Lawsonia intracellularis is an obligate intracellular bacterium and causative agent of proliferative enteropathy. The pathogenesis of L. intracellularis is not completely understood, including the endocytic mechanisms to access the host cell cytoplasm. In this study, we evaluated the mechanisms involved in endocytosis of L. intracellularis in vitro using intestinal porcine epithelial cells (IPEC-J2). Confocal microscopy was used to co-localize L. intracellularis and clathrin. Clathrin gene knockdown was then applied to verify whether L. intracellularis endocytosis is clathrin-dependent. Finally, internalization of viable and non-viable (bacteria were inactivated by heat) L. intracellularis organisms were assessed to study the role of the host cell during bacterial endocytosis. L. intracellularis organisms were observed co-localized with clathrin by confocal microscopy but the amount of L. intracellularis internalized in cells, with and without clathrin knockdown, did not differ statistically. The internalization of non-viable L. intracellularis showed a decrease in the internalization in cells with less clathrin synthesis (P<0.05). The present study is the first to elucidate the involvement of clathrin in the endocytosis of L. intracellularis. Clathrin-mediated endocytosis was shown to be an important, but not required, process for L. intracellularis internalization in porcine intestinal epithelial cells. Independence of bacterial viability for host cell internalization was also confirmed. Lawsonia intracellularis is an obligate intracellular, microaerophilic, Gram-negative bacterium that causes proliferative enteropathy (PE) (thy (PE) . This dithy (PE) , 3.L. intracellularis is still poorly understood. L. intracellularis infection occurs via the fecal-oral route. In the gastrointestinal tract, the microorganism overcomes the hostile environment of the stomach and localizes in the aboral region of the small intestine (ileum) (The pathogenesis of  (ileum) . The bac (ileum)  to crossvia different routes. The zipper mechanism does not require metabolically active invaders for its initiation. The zipper endocytosis process is associated with proteins or structures of the outer membrane of the bacterium that interact directly with specific host cells receptors. In this process, there is a clustering of eukaryotic cell receptors, associated with changes in the actin cytoskeleton resulting in the microorganism being enwrapped by cytoplasmic membrane  in cells able to synthesize normal levels of clathrin (L. intracellularis was significantly decreased (P < 0.05) in clathrin knockdown IPEC-J2 cells  when infecting IPEC-J2 cells under a clathrin gene silencing condition. The method of bacterial inactivation used in the present study (65°C/30 min) would have likely minimal impact on surface structure  mechanisms is very likely.The importance of n et al.  have obsListeria monocytogenes, which expresses two proteins involved in the internalization mechanism . Those proteins mimic some host cell proteins and initiate the process of clathrin-mediated endocytosis  were infected with dead L. intracellularis. This experiment resulted in a decreased internalization of heat-killed bacteria in cells with diminished clathrin. Based on this experiment results it is plausible to hypothesize that live L. intracellularis can be internalized through self-dependent mechanisms, that do not depend on clathrin expression, possibly through the process of endocytosis called trigger. In the trigger endocytosis' process, there is an initial pathogen-host interaction and, from there, bacterial proteins are secreted into the eukaryotic cell cytosol through the T3SS, an apparatus that has already been shown to be present in L. intracellularis , previously isolated from swine with the hemorrhagic form of proliferative enteropathy, was used at passages 12 to 14 to infect the IPEC-J2 cells. A pure culture of the bacterium was thawed and grown in McCoy cell culture incubated in a bag with an atmosphere of ~6% oxygen and 8% carbon dioxide . After monitoring the culture for 6 days, an immunocytochemistry was performed and there were not labeling for L. intracellularis antigens (data not shown). As the aim of the immunocytochemistry is to stain L. intracellularis in the cytoplasm of the inoculated cells, a negative stain proves that there were no bacteria in the inoculum able to infected and to propagate in the IPEC-J2 cells. Experiments were performed three times, each time with triplicates, with the inoculum containing 2.1 × 105, 3.4 × 105, and 1.8 × 105L. intracellularis/ml and 1.7 × 104, 2.4 × 104, and 2 × 104 IPEC-J2 cells/ml, in each triplicate, respectively.Intestinal porcine epithelial cells (IPEC-J2)  were culescribed . L. intr dioxide , 20, for dioxide –23, and  at 65°C . To provL. intracellularis; (b) IPEC-J2 knockdown for clathrin (described below) inoculated with heat-killed L. intracellularis (described above); (c) IPEC-J2 not transfected (control) inoculated with live L. intracellularis; and (d) IPEC-J2 not transfected (control) inoculated with heat-killed L. intracellularis. The cell cultures were allowed to be in contact with L. intracellularis microorganisms for 1 h. After that period, the supernatant was removed, and the cell monolayer was washed three times with PBS and fixed with 4% paraformaldehyde.The experimental groups were: (a) IPEC-J2 knockdown for clathrin (described below) inoculated with viable L. intracellularis internalization. The knockdown of the gene clathrin heavy chain like 1 (CLTCL1) responsible for the synthesis of this protein was performed via silencing RNA (siRNA) followed by the inoculation of the bacteria .IPEC-J2 cells transfected with siRNA and non-transfected IPEC-J2 cells were washed three times with PBS  and lyseL. intracellularis at a multiplicity of infection of 10 at 37°C for 1 h. This period of incubation was chosen after performing a pilot test was performed in which four periods of incubation were tested  and the highest number of fluorescent signals (L. intracellularis and clathrin) was observed at 1 h (data not shown). Subsequently, the cells were washed three times with PBS and fixed in 4% paraformaldehyde for 10 min at room temperature. Thereafter, further washing with PBS was carried out to remove excess paraformaldehyde. Fixed cells were incubated for 30 min at 37°C with the following primary antibodies: anti-clathrin , and anti-L. intracellularis (source: mouse) (IPEC-J2 cells were grown on coverslips in 24-well plates and infected with : mouse) . After wL. intracellularis after 1 h of culture, the IPEC-J2 cells were washed three times with PBS to remove extracellular bacteria  can be found in the article/supplementary material.CP, TR, FV, CG, and RG: conceptualization and formal analysis. CP, TR, and AD: methodology. CP and TR: software. CP, TR, and FV: validation. CP, TR, CG, and RG: data curation. CP: writing—original draft preparation. TR, AD, FV, CG, and RG: writing, reviewing, and editing. CG and RG: funding acquisition. All authors have read and agreed to the published version of the manuscript.


All individuals were evaluated using pure tone audiometry, acoustic immittance measurements, and Transient Evoked Otoacoustic Emissions (TEOAE). The audiological profile in individuals with WS was analyzed, and TEOAE responses were compared between WS individuals without hearing loss and typical developmental individuals.The hearing loss was observed in 50% of patients, being 78.95% sensorineural and 21.05% mixed. This hearing loss was predominantly mild to moderate, affecting mainly frequencies above 3 kHz. As for TEOAE, there was a higher incidence of absence and lower amplitude responses in individuals with WS.WS individuals have hair cell dysfunction, mainly in the basal region of the cochlea. Thus, TEOAE analysis is an important clinical resource to be considered in the routine audiological evaluation. It is estimated to occur in 1:7.500 live births and has an equal prevalence in male and female individuals. The WS phenotype is characterized by multiple physical, neurological, and systemic abnormalities.Williams syndrome (WS) is a rare genetic disorder resulting from a microdeletion in the region of the long arm of chromosome 7 (7q11.23), containing approximately 28 genes-5, an involvement that starts in the late childhood period and early adult life and tends to be progressive,5-7.In the scope of the audiological phenotype, especially at high frequencies, above 3 kHz, sensorineural hearing loss has been pointed out in several studies addressing the WSElastin (ELN) gene, General Transcription Factor (GTF2I) gene, and Lim Domain Kinase 1 (LIMK1) gene. Many studies have observed the expression of these genes in the tissue of the auditory system, damaging blood supply, balance of ion gradient, and functionality of cochlear structures,9,10. Therefore, especially regarding cochlear functionality, the audiological assessment of WS is highly important for this population.Seeking to understand such a scenario, some studies have associated the influence of some genes often deleted in individuals with WS that can contribute to their hearing loss, including the .A way of measuring the functionality of outer hair cells of the cochlea is by capturing Otoacoustic Emissions (OAE), which are responses generated by the energy release in these cells and can occur spontaneously or evoked by an acoustic stimulus. The most commonly used stimuli include the transient-evoked otoacoustic emission (TEOAE), consisting of a brief stimulation through clicks or tone burst, and the Distortion Product (DPOAEs), in which two pure tones of different frequencies are emitted simultaneously generating a reflected response that is a product of the distortion resulting from the combination of the two tones,5,12,13. However, hearing-related studies in patients with WS are still very recent and scarce. Although different hypotheses have been raised since the early studies aiming to find responses that justify the behavioral phenotype of these individuals, little is known on the origin of most auditory characteristics of this population.Some OAE studies have demonstrated a cochlear fragility since even individuals with normal auditory thresholds can present cochlear alterations accompanied by subclinical symptomsTherefore, our goal is to characterize the audiological profile and assess the cochlear functionality of individuals with WS.This is a transversal clinical study approved by the Ethics Committee of the research institution, protocol number 15.825, carried out in individuals with WS (Study Group – SG) attended at the Genetics Unit of the Children's Institute of the Clinical Hospital of the Medicine School, University of São Paulo.The following inclusion criteria were applied for the individuals in the SG: to present confirmed WS diagnosis through the Multiplex Ligation-dependent Probe Amplification (MLPA) and belong to the age group between seven and 17 years old. The exclusion criteria were excess wax in the external acoustic meatus and failure to comply with all procedures.We performed a clinical analysis of the records that resulted in the selection of 34 patients to participate in the study. Four of these patients could not be reached, six did not agree to participate, one had excess wax in the external acoustic meatus and did not return for evaluation after removal, and one did not allow some of the procedures.Thus, 22 patients were excluded from the study: seven females and 15 males with chronological age between seven and 17 years (12.36 ± 3.02) and mental age between three and 14 years (6.52 ± 2.28). The Intelligence Quotient (IQ) assessment based on the Wechsler Abbreviated Intelligence (WASI) revealed results between 37 and 98 (54.05 ± 13.48).For comparison purposes, a Control Group (CG) was composed of a convenience sample containing 17 individuals with typical development and normal hearing – seven female and 10 male – of chronological age between seven and 17 years (11.88 ± 3.12). These individuals did not present any complaints of delayed cognitive neuropsychomotor, speech, or school performance development, which were verified through anamnesis with the respective parents or caregivers.; auditory thresholds obtained through pure tone audiometry below 20 dB NA at all tested frequencies ; Speech Recognition Threshold up to 10 dB HL above the three-tone average, and Speech Recognition Percentage Index containing at least 88% of hits.To emphasize the presence of hearing loss or middle ear involvement in the patients in the CG, we considered the following inclusion criterion: the presence of tympanometric curve types A, Ad or Ar and ipsi-and contralateral acoustic reflexes at the frequencies of 0.5, 1, 2, and 4 kHzBefore starting the procedures, the parents read and signed an Informed Consent Form, and the patients signed a Term of Consent. Next, the parents answered an audiological anamnesis to provide the information from the patients’ records regarding factors that could interfere with the assessment. Additionally, a meatuscopy was performed to verify a possible obstruction in the external acoustic meatus.. The acoustic reflexes were tested manually until the intensity of 110 dB HL and retested in cases of reflex absence to confirm the results.We performed acoustic immittance measures to verify possible middle ear involvement on an immittance meter by Interacustic, model AT235, equipped with a 226 Hz probe. According to the peak admittance measure, the tympanometric curve was characterized following Jerger’s criteria as normal auditory thresholds. Upon thresholds above 20 dB HL at any frequency between 0.5 and 4 kHz, bone-conduction audiometry was performed to determine the type of hearing loss. To establish the hearing loss degree, we considered each frequency individually as follows: from 25 to 40 dB HL – mild; 45 to 70 dB HL – moderate; 75 to 90 dB HL – severe; and above 95 dB HL – deep. The Speech Recognition Threshold (SRT) was applied in the speech audiometry aiming at a lower intensity at which the individual had at least 50% of hits for trisyllable or polysyllable spoken words, in addition to the Speech Recognition Percent Index (SRPI), with a list of 25 monosyllables to be spoken at the intensity of 30 dB HL above the SRT, considering hits above 88% as normal results.Subsequently, we conducted pure tone and speech audiometry using a clinical audiometer by Grason-Stadler, model GSI 61, with the patient inside the acoustic booth, according to regulation ANSI S3.1-1991 for the amount of environmental noise. Pure tone air-conduction audiometry thresholds were measured at the conventional frequencies (0.25 to 8 kHz) considering values up to 20 dB HLThe Transient Evoked Otoacoustic Emissions (TEOAE) were measured only in patients who presented normal auditory thresholds using the equipment Smart EP USB Jr by Intelligent Hearing Systems (IHS 5020) (Miami-Florida), equipped with a 10D phone placed on the patient’s acoustic meatus with latex rubber. The collection parameters encompassed the acquisition of 1024 click stimuli with a non-linear 75-microsecond duration at a rate of 19.30 stimuli per second, a gain of 4000 at the intensity of 80 dB SPL. The analysis window was kept open at 30 ms analyzing at the frequencies of 1, 1.5, 2, 3, and 4 kHz. To determine the presence of TEOAE, we established probe stability of ≥70%, response reproductivity of ≥50%, and applied the criterion of signal-noise relation (S/R) higher than 3 dB SPL for the frequencies of 1 and 1.5 kHz and higher than 6 dB SPL for the remaining frequencies. As for the response, we considered its presence in at least three frequency bands. In patients without TEOAE indication, the probe was repositioned and another measurement was performed considering the best response achieved.2) verified the association between two variable categories, such as groups and presence/absence of response.The data collected were tabulated and descriptive and inferential statistical analyses were performed through parametric tests since the sample followed a normal distribution. An unpaired T-test was applied to compare the control and study groups, while a Pearson's chi-square test . Values regarded as statistically significant are marked with an asterisk (*).Although only two families have reported being aware of the patient’s hearing loss, our study found 11 patients (19 ears) who presented hearing loss (50% of the sample) . Among tAlthough most cases showed a mild hearing loss degree with thresholds below 45 dB HL (12/19 ears = 63.16%), some patients had higher auditory thresholds reaching up to 65 dB HL (5/19 ears = 26.32%), whereas others presented thresholds above 65 dB HL (2/19 ears = 10.52%).The main types of the tympanometric curve were type-A tympanometric curve (14/19 ears = 73.69%), followed by type-Ad and B curves (3/19 ears each= 15.79% each), and type-C curve, found on both ears of a single patient (2/19 ears= 10.52%).The analysis of the presence of hearing loss according to the chronological age showed that the two groups (with hearing loss x without hearing loss) did not differ in this respect , since the data were dispersed, thus not demonstrating any association between these variables .The comparison of auditory thresholds between the groups revealed significantly higher thresholds in the SG than in the CG  ..To prevent the functionality analysis of the outer hair cells from being compromised, we measured the TEOAE only in the ears that did not present any type of hearing loss. Thus, 34 ears were assessed in the CG and 25 in the SG. Also, none of these individuals had a record of ear surgery or middle ear alteration, discarded by the presence of tympanometric curve of types B or C without ipsi- or contralateral acoustic reflexesInitially, we calculated the percentage of responses at each frequency assessed per group. We verified an association between the groups and the presence of TEOAE response, considering the absence of TEOAE as a key differentiator for the WS group .Subsequently, we calculated the descriptive measures considering the signal-to-noise ratio per frequency in each group. Statistically significant differences were found between the groups, with the signal-to-noise ratio with the lowest value in the SG at all analyzed frequencies .The goal of this study was to assess the audiological profile of individuals with WS and analyze the functionality of outer hair cells of the cochlea in patients without middle ear involvement, a record of ear surgery, or hearing loss. In addition, due to the inclusion of a Control Group, the results obtained from the TEOAE of individuals with WS could be compared with those of individuals with neurotypical development and without audiological involvement.Our findings point out the relevant prevalence of hearing loss in individuals with WS, thus suggesting that such population has a dysfunction in hair cells, especially in cochlea basal regions, a cochlear involvement that can occur even in patients without any apparent alteration in the basic audiological assessment., corresponding from 25% of individuals between 6 and 14 years to 59% of individuals at school age. In adult individuals, the literature indicates the presence of hearing loss in more than 38.46% of the assessed cases.The results from the pure tone audiometry threshold pointed to hearing loss in 50% of the studied population with WS, which corroborates other findings reported in the literature describing a high occurrence of hearing loss in WS cases,8,16,17 from mild to moderate degrees-6 with major damage at high frequencies,4,6-8,17.We found that most cases involved a mild sensorineural hearing loss emerging especially at high frequencies, from 3 kHz, which corroborates other findings reported in the literature describing the predominance of sensorineural hearing loss,18. In this study, we observed only six ears (13.63%) with auditory lowering at the frequencies of 4 kHz and/or 6 kHz, with recovery at the frequency of 8 kHz, including some presenting reflexes.Some studies have highlighted that the hearing loss in WS has a similar configuration to the noise-induced hearing loss, probably due to the absence of acoustic reflex, since the physiological mechanism of reflex can protect the auditory system of strong sounds.It is known that noise-induced hearing loss is related to oxidative stress. Therefore, an increase in the production of reactive oxygen species – ROS can result from uncontrolled mitochondria functions, ischemia, and toxic excitation, causing a production boost of free radicals leaving some regions of the cochlea, especially outer hair cells, vascular striae, and internal hair cells in the cochlea base, which are more vulnerable to lesionsGSTM1, SOD2, PON, KCNE1, CAT, PCDH15, and MYH14 gene. Thus, the theory of noise-induced hearing loss in WS should be further explored.Several studies have assessed the genes involved in the regulation of reactive oxygen species. Even though, the most commonly mentioned genes are not those deleted in the WS, like ,16 to 47% of the cases, in which individuals with WS can be more likely to present middle ear alterations than the general population.It is also worth noting that despite the prominent sensorineural hearing loss in this syndrome, we should not disregard the existence of conductive involvement due to the presence of both type-B and C tympanometric curves and mixed hearing loss, observed in 21.05% of the patients. Other studies reached an even higher percentage in patients with middle ear involvement, corresponding from 23%elastin (ELN) gene is expressed in many regions of the human body, including the tympanic membrane, auditory tube, and tendons of muscles that sustain the tympanic ossicular chain (as stapedius muscle and eardrum tensor),26. Therefore, the absence of such gene in WS can influence the mobility of the structures in the ossicular-tympanic system that conduct the acoustic stimulus to the internal ear, thus generating a deficiency in the middle ear functionality and consequently conductive hearing loss.Some studies have found that the  and has an early onset, starting in adolescence or early adult life,8,12,16. Although our study ranges a small sampling number of individuals at a limited age group gap (only 10 years), Another highlighted characteristic in the literature is that hearing loss in WS is progressive responses, especially at high frequencies,5, even in patients without any evident hearing loss in the assessment of conventional pure tone audiometry,13,17,27.Still, concerning the involvement of the cochlear function, our results demonstrated that individuals with WS presented a higher percentage of absent responses and a lower amplitude of TEOAE concerning individuals without the syndrome. Other previous studies had observed the absence or at least reduction of the amplitudes of OAE,12,17. It is worth pointing out that this study observed cochlear involvement in young patients (children and adolescents), which once again confirms that the dysfunction in the cochlea hair cells has an early onset.Some studies also found an attenuation in the TEOAE responses along timeIt is believed that the deletion of some genes in WS is responsible for the behavioral phenotype presented by these individuals and can explain some of the findings from the audiological assessment.ELN gene can be responsible for compromising the cochlear function as well, since it can reduce blood supply in the cochlea via vascular stenosis, resulting in hypoxia and cell death, in addition to causing stiffening of the basilar membrane, deregulating cochlea cell proliferation, and modifying the transduction signal of hair cells. Also, a deficiency in the ELN gene can lead to an unsynchronized movement of the stereocilia, resulting in delayed activation of the cochlear nerve and hearing loss.Many studies consider that the ELN, the GTF2IRD1 gene was found in the sensorineural tissues of the cochlea (acting as a receptor in the hair cells), spiral ganglion neurons – responsible for triggering the potential of action to conduct the auditory stimulus to the central auditory pathways –, vascular striae, Reissner membrane, among other types of cells in the rat organ of Corti. Thus, such dysfunctions can impair cochlear amplification through disturbances in the ion gradient, thus contributing to the hearing loss found in patients with WS.In addition to the LIMK1 gene has also been associated with the regulation of mobility in the cochlea hair cells, thus its deletion can generate a dysfunction in the mechanisms that regulate the mobility of hair cells and damage cochlear amplification, leading to the early and progressive hearing loss commonly presented by individuals with WS.The FZD9 gene, which has been found in spiral ganglion neurons, and the STX1 gene, observed in the spiral ganglion and synapse of hair cells in the organ of Corti.Additionally, other genes that are often deleted in individuals with WS have been associated with alterations in the organs of the auditory system, like the ,13,17,27. Thus, all participants’ families were instructed about the importance of annual hearing monitoring, and the patients with any alteration in the auditory assessment were referred for evaluation and medical conduct.Accordingly, several studies have highlighted the importance of audiological monitoring in patients with WS, a scope in which OAE analysis is an important resource to be considered in audiological assessment batteries for being able to provide evidence on damages in the function of cochlea hair cells, even before the thresholds reached in the pure tone audiometry became alteredIn this context, together with our findings, it is worth highlighting the importance of introducing public policies aimed at the audiological monitoring for this syndrome, including otorhinolaryngological and audiological assessments that range not only the basics through pure tone and speech audiometry but also acoustic immittance measures and OAE analysis, even in patients without apparent complaints, especially in individuals with difficulties in answering accurately the pure tone audiometry.Furthermore, the audiological assessment in this study is limited to conventional frequencies. However, since the main involvement of individuals with WS seems to occur in the basal regions of the cochlea, monitoring through high-frequency (above 8 kHz) audiometry could also provide evidence of early alteration in the sensorial hearing organ, and an early diagnosis is important to better guide hearing-related care.It is also worth emphasizing the difficulty of parents and family members in noticing hearing loss. During the anamneses, none of the parents or caregivers reported considering that the participant had any type of hearing loss. Since hearing losses are generally mild, they can easily go unnoticed in daily life.Even to a mild degree, hearing loss can have a negative influence on the cognitive skills of attention and memory, compromise learning processes, affect language development, and jeopardize academic performance. Thus, despite being often disregarded, mild hearing loss can compromise the quality of life as a whole, thus requiring proper monitoring by professionals in the area.Individuals with WS have a high occurrence of hearing loss, especially mild sensorineural and at high frequencies, thus suggesting a dysfunction in hair cells, mainly in the basal regions of the cochlea.The TEOAE analysis presents some subclinical findings since the alteration in the TEOAE responses was observed in patients without hearing loss, thus representing an important clinical resource to be considered in the routine audiological assessment of these patients. ,2. Sua incidência é estimada entre 1:7.500 nascidos vivos e possui prevalência igual em ambos os sexos. O fenótipo da SW é caracterizado por múltiplas anormalidades físicas, neurológicas e sistêmicas.A síndrome de Williams (SW) é uma doença genética rara, decorrente de uma microdeleção na região do braço longo do cromossomo 7 (7q11.23), contendo aproximadamente 28 genes-5, sendo que este comprometimento inicia-se no fim da infância ou no começo da vida adulta e tende a ser progressivo,5-7.Quanto ao fenótipo audiológico, a perda auditiva neurossensorial, principalmente nas frequências altas, acima de 3 kHz, tem sido observada em diversos estudos com SWElastin (ELN), o gene General Transcription Factor (GTF2I) e o gene Lim Domain Kinase 1 (LIMK1). Diversos estudos têm observado a expressão desses genes em tecidos do sistema auditivo, prejudicando a irrigação sanguínea, o equilíbrio do gradiente iônico e a funcionalidade das estruturas cocleares,9,10. Considerando isto, a avaliação audiológica na SW, principalmente no que tange à funcionalidade coclear, é de muita importância para esta população.Afim de compreender este quadro, alguns estudos têm associado a influência de alguns genes frequentemente deletados em indivíduos com SW que podem contribuir para a perda auditiva destes pacientes, entre eles o gene tone burst, e o Produto de Distorção (EOAPD) onde dois tons puros de diferentes frequências são emitidos simultaneamente e a resposta refletida é um produto de distorção resultante da combinação dos dois tons.Uma forma de mensurar a funcionalidade das células ciliadas externas da cóclea é por meio da captação das Emissões Otoacústicas (EOA) que são respostas geradas pela liberação de energia destas células, que podem ocorrer de forma espontânea ou evocada por meio de algum estímulo acústico. Dentre os estímulos mais utilizados, está o Transiente (EOAT), que é uma estimulação breve por meio de cliques ou ,5,12,13. No entanto, estudos relacionados à audição em pacientes com SW ainda são recentes e escassos. Muitas hipóteses foram levantadas desde os primeiros estudos, afim de encontrar respostas que justifiquem o fenótipo comportamental desses indivíduos, no entanto, ainda pouco se sabe sobre a origem da maioria das características auditivas nesta população.Alguns estudos com EOA evidenciaram uma fragilidade colear, sendo que, mesmo indivíduos que apresentaram limiares auditivos dentro da normalidade, podem apresentar alterações cocleares com sintomas sub-clínicosDesta forma, o objetivo do presente estudo é caracterizar o perfil audiológico e avaliar a funcionalidade coclear em indivíduos com SW.Estudo clínico transversal, aprovado pela Comissão de Ética da instituição de pesquisa, sob número 15.825, realizado em indivíduos com SW (Grupo Estudo – GE), atendidos na Unidade de Genética do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo.Multiplex Ligation-dependent Probe Amplification (MLPA) e ter faixa etária entre sete e 17 anos. Quanto aos critérios de exclusão, foram considerados excesso de cera no meato acústico externo ou não realizar todos os procedimentos.Os critérios de inclusão dos indivíduos do GE foram apresentar diagnóstico confirmado da SW pelo Foi realizada uma análise clínica dos prontuários, por meio da qual 34 pacientes foram selecionados para o estudo; dentre estes, não foi possível obter contato com quatro pacientes, seis pacientes não aceitaram participar, um estava com excesso de cera no meato acústico externo e não retornou para avaliação após remoção, e um não permitiu realizar todos os procedimentos.Wechsler Abreviada de Inteligência (WASI), mostrou resultados entre 37 e 98 .Sendo assim, 22 pacientes foram incluídos no estudo, sendo sete do sexo feminino e 15 do sexo masculino, com idade cronológica entre sete e 17 anos  e idade mental entre três e 14 anos . A avaliação do Quociente de Inteligência (QI), por meio da escala Para fins de comparação dos resultados, um Grupo Controle (GC) foi composto por uma amostra de conveniência, contando com 17 indivíduos com desenvolvimento típico e com audição normal, sendo sete do sexo feminino e 10 do sexo masculino, com idade cronológica entre sete e 17 anos . Esses indivíduos não apresentavam queixas de atraso no desenvolvimento neuropsicomotor cognitivo, de fala ou escolar, que foi verificado por meio de anamnese com os pais ou responsáveis.; limiares auditivos obtidos pela audiometria tonal inferiores a 20 dB NA em todas as frequências testadas ; Limiar de Reconhecimento de Fala até 10 dB NA acima da média tritonal; e Índice de Percentual de Reconhecimento de Fala alcançando, no mínimo, 88% de acertos.Para descartar a presença de perda auditiva ou comprometimento de orelha média nos pacientes do GC, foi considerado como critério de inclusão: presença de curva timpanométrica tipo A, Ad ou Ar e reflexos acústicos presentes nas frequências de 0,5, 1, 2 e 4 kHz, ipsi e contralateraisAntes de iniciar os procedimentos, os pais leram e assinaram o Termo de Consentimento Livre e Esclarecido e os pacientes assinaram o Termo de Assentimento. Em seguida os pais responderam a uma anamnese audiológica para se obter informações do histórico dos pacientes quanto à fatores que pudessem interferir na avaliação, e foi realizada uma meatoscopia para verificar possível obstrução do meato acústico externo.Interacustic, modelo AT235, com sonda de 226 Hz. De acordo com a medida do pico de admitância, a curva timpanométrica foi caracterizada de acordo com os critérios de Jerger. Os reflexos acústicos, foram testados manualmente até a intensidade de 110 dB NA, e foram retestados em casos de ausência de reflexos para confirmação dos resultados.Para verificar possível comprometimento de orelha média, as medidas de imitância acústica foram realizadas com o Imitanciômetro marca Grason-Stadler, modelo GSI 61, com o paciente dentro de cabina acústica atendendo à norma ANSI S3.1-1991 de quantidade de ruído ambiental. Foram mensurados os limiares tonais por via aérea, nas frequências convencionais , sendo considerados como limiares auditivos normais valores até 20 dB NA. Quando qualquer frequência entre 0,5 e 4 kHz apresentava limiares superiores 20 dB NA, foi realizada a audiometria tonal por via óssea para determinar o tipo da perda auditiva. Para determinar o grau da perda auditiva foi considerada cada frequência separadamente, sendo: de 25 a 40 dB NA- grau leve; de 45 a 70 dB NA - grau moderado; de 75 a 90 dB NA - grau severo; a partir de 95 dB NA - grau profundo. Para a audiometria vocal, foi utilizada a pesquisa do Limiar de Reconhecimento de Fala (LRF), buscando a menor intensidade em que o indivíduo acertava pelo menos 50% das palavras trissílabas ou polissílabas faladas, e o Índice Percentual de Reconhecimento de Fala (IPRF), onde uma lista de 25 monossílabas era falada na intensidade de 30 dB NA acima do LRF, sendo considerados como normais resultados acima de 88% de acertos.A seguir, as audiometrias tonal e vocal foram realizadas utilizando Audiômetro clínico da marca Smart EP USB Jr da marca Intelligent Hearing Systems (IHS 5020) (Miami-Flórida) com fone 10D, posicionado no meato acústico do paciente com uma oliva de látex. Os parâmetros de coleta contemplaram a aquisição de 1024 estímulos clique com duração de 75 microssegundos, não linear, com taxa de apresentação de 19,30 estímulos por segundo, ganho de 4000 na intensidade de 80 dB NPS. A janela de análise foi mantida aberta em 30 ms, sendo analisadas as frequências de 1, 1,5, 2, 3 e 4 kHz. Para determinar a presença das EOAT, foi determinada estabilidade da sonda ≥70%, reprodutibilidade de resposta ≥50% e foi utilizado o seguinte critério: relação sinal ruído (S/R) maior do que 3 dB NPS para as frequências de 1 e 1,5 kHz e maior do que 6 dB NPS para as demais frequências. Para a Response, foi considerada a presença de resposta em pelo menos três bandas de frequência. Em pacientes que apresentaram EOAT ausentes, a sonda foi reposicionada e uma nova mensuração foi realizada, sendo considerada a melhor resposta obtida.As Emissões Otoacústicas com Estímulo Transiente (EOAT) foram mensuradas apenas nos pacientes que apresentavam limiares auditivos dentro da normalidade e foram realizadas com o equipamento 2) foi adotado para verificar a associação entre duas variáveis categóricas, tais como grupos e presença/ausência de resposta.Os dados coletados foram tabulados e foi realizada uma análise estatística descritiva e inferencial utilizando testes paramétricos, uma vez que a amostra seguiu uma distribuição normal. Assim, o teste T não pareado foi utilizado para comparar os grupos controle e estudo e o teste do qui-quadrado de Pearson . Os valores considerados estatisticamente significantes foram marcados com um asterisco (*).Embora apenas duas famílias tenham relatado ciência do paciente ter qualquer perda auditiva, observou-se no presente estudo 11 pacientes (19 orelhas) que apresentaram perda auditiva (50% da amostra) . Dentre No que tange ao grau da perda auditiva, observou-se que esta foi de grau leve na maioria dos casos, com limiares inferiores à 45 dB NA ; no entanto, observou-se alguns pacientes que apresentaram limiares auditivos superiores a este chegando até 65 dB NA  e ainda alguns que apresentaram limiares acima de 65 dB NA .Quanto ao tipo de curva timpanométrica, observou-se principal ocorrência da curva timpanométrica do tipo A , seguido da curva do tipo Ad e B  e por último da curva do tipo C observada em apenas um paciente em ambas as orelhas .Ao analisar a presença de perda auditiva de acordo com a idade cronológica, observou-se que os grupos (com perda auditiva x sem perda auditiva) não diferiram quanto à idade cronológica , sendo possível observar que os dados foram dispersos, não demonstrando nenhuma associação entre essas variáveis .Ao comparar os limiares auditivos entre ambos os grupos, observou-se limiares significativamente elevados no GE em comparação ao GC  ..Para não comprometer a análise da funcionalidade das células ciliadas externas, foram mensuradas as EOAT apenas nas orelhas que não apresentaram qualquer tipo de perda auditiva. Assim, avaliou-se 34 orelhas no GC e 25 orelhas no GE. Além disso, nenhum desses indivíduos apresentavam histórico de cirurgia otológica ou alteração de orelha média, que foi descartada pela presença de curva timpanométrica do tipo B ou C com ausência de reflexos acústicos ipsi ou contralateraisInicialmente foram calculadas as porcentagens de respostas presentes em cada frequência avaliada, para cada grupo. Foi verificada associação entre os grupos e a presença de resposta das EOAT, sendo que a ausência das EOAT foi um diferencial para o grupo com SW .A seguir, foram calculadas as medidas descritivas considerando a relação S/R para cada frequência em cada grupo. Observou-se diferenças estatisticamente significativas entre os grupos, sendo que a relação S/R foi menor no GE, em todas as frequências analisadas .O presente estudo teve por objetivo avaliar o perfil audiológico de indivíduos com SW e analisar a funcionalidade das células ciliadas externas da cóclea dentre pacientes que não apresentavam comprometimento de orelha média, histórico de cirurgia otológica ou perda auditiva. Além disso, por contar com um grupo controle, os resultados obtidos nas EOAT de indivíduos com SW puderam ser comparados com o de indivíduos com desenvolvimento neurotípico e sem comprometimento audiológico.Os achados desde estudo demonstraram importante prevalência de perda auditiva em indivíduos com SW, sugerindo que esta população apresenta uma disfunção das células ciliadas, principalmente das regiões basais da cóclea, sendo que este comprometimento coclear pode estar presente mesmo em pacientes sem nenhuma alteração aparente na avaliação audiológica básica., correspondendo de 25% dos indivíduos entre 6 e 14 anos à 59% dos indivíduos em idade escolar; em indivíduos adultos, a literatura demonstra presença de perda auditiva em mais de 38,46% dos casos avaliados.Os resultados obtidos por meio da audiometria tonal limiar demonstraram perda auditiva em 50% da população estudada com SW, o que corrobora com outros achado da literatura que relatam uma alta incidência de perda auditiva na SW,8,16,17, de grau leve a moderado-6 e com principal prejuízo das frequências altas,4,6-8,17.No que tange às características desta perda auditiva, observou-se que esta foi principalmente neurossensorial de grau leve para a maioria dos casos e acometendo principalmente as frequências altas, a partir de 3 kHz, o que corroborou também com os achados da literatura que descreve predominância da perda auditiva neurossensorial,18. No presente estudo nós observamos apenas 6 orelhas  com rebaixamento auditivo nas frequências de 4 kHz e/ou 6 kHz com recuperação na frequência de 8 kHz, sendo que algumas delas apresentaram reflexos presentes.Alguns estudos destacaram que a perda auditiva na SW tem configuração semelhante à da perda auditiva induzida por ruído, e que isto pode ser em decorrência da ausência de reflexo acústico, visto que o mecanismo fisiológico do reflexo pode proteger o sistema auditivo de sons de forte intensidadereactive oxigen species - ROS) pode ocorrer por descontrole das funções da mitocôndria, isquemia e excitação tóxica, levando a um aumento na produção de radicais livres deixando algumas regiões da cóclea, principalmente as células ciliadas externas, a estria vascular e as células ciliadas internas da base da cóclea, mais vulneráveis a lesões.Sabe-se que a perda auditiva induzida por ruído está relacionada ao estresse oxidativo. Assim, um aumento na produção de espécies reativas de oxigênio  é expresso em muitas regiões do corpo humano, incluindo a membrana timpânica, a tuba auditiva e os tendões dos músculos que sustentam a cadeia tímpano ossicular (tais como músculo do estapédio e tensor do tímpano),26. Assim, a ausência deste gene na SW pode impactar na mobilidade das estruturas do sistema tímpano-ossicular que conduzem o estímulo acústico para a orelha interna, gerando uma deficiência na funcionalidade da orelha média, e consequente perda auditiva condutiva.Alguns estudos observaram que o gene da elastina . Apesar disto, observou-se na Outra característica destacada na literatura é que a perda auditiva na SW é progressiva, principalmente para as frequências altas,5, mesmo em pacientes sem nenhuma perda auditiva evidente pela avaliação da audiometria tonal convencional,13,17,27.Ainda no que tange ao comprometimento da função coclear, os resultados do presente estudo demonstraram que indivíduos com SW apresentaram maior porcentagem de respostas ausentes e de menor amplitude das EOAT em comparação aos indivíduos sem a síndrome. Outros estudos publicados anteriormente já haviam observado ausência ou ainda diminuição das amplitudes das respostas das EOA,12,17. Destaca-se que no presente estudo observou-se comprometimento coclear em pacientes jovens (crianças e adolescentes), o que confirma mais uma vez que a disfunção das células ciliadas da cóclea se inicia precocemente.Alguns estudos também observaram atenuação das respostas das EOAT ao longo do tempoAcredita-se que a deleção de alguns genes na SW seja responsável pelo fenótipo comportamental que esses indivíduos apresentam e podem explicar alguns achados encontrados na avaliação audiológica.ELN também pode ser responsável por comprometer a função coclear, pois é capaz de reduzir a irrigação sanguínea na cóclea por estenose vascular, resultando em hipóxia e morte celular; também, pode enrijecer a membrana basilar, desregular a proliferação celular da cóclea, e ainda modificar a transdução do sinal das células ciliadas. Além disso, uma deficiência no gene da ELN pode levar à movimentação dessincronizada dos estereocílios, resultando em atraso na ativação do nervo coclear e em perda auditiva.Diversos estudos acreditam que o gene da ELN, o gene GTF2IRD1 foi observado nos tecidos neurossensoriais da cóclea (servindo como um receptor das células ciliadas), nos neurônios do gânglio espiral que são responsáveis por desencadear o potencial de ação para conduzir o estímulo auditivo para as vias auditivas centrais, na estria vascular, na membrana de Reissner e em vários outros tipos de células do órgão de Corti de ratos. Sendo assim, estas disfunções podem prejudicar a amplificação coclear por meio de um distúrbio no gradiente iônico, e contribuir para a perda auditiva verificada nos pacientes com SW.Além da LIMK1 tem sido associado à regulação da mobilidade das células ciliadas da cóclea. Desta forma, a deleção deste gene pode levar a uma disfunção dos mecanismos que regulam a mobilidade das células ciliadas e prejudicar a amplificação coclear, levando à perda auditiva precoce e progressiva comumente observada em indivíduos com SW.Também, o gene FZD9, que tem sido observado nos neurônios do gânglio espiral, e o gene STX1, que tem sido observado no gânglio espiral e na sinapse das células ciliadas do órgão de Corti.Além desses, outros genes que se encontram frequentemente deletados em indivíduos com SW, têm sido associados às alterações dos órgãos do sistema auditivo, tais como o gene ,13,17,27. Sendo assim, todas as famílias dos participantes do presente estudo foram orientadas quanto à importância do monitoramento anual da audição, e os pacientes com qualquer alteração na avaliação auditiva foram encaminhados para avaliação e conduta médica.Visto isso, diversos estudos destacaram a importância do monitoramento audiológico em pacientes com SW, sendo que a análise das EOA é um importante recurso a ser considerado na bateria de avaliação audiológica, por tratar-se de um método capaz de dar indícios de prejuízos na função das células ciliadas da cóclea, mesmo antes dos limiares obtidos pela audiometria tonal estarem alteradosConsiderando isto, em conjunto com os achados do presente estudo, deve-se destacar a importância da inclusão de políticas públicas voltadas ao monitoramento audiológico nesta síndrome, incluindo avaliação otorrinolaringológica, bem como, avaliação audiológica que contemple, além da avaliação básica por meio da audiometria tonal e vocal, e as medidas de imitância acústica, a análise das EOA, mesmo em pacientes sem queixas aparentes, e principalmente em indivíduos que apresentem dificuldades para responder fidedignamente à audiometria tonal.Além disso, o presente estudo foi limitado à avaliação audiológica nas frequências convencionais. No entanto, como indivíduos com SW parecem ter principal comprometimento das regiões basais da cóclea, o monitoramento por meio da audiometria de altas frequências (acima de 8 kHz) poderiam também dar indícios de alteração precoce do órgão sensorial da audição, e o diagnóstico precoce é importante visando melhor orientação quanto aos cuidados da audição.Cabe destacar, também, a dificuldade dos pais e familiares em perceber a perda auditiva. Durante a anamnese, nenhum dos pais ou responsáveis relataram achar que o participante da pesquisa teria qualquer tipo de perda auditiva. Como a perda auditiva geralmente é de grau leve, esta pode passar facilmente despercebida na vida diária.A perda auditiva, mesmo de grau leve, pode influenciar negativamente nas habilidades cognitivas de atenção e memória, comprometer o aprendizado, afetar o desenvolvimento de linguagem e prejudicar o rendimento acadêmico. Desta forma, a perda auditiva de grau leve, embora seja ignorada em muitos casos, pode comprometer a qualidade de vida como um todo, e por esse motivo é necessário que seja acompanhada com a devida importância por profissionais da área.Indivíduos com SW apresentam alta incidência de perda auditiva principalmente neurossensorial de grau leve e nas frequências altas, sugerindo uma disfunção das células ciliadas principalmente das regiões basais da cóclea.A análise das EOAT demonstrou achados subclínicos, visto que alteração nas respostas das EOAT foram observadas em pacientes sem perda auditiva, e assim é um recurso clínico importante a ser considerado na avaliação audiológica de rotina destes pacientes.


There are some motivational processes associated with the use of an Individual Sound Amplification Device (ISAD): acceptance, benefit and satisfaction.To make a cultural adaptation of the SADL (Satisfaction with Amplification in Daily Life) questionnaire to use with the Brazilian population; to assess its reproducibility and to describe its results in patients fit with an ISAD.Clinical study. Translation and cultural adaptation of the questionnaire; translation from English into Portuguese and linguistic adaptation; review of the grammatical and idiomatic equivalence; evaluation of the inter and intra-researcher assessment. There were 30 ISAD users older than 18 years of age participating in the study (mean: 66.36 years); 19 men (63%); 11 women (37%).The participants reported greater satisfaction (mean) for questions: 1 (6.35); 3 (6.85); 5 (6.10); 6 (6.80); 8 (6.33); 9 (6.80); 11 (6.16); 12 (6.93) and 15 (6.52). Less satisfaction: question 4 (3.16); 7 (3.41) and 13 (2.83). The comparison of each question with the first and second application of the questionnaire did not present statistically significant results, thus yielding good reproducibility. There was a greater satisfaction for subscales: Positive Effect (6.50) and Services and Costs (6.26) and less satisfaction for Negative Factors (4.73).It was possible to adapt the questionnaire for the Brazilian population. Hearing is fundamental for human life; it plays an important role in society, because it is the very basis of human communication development. Individuals with hearing impairment may suffer severe loss in their social, psychological and professional lives, also having feelings of insecurity, fear, depression, isolation and also family tensions because of the lack of attention affecting those with hearing disorders.The problems caused by sensorial deprivation may be minimized with the use of an Individual Sound Amplification Device (ISAD), which helps rescue speech sound perception, and also that of environmental sounds, promoting an improvement in communication skillsIn the year 2000, the Ministry of Health (MH) approved Ordinance SAS#4327Thus, the Individual Sound Amplification Device provision program proposes care for children and adults with hearing impairment concerning the functional use of their auditory reserve, enabling the use of residual hearing and helping the individual interact with society.During the process of advising hearing impaired individuals, we must be concerned with three motivational processes associated with the use of a sound amplification device: acceptance, benefit and satisfactionWe can say that the checking procedures, such as functional gain and insertion measures, are not enough to assess the patient's satisfaction with the hearing aid in daily communication situations. There was a growing interest in developing validation procedures which would allow the assessment of the user's benefit outside the clinical setting, made up of self-assessment questionnairesSelf-assessment is a simple, fast and efficient procedure, which enables the evaluation of the individual during his/her fitting process. This procedure enables the comparison between different devices and/or calibrations, as well as the assessment of the benefits achieved with the hearing aid along time, enabling the user to recognize the advantages provided with the fitting of an individual sound amplification device (ISAD) in relation to his-her hearing difficulties and psychosocial disadvantages. Thus, by means of questionnaires which enable measuring these auditory difficulties or handicaps, it is then possible to optimize the time necessary for one to adapt to sound amplificationSatisfaction is the measure of the auditory rehabilitation outcome, which aims at encompassing the most complete constellation of factors which are needed for the final result, since the variable of interest is the patient's opinion and it is not associated only with the ISAD performance, depending solely on the person's perceptions and behaviorsThere are numerous assessment tools based on scales to assess the individual's level of satisfaction, because there are many factors impacting different dimensions associated with ISAD useAbbreviated Profile of Hearing Aid Benefit), the HHIE (Hearing Inventory for the Elderly) and the HHIA (Hearing Handicap Inventory for Adults), which were translated and adapted to our country settings, are used to investigate the level of user satisfaction, the benefits obtained from the use of hearing aids and the reduction in hearing impairment with the use of the ISAD, and others which aimed at comparing the benefits of different technologies and to check the hearing aid fitting by means of objective and subjective measuresIn Brazil, some self-assessment questionnaires, the APHAB , Service and Costs , Negative Factors  and personal image (3 items associated with cosmetic factors and the very stigma associated with using an ISAD) .Considering the 15 items present in the SADL, in 11 of them the score provided by the individuals coincided with the scoring scale and in the other 4 items  there was an inverse relation between the score and the scale . The higher the numeric results obtained by the mean values of the answers from each subscale, the greater the individual satisfaction. Questions 1,3,5,6,9 and 10 belong to the Positive Effects subscale; questions 2,7 and 11 belong to the Negative Factors subscale; questions 4,8 and 13 belong to the Personal Image subscale; and questions 12,14 and 15 belong to the Service and Costs subscale.Translation from English into Portuguese and linguistic adaptationBrazilian Satisfaction With Amplification in Daily Life Questionnaire, or Brazilian SADL , who analyzed the three resulting documents and, by consensus, reduced the differences found in the translations, choosing the best words and expressions used to translate all the questions, adapting the text to the Brazilian Culture. This stage was based on choosing the best translation for the questions and the modification by approximation of the most adequate terms, chosen to enable full understanding by the Brazilian population. Thus, we obtained and new and single questionnaire which was called ian SADL .Review of the idiomatic and grammar equivalence Following, a copy of the Brazilian SADL was sent to three other Portuguese-English translators with the same language expertise of the first ones. These translators, without knowledge of the original text, translated it back into English. We did not allow these new translators to have access to the original text - written in English, in order to avoid any influence on their translation of the words. The same revisers assessed the three resulting versions, comparing them with the original document in English.The cultural adaptation of the Brazilian SADL aimed at establishing the cultural equivalence between the English and the Portuguese versions of the questionnaire. According to Guillemin et al. (1993)In order to test inter-researcher reproducibility, the questionnaire was used with the same thirty interviewed patients in the phase of cultural adaptation by a second interviewer (interviewer 2). On the same day of the interview the questionnaire was employed again by the first interviewer (interviewer 1) in order to assess intraexaminer reproducibility.In order to obtain the results from the mean, median, minimum and maximum values and standard deviation for each question on the questionnaire in both applications of it we used a descriptive statistical analysis. As far as reproducibility was concerned, we used the Spearman Coefficient Test and, to compare each application of the SADL we used the Wilcoxon test.In the present study, the mean age of the participants who answered the SADL was 66.36 years of age, ranging between 19 and 88 years. As to gender, we had 19 men (63%) and 11 women (37%).The ISAD users who participated in this study showed a greater satisfaction level associated with questions: 1 (mean: 6.35), 3 (6.85), 5 (6.10), 6 (6.80), 8 (6.33), 9 (6.80), 11 (6.16), 12 (6.93) and 15 (6.52), while a lower satisfaction rate was found with the following questions 4 (3.16), 7 (3.41) and 13 (2.83). We compared each question in terms of the first and second questionnaire application and we did not observe statistically significant results .Table 1DThe results associated with the questionnaire subscales revealed a greater satisfaction associated with the Positive Effect (6.50) and Service and Costs (6.26) subscales. We observed a lower satisfaction level associated with the Negative Factors subscale (4.73) .Table 2DThese results indicated good questionnaire reproducibility.The users were considerably pleased with their ISAD use.The hearing loss may be considered as one of the most disabling sensorial changes that affect human beings. Facing such change, we noticed a reduction in social contacts, yielding emotional disorders, which often times can be devastating for this populationAmong the currently available technologies and resources, the ISAD cropped up as a means to minimize the hearing loss effects.The Brazilian literature has some self-assessment questionnaires which were translated and adapted to our country, used to investigate the level of satisfaction, the benefits obtained from the use of ISAD and the reduction in auditory handicap with the use of amplification.For this study we selected the SADL questionnaire in order to check its real distribution in the clinical practice, to assess the level of satisfaction of the individual users of ISAD and to culturally adapt the SADL in order to apply it to the Brazilian population.The SADL tool has 15 questions broken down into four subscales: Positive Effects , Services and Costs , Negative Factors  and Personal Image (3 items associated with aesthetic factors and the very stigma of using ISAD) .The mean age of the participants who answered the SADL was 66.36 years, ranging between 19 and 88 years of age. As far as gender is concerned, our study counted on 19 men (63%) and 11 women (37%).ISAD users who participated in this study showed a greater user satisfaction associated with the following questions: 1 (mean: 6.35), 3 (mean: 6.85), 5 (mean: 6.10), 6 (mean: 6.80), 8 (mean: 6.33), 9 (mean: 6.80), 11 (mean: 6.16), 12 (mean: 6.93) and 15 . Considering less satisfaction, we found the following results: question 4 (mean: 3.16), 7 (mean: 3.41) and question 13 (mean: 2.83) . We compResults associated with the questionnaire subscales revealed a greater user satisfaction for the Positive Effect (mean: 6.50) and Services and Costs (mean: 6.26) and less satisfaction for the Negative Factors subscale (mean: 4.73) . We did Having these results, it is important to mention that to monitor patient satisfaction is fundamental to assess clinical procedures, to obtain quality assurance and because satisfaction reflects the true results of a health care initiative. As we identify the factors which may contribute to patient satisfaction and as we attempt to provide such attributes to the processes involved, we have the potential to obtain a more effective outcome at the health care servicesFindings indicated a large user satisfaction for the Positive Effect subscale, and this fact is corroborated by other studiesISAD users in this study did not complain of device repair, and they were very much pleased in relation to question 15 (mean: 6.57). . ResearcIn regards of the ISAD fitting time and SADL application, we found the use of the questionnaire after six weeks of fittingWe also noticed a high satisfaction rate in relation to the Services and Costs subscale, indicating that the users did not complain of acoustic issues, in relation to the audiologist's competence, and the quality of the ISAD . In one The items in the Negative Factors subscale which investigate the performance of users in a noisy environment, feedback and the use of telephone were listed as dissatisfactory by the users of hearing aid in the study and such domain was then created by the authors as a thermometer of fitting problemsAs to speech understanding under noise, the authorsIt was noticed that the difficulty in using the telephone was considered one of the most important complaints in the SADL questionnaire seen in the studiesIn regards of question four (4), which is associated with the hearing aid image and the stigma of hearing loss, it was considered non-satisfactory by the participants in this study. In a given studyDuring our bibliographic survey on SADL, we noticed that this questionnaire was very useful in different situations in order to learn about the degree of satisfaction of individuals with hearing loss, since the literature showed its efficacy to check the satisfaction of mothers of children using ISADsMoreover, some studies tell us that the SADL may be considered a gold standard evaluation tool to learn about the satisfaction of ISAD usersFinally, the benefits of hearing aids are associated with an improvement in communication in daily life, including the reduction in the auditory handicap of ISAD users. Results with the hearing aid go beyond its benefits, and satisfaction stands out as a more reliable measure of such results, because it encompasses a number of factors, it bears a dynamic character, and it depends on the user's perception and behavior, which is not associated only with the ISAD performance•We did not find statistically significant results among the questionnaire's subscales;•Reproducibility was considered satisfactory;•This study yielded a good performance to identify the questionnaire efficacy, because the users were considerably satisfied with the ISAD.SATISFACTION WITH AMPLIFICATION IN DAILY LIFE ATISFACTION WITH AMPLIFICATION IN DAILY LIFE WITH AMPLIFICATION IN DAILY LIFEName ____________________ Date of Birth___/___/___ Today's Date___/___/___INSTRUCTIONSListed below are questions on your opinions about your hearing aid(s). For each question, please circle the letter that is the best answer for you. The list of words on the right gives the meaning for each letter.Keep in mind that your answers should show your general opinions about the hearing aids that you are wearing now or have most recently worn.A Not At AllB A LittleC SomewhatD MediumE Considerably1Compared to using no hearing aid at all, do your hearing aids help you understand the people you speak with most frequently?A B C D E F G2Are you frustrated when your hearing aids pick up sounds that keep you from hearing what you want to hear?A B C D E F G3Are you convinced that obtaining your hearing aids was in your best interests?A B C D E F G4Do you think people notice your hearing loss more when you wear your hearing aids?A B C D E F G5Do your hearing aids reduce the number of times you have to ask people to repeat?A B C D E F G6Do you think your hearing aids are worth the trouble?A B C D E F G7Are you bothered by an inability to get enough loudness from your hearing aids without feedback (whistling)?A B C D E F G8How content are you with the appearance of your hearing aids?A B C D E F G9Does wearing your hearing aids improve your self-confidence?A B C D E F G10How natural is the sound from your hearing aids?A B C D E F G11How helpful are your hearing aids on MOST telephones with NO amplifier or loudspeaker?A B C D E F G12How competent was the person who provided you with your hearing aids?A B C D E F G13Do you think wearing your hearing aids makes you seem less capable?A B C D E F G14Does the cost of your hearing aids seem reasonable to you?A B C D E F G15How pleased are you with the dependability (how often they need repairs) of your hearing aids?A B C D E F GPlease respond to these additional items.For audiologists use only.Directional Microphones T-coilMultiple Microphones Peak ClippingMulti-channel Compression LimingContinues in Attachment 1Remote Control TILLMulti-program WDRCNo volume control BILLOtherSATISFAÇÃO COM AMPLIFICAÇÃO NA VIDA DIÁRIANome: _____________________ Data de Nascimento __/__/__Data de Hoje __/__/__INSTRUÇÕESAs questões listadas abaixo se referem as suas opiniões sobre o seu aparelho auditivo.Para cada questão, por favor, circule a letra que responde melhor a questão para você. A lista à direita fornece o significado de cada letra.Lembre-se que suas respostas devem mostrar suas opiniões gerais sobre o aparelho auditivo que você está usando atualmente ou que tem usado mais recentemente.A NãoB Muito poucoC PoucoD MédioE Ás vezesF Quase sempreG Sempre1O seu aparelho auditivo lhe ajuda a entender as pessoas com as quais você fala quando comparado a época que não usava aparelho auditivo?A B C D E F G2Você fica frustrado quando o seu aparelho auditivo capta sons que lhe impedem de ouvir o que você quer?A B C D E F G3Você está convencido de que a obtenção de seu aparelho auditivo fazia parte dos seus maiores interesses?A B C D E F G4Você acha que as pessoas percebem mais a sua perda auditiva quando você usa o aparelho?A B C D E F G5O seu aparelho auditivo reduz o número de vezes que você tem que pedir para as pessoas repetirem?A B C D E F G6Você acha que vale a pena usar o aparelho auditivo?A B C D E F G7Você se sente incomodado quando necessita aumentar o volume e ocorre a microfonia?A B C D E F G8Você está contente com a aparência do seu aparelho auditivo?A B C D E F G9O uso do seu aparelho auditivo melhora a sua autoconfiança?A B C D E F G10O som do seu aparelho auditivo é natural?A B C D E F G11O seu aparelho auditivo é útil na MAIORIA dos telefones sem amplificador ou caixas de som?A B C D E F G12A pessoa que lhe forneceu o aparelho auditivo era competente?A B C D E F G13Você acha que usar o aparelho lhe faz parecer menos capacitado?A B C D E F G14O custo do seu aparelho auditivo parece razoável para você?A B C D E F G15Você está satisfeito com a frequência com a qual seu aparelho auditivo precisa de reparos?A B C D E F GPor favor, responda os itens adicionais.Microfone Direcional Peak ClippingMicrofones Múltiplos Limitação por CompressãoMulticanal TILLControle Remoto WDRCMultiprograma BILLSem controle de volume T-CoilOutros


Otoacoustic Emissions are generated by the cochlea in response to sound stimuli. They can be generated by clicks or specific frequency stimuli, such as tone pips. This is a quick and objective test with several applications.To investigate the influence of the type of stimulus achieving otoacoustic emissions in individuals with mild and moderate sensorineural hearing loss of sloping configuration.Thirty-two male and female patients aged from 17 to 63 years, with symmetric sensorineural hearing loss with a sloping configuration were evaluated. All subjects underwent transient otoacoustic emissions testing elicited by clicks and 2.000Hz and 4.000Hz tone pips.The degree of hearing loss and gender influenced otoacoustic emissions; it was significant for click stimulus and tone pips at 2.000Hz. Emissions were absent more often in females with both procedures.Otoacoustic emissions evoked with clicks coincided with the emissions of tone pips at 2.000Hz. Tone pips at 4.000Hz were more sensitive than clicks for detecting impairment in individuals with high frequencies hearing loss. Gender and the degree of hearing loss ere factors that affected OAE registration. Otoacoustic emissions (OAEs) consist of low intensity sound energy that after amplification may be recorded in the outer ear canal. OAEs originate in the cochlea, specifically in outer hair cellsOAEs are an important tool for objectively evaluating the function of the inner ear, especially the outer hair cells of the cochleaIt is a quick, objective, non-invasive, and easily applied procedure that may be done at any ageOAEs are not used for quantifying hearing loss, but rather to detect their presence, as they are present in all normally functioning ears. OAEs become absent when behavioral auditory thresholds are above 30 dBNA, that is, when there is any degree - even if mild - of hearing lossOAEs have several clinical uses, including neonatal and school-age hearing screening, in the differential diagnosis of cochlear and retrocochlear hearing losses, in monitoring subjects exposed to noise or ototoxic drugs, in analyzing and defining the progression of sudden and progressive hearing loss, and in studying cochlear dysfunction. OAEs may be tested in all age groups, including those that are hard to testTransient otoacoustic emissions (TOAEs) are responses evoked by short stimuli, such as clicks at 500 to 6,000 Hz. OAE responses may be seen per frequency band. However, these responses are due to a filtering process in the equipment, which separates them by frequency band. Click-generated responses do not necessarily match the cochlear region involved in that specific frequency; they reflect a global cochlear responseA more specific method for evaluating OAEs by frequency is to use a tone pip stimulus. Tone pips present the energy as a single frequency pure tone uncontaminated by the frequencies of adjacent energies. This stimulus causes the basilar membrane of the cochlea to move in the frequency cochlear region that coincides with the stimulus frequencyLow frequencies are preserved - or at least with lower auditory thresholds - in sloping sensorineural hearing loss. Patients with this type of hearing loss may present TOAEs when stimulated by clicks because of lack of frequency selectivity in responses.The purpose of this study was to compare TOAEs with clicks and tone pips at 2,000 Hz and 4,000 Hz in subjects with mild and moderate sloping sensorineural hearing loss; the variables were gender, degree of hearing loss, and first ear to be tested.The institutional review board analyzed and approved the study design (no. 01988/08). All subjects were informed beforehand about the procedures, and participants signed a free informed consent form.Inclusion criteria were: being a patient of the institution, having mild or moderate bilateral sloping sensorineural hearing loss, presenting a bilateral type A tympanometric curve, and aged from 15 to 65 years.A clinical history was taken of the selected patients, followed by visual inspection of the outer ear canal, pure tone and voice audiometry, acoustic immittance testing, and recording of TOAEs with clicks and tone pips.OAEs were recorded by placing a probe in the outer ear canal - this probe has a miniature microphone that seals the outer ear canal with an olive-shaped tip. The stimulus passes from an amplifier to an analog-digital converter and a digital signal processor, which makes it possible to analyze the recorded sounds spectrally and to separate them from other sounds generated in the body.TOAEs were recorded following a click and then a tone pip at 2,000 Hz and 4,000 Hz. Testing took place in a non-acoustically lined silent room; the testing device was an ILO 88 - otoacoustic emissions analyzer  coupled to a microcomputer. The probe was placed only once; OAEs were recorded with both stimuli first on one side and then on the other. Testing started on the right ear in half of the sample, and on the left ear in the other half.A 260 click recording was used; the gain was adjusted to keep stimulus intensities between 75 and 85 dB in the full-menu mode (20 ms window). The criteria for the presence of responses was a general reproducibility correlation of at least 50%, by tested frequency , and general amplitude (response), and wave stability above 70% with both stimuli.menu”, “stimulus”, “tone pip” and entered the frequency at which testing was done - first 2,000 Hz, then 4,000 Hz.OAEs with tone pips required the equipment to be modified. Before testing, the examiner selected on the screen: “st and 3rd quartiles, and confidence intervals at a 95% statistical confidence level.Statistical analysis of the data aimed at comparing OAEs obtained with clicks and tone pips at 2,000 Hz and 4,000 Hz. The equality of two proportions test was applied, as well as measurements of the median, standard deviation, 1Results where statistical significance was attained are marked with the symbol (*); the p-value was 0.05.The results of comparing TOAEs evoked by clicks and tone pips at 2,000 Hz and 4,000 Hz are presented below.The response amplitude ranged from 4.3 dB to 17.8 dB (mean - 8.19 dB), and was higher with clicks, followed by tone pips at 2,000 Hz and by tone pips at 4,000 Hz. Similarly, reproducibility was higher with OAEs evoked by click, followed by tone pips at 2,000 Hz and tone pips at 4,000 Hz; it ranged from - 67% to 84% (mean - 3.6%). Stability was higher than 70% (mean - 84.7%), and the stimulus level was between 75 dB and 85 dB (mean - 79.18 dB) for three stimuli according to click parameters defined in the literature.This Table shows that OAEs evoked by clicks and tone pips at 2,000 Hz were similar. There was a high rate of absent responses for OAEs evoked by tone pips at 4,000 Hz; this was compatible with the type of hearing loss that was studied - auditory thresholds at high frequencies were above 25 dB, where OAEs are not expected.p-values of The variable gender affected the recording of OAEs; it was statistically significant for OAEs evoked using clicks and tone pips at 2,000 Hz, with more absent results in women for clicks and tone pips at 2,000 Hz. There were more absences than presences of OAEs with tone pips at 4,000 Hz in both sexes, which was not statistically significant.The variable degree of hearing loss affected the recording of OAEs; it was statistically significant for clicks and tone pips at 2,000 Hz, where there was complete agreement between both stimuli types. In both cases, emissions were present in subjects with mild hearing loss, and absent in subjects with moderate hearing loss. The comparison between OAEs evoked by clicks ant tone pips at 4,000 Hz showed that emissions were absent in most cases of moderate hearing loss.This study showed that the amplitude of TOAEs evoked by specific frequency stimuli (tone pips) decreased as the stimulus frequency increased, which concurred with another published studyClicks of any intensity for evoking OAEs are powerful activators of efferent activity, while tone bursts and/ or tone pips activate this system less effectively, as they stimulate fewer efferent fibersAs in our study, McPherson et al. (2006) included tone burst-evoked OAEs in their protocol to reduce falsepositive results in neonatal screening. These authors found, however, that tone burst responses at 1,000 Hz, 2,000 Hz, and 3,000 Hz were consistently higher and more effective compared with clicksAccording to some authors, tympanometric measures and the intensity of the recorded stimulus in the outer ear canal affected the response level of TOAEs (significant positive correlation)A few authors have found consistent responses for emissions using tone bursts at 1,000 Hz, 2,000 Hz and 4,000 Hz in normal-hearing and hearing-impaired children. These authors found that tone bursts at 2,000 Hz and 4,000 Hz discriminated normal and altered ears more effectively, and concluded that tone bursts were superior to clicks for identifying hearing lossOur findings differ from those of a few authors that recorded TOAEs in young adultsA study of OAEs evoked by tone bursts showed advantages for the right ear, female gender, and neonates with no risk indicators for hearing loss, but which was not statistically significantThe hearing level was also a significant factor in some published papers and in the present study; subjects with more severe hearing loss did not respond . We founOur findings concur with those of other authors that tested OAEs in normal-hearing subjects and in patients with sensorineural hearing loss. These authors found that OAEs were present in normal-hearing individuals, and absent in subjects with auditory thresholds worse than 35 dBThe present study is very important for clinical practice, as it suggests that recording OAEs with clicks combined with tone pips add to the hearing loss detection protocols. This may reduce the need for second visits for retesting, and decrease false-positives, especially in subjects with mild and moderate sloping sensorineural hearing loss.•Click-evoked OAEs coincide with OAEs evoked by tone pips at 2,000Hz.•The variable degree of hearing loss affected the recording of OAEs, and was statistically significant for recordings with click and 2,000 Hz tone pip stimuli.•Gender affects the recording of OAEs, and was statistically significant for recordings with click and 2,000 Hz tone pip stimuli; there were more absence of OAEs in female subjects in both procedures.Based on an assessment of subjects with mild and moderate sloping sensorineural hearing loss using TOAEs evoked by clicks and tone pips at 2,000 Hz and 4,000 Hz, we concluded that:


Noise-induced hearing loss (NIHL) is a common occupational hazard and a major cause of deafness among airport workers. However, few studies have been conducted to investigate the various risk factors related to hearing loss. Purpose: the purpose of this study was to measure the prevalence and risk factors of NIHL among Muscat International Airport airside workers. Method: Their daily noise exposure level at the airport was measured, and the time-weighted average (TWA) was calculated for each airside department. A cross-sectional study design involving 312 workers from the eight departments of the airport was chosen and the prevalence of NIHL among workers was assessed. The study participants then completed a self-administered questionnaire that covered their socio-demographic characteristics, occupational exposure history and the health-related risk factors of NIHL. Results: The TWA recorded for the workers was above the accepted limit in some departments, namely, cabin appearance, ramp, line maintenance and hangar. The prevalence of NIHL among participants was 21.79% (n = 68). Of these 68 participants with NIHL, 22.30% were exposed to job-related high noise levels. NIHL was common among participants aged 40 or above  and high school degree holders , as well as those who were exposed to higher noise levels  or who did not wear their hearing protection devices (HPDs) regularly . Conclusion: around a quarter of our study participants who were exposed to high noise levels suffer from NIHL. Noise-induced hearing loss (NIHL) is the second most common form of sensorineural hearing loss, after presbycusis , and is one of the most common occupational diseases worldwide . The WorUnfortunately, continuous and prolonged exposure to loud noise can kill nerve endings in the inner ear, resulting in permanent hearing loss that cannot be corrected via surgery or medication. Hence, this limits the ability to hear high frequency sounds and understand speech, which seriously impairs a person’s ability to communicate . The damSeveral studies have been conducted to measure the prevalence of NIHL in several international airports. In a 2014 Korean study, the prevalence of NIHL was 49.4% . In a SuIn Oman, Muscat International Airport has a newly opened airport building that was built over the last two years based on international standards to minimize noise levels airside . HoweverA cross-sectional study was conducted airside in the Muscat International Airport from July 2019 to August 2020 after obtaining both permission from the companies that worked there as part of the Oman Aviation Group and ethical approval from the Research and Ethical Review Board (MoH/CSR/19/10615) in Oman. The guidelines of the Declaration of Helsinki were used to inform the participants about the study; it was ensured that all information was in their language, including the purpose of the study, and that all information obtained in this study would be kept strictly confidential and anonymous. Airports are divided into landside and airside areas. The landside area is open to the public, whereas access to the airside area is tightly controlled. The airside is the part of an airport used by aircraft for loading, unloading and takeoffs, and includes runways, taxiways, the ramp, the storage hangar and aircraft maintenance facilities. The ramp is where aircraft are parked, unloaded or loaded, refueled, boarded or maintained. Line maintenance is any maintenance task that can be performed for the aircraft outside of a hangar in a short period, and is usually conducted on the ramp, whereas the hangar is where any longer aircraft maintenance is carried out. The maintenance department includes all maintenance work to ensure the safe operation of civil, mechanical, electrical and building equipment, infrastructure and facilities, and to ensure compliance with standards. Airfield operation workers coordinate between air-traffic control and maintenance personnel by dispatching, using airfield landing and navigational aids, implementing airfield safety procedures and monitoring. The cabin appearance department is responsible for cabin cleaning operations in aircrafts in ramp areas. The workshop department maintains equipment at the workshop, and lastly, there is the fire department which will respond to any incident and is usually located near the runway.The study population was stratified to cover the eight departments at the airside of Muscat International Airport .This study was carried out in two steps. Firstly, workers’ daily exposure to noise from the airport airside was measured using a time-weighted average (TWA) sound level of 85 dB for 8 h per workday and a 3 dB exchange rate , as this is the noise exposure limit recommended by the National Institute for Occupational Safety and Health (NIOSH) . The TWAmax.), minimum sound level (Lmin.) and the level of the time-weighted average (LTWA) were taken at the end of each shift before calculating the mean of these measurements for several shifts. Based on TWA readings, the employees were divided into employees with a low noise exposure level  and high-level exposure .The second step was subdivided into two steps: second step A and second step B. In the second step A, data were collected from participants (n = 390) using a self-administered questionnaire, optionally validated by three otolaryngology (ORL) and occupational medicine consultants and divided into three sections. The first section was about a participant’s socio-demographic characteristics such as age , nationality , gender, marital status  and education level , high school degree and lower than high school degree ). The second section was about the risk factors of NIHL, including family history of ear disease , living near noisy areas , current occupation location , daily time spent on the job, years of working at the airport, chemical exposure , hobbies , smoking status  and current smoker (who is still smoking during the study)), previous medical illnesses such as diabetes, hypertension and others  and usage of ototoxic drugs such as aspirin, carbamazepine, amitriptyline and others . The third section was about evaluation of noise risk at the workplace, including difficulty of communication due to noise , wearing of hearing protective devices , no), time of wearing HPDs  and available methods of HPDs for the current job .In the second step B, a substudy was designed to screen for noise-induced hearing loss among the airside workers. This substudy sample was stratified to cover the eight departments at the airside of Muscat International Airport . These airside workers were chosen randomly from a name list while considering shift times to cover all shifts and days of the week. The sample size was calculated using Epi Info software version 22, knowing that the total population size was n = 1948, assuming a 5% margin of error and 95% CI, and using a prevalence equal to that of the prevalence of NIHL at King Khalid International Airport in Saudi Arabia, which was found to be 48% . AccordiThe excluded participants were those who had not yet completed 6 months in the airside area, workers with pre-employment ear disease or ear deformity, workers with previously diagnosed conductive hearing loss, workers with a history of a previous occupation with a high noise level exposure or those who were wearing medical hearing aids.The prevalence of noise-induced hearing loss was determined based on the positive results of an audiogram of the pure audiometry test for more than 25 dB in high frequencies of 3–4 kHz and 6 kHz, interpreted by the researchers. The pure audiometry screening test has a sensitivity of 92% and specificity of 94% in detecting sensorineural hearing impairment  and in dTWA for each shift per department. The chi-square test and Fisher’s exact test were used as appropriate to investigate the differences in the individual characteristics between the groups of our study participants. Univariate logistic regression models were used to estimate the risk of hearing loss in relation to several risk factors before these models were adjusted for age- and job-related noise exposure level. During the analyses, a p-value < 0.05 was considered significant.The analyses were performed using the SPSS version 22 software. The mean and standard deviation were calculated for the LTWA = 84.7 dB, SD = 3.96, lmin. = 60.70 lmax. = 114.05), whereas the lowest was reported among airfield department employees during their night shift . On the other hand, when considering eight-hour shift workers, the highest noise exposure level was reported for cabin appearance department employees during the night shift , whereas the lowest noise exposure level was reported among the workshop department employees during their 8 h morning shift . Based on these reported average LTWA measurements, which are presented in The airside workers’ noise exposure levels are summarized in The number of included participants was 312 out of 390 potential participants approached, with a response rate of 80%. Looking at the rate of NIHL among the participants, the audiometry test revealed that there were 68 cases, and of these 68 cases, 76.47% were exposed to high noise levels. Furthermore, 5.13% (n = 16) of the participants had a family history of hearing loss, 11.53% (n = 36) were current smokers, 9.29% (n = 29) lived near the airport, 16.99% (n = 53) practiced hobbies with possibly high noise levels , 12.82% (n = 40) had a medical condition that might affect their hearing and 7.37% (n = 23) used medication that might affect their hearing.p < 0.004) and in participants older than 40 years old (x2 (2) = 57.35, p < 0.001). Additionally, NIHL was more common among participants who held a high school qualification  = 42.65, p < 0.001), as shown in The mean work experience of our participants was 10.53 years (SD = 7.46) and NIHL was more common among participants with 10 years or less of work experience  = (44.12), As shown in TWA was above the accepted limit among the workers in the cabin appearance, ramp, line maintenance and hangar departments, which might be due to noise exposure in the working environment and peak exposures to noise, as well as the time spent at work.Measurements of noise exposure at Muscat International Airport showed that the mean LAdditionally, in this study some departments  reported a high peak of noise during some shifts (>100 dB), which might be sufficient to cause hearing loss as the workers need only 15 min of this level of noise to start developing NIHL based on the 3 dB exchange rate  ). The stThe prevalence of NIHL among the total study population of workers was 21.79%, whereas it was 5.13% in employees with low noise exposure and 16.67% in employees with high noise exposure. Comparing these results with the reported prevalence of NIHL in the literature, which was around 33.5–49.4%, the reported NIHL prevalence in this study was low . It migh16 which emphasizes the importance of frequent hearing screening for workers to detect and prevent future permanent nerve damage due to noise.Studies have revealed that several factors could contribute to the development of hearing loss besides high noise exposure levels, such as aging and prolonged working experience . Nasir aOther factors that may affect the severity of NIHL and elevate its risk in an occupationally noise-exposed worker is the presence of chronic diseases such as diabetes, as well as smoking—especially if smoking more than 20 packs per year ,19. In tIn our study, most airside airport workers infrequently wore HPDs (such as earmuffs and ear plugs). Studies have shown that infrequent usage of HPDs with a high occupational level of noise exposure might be a contributing factor for hearing loss .In this regard, it was recommended by the Occupational Safety and Health Administration (OSHA) that airport employers should provide HPDs to all their employees, who must use them whenever noise exposure meets or exceeds 85 dB . In addiUsing a cross-sectional design did not allow us to establish a causality relationship in addition to errors that arose from recall bias, especially in relation to the magnitude and frequency of noise exposure.In conclusion, although the airport is well established and has the highest standards of noise reduction measures, the noise exposure was just above the accepted level in some departments and included some very high peak exposures in others, which is relevant to our results concerning NIHL. In addition to noise exposure level as a predictor for hearing loss, NIHL was found to be related to older age, lower educational achievements, fewer years of experience and irregular wearing of hearing protection. This study highlighted the importance of further preventive measures, including regular audiometric screening for early detection of hearing loss and ongoing hearing protection conservation programs. In addition, the study demonstrated the importance of administrative controls and engineering controls, such as having SOPs (standard operating procedures) to minimize work hazards in the workplace, employee training and education, frequent airport machine maintenance, regular inspection, recording and supervision, and advisors emphasizing to their workers that they must always wear their HPD in the proper manner during any noise exposure.