type 1 Stickler syndrome (COL2A1 mutation) and four with type 2 Stickler syndrome (COL11A1 mutation) underwent an otological questionnaire, clinical examination, pure tone and speech audiometry, tympanometry and otoacoustic emission testing. Cross-sectional and longitudinal regression analysis of the audiograms was performed to assess progression. In type 1 Stickler syndrome, 75% demonstrated hearing loss, predominantly in the high frequencies. No significant progression beyond presbyacusis was observed. All type 2 Stickler patients exhibited mild-to-moderate low- and mid-frequency SNHL and moderate-to-severe high-frequency SNHL. In both types, hearing loss was observed in childhood. Otoacoustic emissions were only detectable in 7/40 ears and had very low amplitudes, even in frequency bands with normal hearing on pure tone audiometry. Type 1 Stickler syndrome is characterized by a mild high-frequency SNHL, emerging in childhood and non-progressive. Absent otoacoustic emissions are a frequent finding. Patients with type 2 Stickler syndrome exhibit early-onset moderate SNHL affecting all frequencies with a sloping audiogram. Taking into account the visual impairment in many patients, we recommend regular auditory follow-up in patients with Stickler syndrome, especially in childhood.

Study of the prevalence of impaired hearing and its determinants in the city of Itajaí, Santa Catarina State, Brazil

The number of people with impaired hearing is increasing; knowing its magnitude is essential for public health. ObjectiveTo study the prevalence and determinants of impaired hearing in Itajaí/SC. MethodsA population-based survey based on a World Health Organization protocol. Field research was carried out from July 2008 to 2011. Procedures for evaluating hearing in households: questionnaire, measurement of noise, otoscopy, pure tone audiometry at 1000, 2000, and 4000 Hz, tympanometry, and acoustic reflexes: individuals above 4 years; children under 4 years: evoked otoacoustic emissions (OAE), cocleo-palpebral reflex(CPR), tympanometry, and acoustic reflexes. In the elderly population a questionnaire of perception of hearing loss was applied. ResultsThe study sample consisted of 379 individuals – 45.38% were males and 54.62% were females. Age-range: 11.34% up to 10 years; 64.39% 10 to 60 years, 24.27% over 60 years. Evaluation of the best hearing ear showed that 74.1% of residents had normal hearing, 18.9% had mild hearing loss, 5.1% had moderate hearing loss, 1.9% had severe hearing loss. Disabling impaired hearing was detected in 26 subjects: one child (otitis media); four adults (one otitis, one noise-induced, two idiopathic); 21 elderlies (presbyacusis). Of eight children under 4 years all presented CPR, three were normal examinations, two had absent OAE bilaterally, one had absent OAE in the right ear and one in the left ear. ConclusionThe prevalence of disabling impaired hearing in Itajaí was 7%; the highest prevalence was in the 50-year and above age group – the main cause was presbyacusis.

Otite média com efusão em crianças menores de um ano

ObjectiveTo determine the prevalence of otitis media with effusion in children younger than 1 year and its association with the season of the year, artificial feeding, environmental and perinatal factors. MethodsRetrospective study of 184 randomly included medical records from a total of 982 healthy infants evaluated for hearing screening tests. Diagnosis of otitis media with effusion was based on otoscopy (amber‐gold color, fluid level, handle of malleus position), type B tympanometric curves and absence of otoacoustic emissions. Incomplete medical records or those describing acute otitis media, upper respiratory tract infections on the assessment day or in the last 3 months, neuropathies and craniofacial anomalies were excluded. Data such as gestational age, birth weight, Apgar score, type of feeding and day care attendance were compared between children with and without otitis media with effusion through likelihood tests and multivariate analysis. Results25.3% of 184 infants had otitis media with bilateral effusion; 9.2% had unilateral. In infants with otitis media, the following were observed: chronological age of 9.6±1.7 months; gestational age >38 weeks in 43.4% and birth weight >2,500g in 48.4%. Otitis media with effusion was associated with winter/fall, artificial feeding, Apgar score <7 and day care attendance. The multivariate analysis showed that artificial feeding is the factor most often associated to otitis media with effusion. ConclusionsOtitis media with effusion was found in about one third of children younger than 1 year and was mainly associated with artificial feeding.

Cockayne syndrome group B (Csb) and group a (Csa) deficiencies predispose to hearing loss and cochlear hair cell degeneration in mice.

Sensory hair cells in the cochlea, like most neuronal populations that are postmitotic, terminally differentiated, and non-regenerating, depend on robust mechanisms of self-renewal for lifelong survival. We report that hair cell homeostasis requires a specific sub-branch of the DNA damage nucleotide excision repair pathway, termed transcription-coupled repair (TCR). Cockayne syndrome (CS), caused by defects in TCR, is a rare DNA repair disorder with a broad clinical spectrum that includes sensorineural hearing loss. We tested hearing and analyzed the cellular integrity of the organ of Corti in two mouse models of this disease with mutations in the Csb gene (CSB(m/m) mice) and Csa gene (Csa(-/-) mice), respectively. Csb(m/m) and Csa(-/-) mice manifested progressive hearing loss, as measured by an increase in auditory brainstem response thresholds. In contrast to wild-type mice, mutant mice showed reduced or absent otoacoustic emissions, suggesting cochlear outer hair cell impairment. Hearing loss in Csb(m/m) and Csa(-/-) mice correlated with progressive hair cell loss in the base of the organ of Corti, starting between 6 and 13 weeks of age, which increased by 16 weeks of age in a basal-to-apical gradient, with outer hair cells more severely affected than inner hair cells. Our data indicate that the hearing loss observed in CS patients is reproduced in mouse models of this disease. We hypothesize that accumulating DNA damage, secondary to the loss of TCR, contributes to susceptibility to hearing loss.