Different Types Of Hearing Loss Research Articles

The prevalence of hearing loss in infants hospitalized in the neonatal intensive care units

Background and Aim: Studies have shown that several factors affect the hearing loss of infants hospitalized in the neonatal intensive care unit (NICU). These factors include hyperbilirubine­mia, low birth weight, asphyxia, and prematurity. This study aimed to investigate the prevalence of hearing loss and its risk factors in NICU infants.
 Methods: This is a cross-sectional study. Sam­ples were 159 infants admitted to the NICU in Fatemieh and Shahid Beheshti hospitals in Ham­adan, Iran, who were selected using a conveni­ence sampling method. Their hearing was scree­ned using transient evoked otoacoustic emission (TEOAE) and automated auditory brainstem res­ponse (AABR) tests; in case of failure in these tests, auditory brainstem response (ABR) was performed.
 Results: Of 159 infants, 9 (5.66%) were iden­tified with different types of hearing loss where 4 (2.51%) had sensory-neural hearing loss, one (0.62%) had auditory neuropathy and 4 (2.51%) had conductive hearing loss. There was a statis­tically significant relationship between hearing loss and birth weight < 1500 g, hyperbilirubine­mia, antibiotic therapy, family history of hearing loss, asphyxia and Apgar score < 5.
 Conclusion: Prevalence of hearing loss in NICU infants is noticeable so hearing assessment after discharge is necessary. Due to the presence of auditory neuropathy, simultaneous use of TEOAE and AABR tests in these infants is recommended.
 Keywords: Infant hearing screening; neonatal intensive care unit; auditory brainstem response

Prevalence and Risk Factors for Hearing Loss in Neonates Admitted to the Neonatal Intensive Care Unit: A Hospital Study.

Introduction: Hearing loss is one of the most common congenital disabilities in neonates. The aims of this study were to investigate the prevalence of hearing loss and identify the most significant risk factor in neonates hospitalized at the Neonatal Intensive Care Unit (NICU).Methods: This cross-sectional study involved 530 neonates admitted to NICU Abuzar Hospital with risk factors for hearing loss based on Joint Committee of Infant Hearing (JCIH). The hearing screening tests include transient evoked otoacoustic emissions (TEOAES) and the automated auditory brain stem response (AABR). For infants with abnormal AABR and TEOAE results, the Auditory Brainstem Response (ABR) and Auditory Steady-State Responses (ASSR) tests were performed.Result: Of 530 infants, 27 (5.09%) were diagnosed with different types of hearing loss. Ototoxic drugs, hyperbilirubinemia requiring exchange transfusion, asphyxia, low weight birth, Apgar score < 5, and a kinship marriage of parents were significant risk factors for hearing loss in our study population.Conclusion: Due to the high prevalence of hearing loss in the NICU, it is recommended that a hearing screening program be performed for all infants admitted to the NICU. Implement a comprehensive plan for neonatal hearing screening for early detection and intervention of hearing loss is essential.

Programmed cell death pathways in hearing loss: A review of apoptosis, autophagy and programmed necrosis.

Programmed cell death (PCD)—apoptosis, autophagy and programmed necrosis—is any pathological form of cell death mediated by intracellular processes. Ototoxic drugs, ageing and noise exposure are some common pathogenic factors of sensorineural hearing loss (SNHL) that can induce the programmed death of auditory hair cells through different pathways, and eventually lead to the loss of hair cells. Furthermore, several mutations in apoptotic genes including DFNA5, DFNA51 and DFNB74 have been suggested to be responsible for the new functional classes of monogenic hearing loss (HL). Therefore, in this review, we elucidate the role of these three forms of PCD in different types of HL and discuss their guiding significance for HL treatment. We believe that further studies of PCD pathways are necessary to understand the pathogenesis of HL and guide scientists and clinicians to identify new drug targets for HL treatment.

High performance hardware design of compressor adder in DA based FIR filters for hearing aids

Hearing aid is an acoustic device which is worn by hearing loss people. To compensate the different types of hearing loss, it is necessary to selectively amplify sounds at required frequencies. The main aim of the hearing aid is to selectively remove the noise signal such that the processed sound matches ones audiogram. To achieve this, the decimation filter in hearing aids can be design using multiplier less architecture which should be able to adjust sound levels at arbitrary frequencies within a given spectrum. In hearing aids, decimation filter plays a key role. This paper presents a low complexity design of a digital finite impulse response (FIR) filter for digital hearing aid application. This paper proposed approximate 4:2 compressor adders in memory less DA based FIR filter architecture. In DA architecture the area of the ROM increases gradually when filter order is increased. Memory less DA is designed using compressor adders is a solution to decrease the power consumption and area of the FIR filters and makes the area and power reduction for hearing aid application. The proposed DA based FIR filter architecture is synthesized on 90 nm technology using Synapsis Application Specific Integrated circuit design compiler. The proposed architecture has 45% reduction in area delay product when distinguish with systolic architecture and 10% less ADP when compare with OBC DA architecture. The proposed design is also implemented Field Programmable Gate Array and the results shows that the proposed architecture has less slices than best existing designs. The proposed architecture is used in decimation filter of hearing aids applications using matlab simulink, which removes the unwanted signal.

Risk of hearing loss in patients with fibromyalgia:A nationwide population-based retrospective cohort study.

ObjectivesOur objectives are to examine whether patients with fibromyalgia (FM) present an increased risk of hearing loss (HL) compared with those without FM and to explore the relationship between comorbidities/drugs and development of HL in patients with FM. Furthermore, we investigated the incidence rate of different types of HL and the joint effects for HL with FM and comorbidities.MethodsThis population-based retrospective cohort study included patients with new-onset FM from 2000 to 2002 (the FM group) and age- and sex-matched randomized patients without FM (the non-FM group) from Taiwan’s National Health Insurance Research Database. Patients were followed up from baseline (3 months after FM diagnosis) until death, withdrawal, HL development, or December 31, 2013. The primary outcome was the risk of HL, which was assessed using Cox proportional-hazards analysis.ResultsThe overall HL risk in the FM group was 1.46-fold (95% confidence interval [CI]: 1.38–1.55) higher than that in the non-FM group after adjustment for sex, age, and comorbidities (p < 0.0001). Patients with FM had significantly greater sensorineural HL (adjusted hazard ratio = 1.46, 95% CI: 1.37–1.56) than those without FM. Patients with FM having comorbidities of diabetes, hyperlipidemia, depression, and Meniere’s disease had a higher risk of HL than those without FM.ConclusionOur findings support the notion that FM influences HL and is in line with the hypothesis that the FM mechanism is related to a central nervous system abnormality in sensory processing. Health care professionals should provide appropriate screening for the risk of HL and prevention and counseling methods for patients with FM.

Asymptomatic Otitis Media With Effusion in Children With Adenoid Enlargement

ObjectivesTo estimate the burden, and clinical factors associated with development of Otitis media with effusion (OME) in children with enlarged adenoids. MethodsConsecutive eligible patients with adenoid (sometimes with tonsillar) enlargement managed operatively in a period of five years. Patients had no complaints suggestive of hearing impairment. Age, sex, clinical diagnosis, and weight of patients were recorded. Otoscopic, audiometric, and radiological examinations findings were recorded. Tympanometry patterns were classified according to Jerger's classification; type B and C tympanograms represented OME. Some patients had Pure Tone Audiometry (PTA). Plain nasopharyngeal X-ray assessed the adenoid: nasopharyngeal (AN) ratio with >0.5 regarded as obstructive adenoid. Results216 ears of 108 children were assessed. 49.1% of children were in age range 1-3 years, mean 3.6 ± 2.6 years. 62.0% were males, weight ranged from 7.8 to 31.0 kg, mean was 14.3 ± 5.2 kg. 63.9% had associated tonsillar enlargement. Mean AN ratio was 0.69 ± 0.07.Tympanometric findings showed prevalence of OME in all ears was 63/216 = 29.2%, consisting of 30.5% with unilateral (Right ear 19.4%, Left ear 11.1%), and 13.9% with bilateral OME. Acoustic reflexes were absent in both ears in 32 (29.6%) of the patients. There was normal hearing in 16/38 ears (42.1%), while others had different types of hearing loss. Two factors namely increased age and weight were significantly associated with OME in patients with Adenoid enlargement. Conclusions29.2% of children with adenoid enlargement had a co-morbidity of asymptomatic OME. The factors associated with OME were increased age and weight of the patients.

Can Differences in Early Hearing Development Be Distinguished by the LittlEARs Auditory Questionnaire?

This study asks whether the LittlEARs Auditory Questionnaire (LEAQ), a caregiver measure, can differentiate between the early auditory development of children with bilateral cochlear implants (CIs), bilateral hearing aids (HAs), and children with Auditory Neuropathy Spectrum Disorder (ANSD) who wear CIs or HAs. The LEAQ is sensitive to impaired auditory development but has not previously been used to distinguish developmental changes between groups of children using different hearing technologies or with different types of hearing loss. We collected retrospective longitudinal LEAQ results from 43 children with HAs, 43 with CIs, and 18 with ANSD. The children with ANSD wore hearing technology. They were a similar age to the children without ANSD (23 months; SD = 15), while the CI group (14 months; SD = 8) was younger than the HA group (24 months; SD = 18) [F(2,98.48) = 3.4; p = 0.04]. The CI group often participated in their first LEAQ pretreatment. Participants completed between one and seven LEAQs. Scores ranged between zero and 35 (mean = 18.36). We conducted a linear mixed-effects analysis, which included age or time since device fitting, hearing type (HA, CI, or ANSD), and presence of a comorbidity as fixed effects. A secondary analysis assessed effects of device audibility, measured by the Speech Intelligibility Index or Articulation Index, and consistency of device use obtained from device datalogs. Children with CIs progressed faster than their peers with HAs or ANSD [χ2(8) = 24.51; p = 0.002]. However, within a subsample that included consistency of device use (β7 = -0.20 ± 0.38, t = -0.52; β8 = 0.93 ± 0.82, t = 1.13) and audibility (β6 = -0.70 ± 1.45, t = -1.87; β7 = 0.87 ± 0.89, t = 0.98), study group did not significantly influence rate of improvement on the LEAQ. In addition, children with developmental delays in all three study groups demonstrated significantly slower LEAQ score improvement [χ2(6) = 23.60; p < 0.001] and a trend toward decreased consistency of device use [F(1) = 3.31; p = 0.07]. As we expected, children in the CI and HA groups were more likely to achieve auditory skills indicated in early rather than later LEAQ questions. There was less variability in the responses of the ANSD group [CI: interquartile range (IQR) = 9; HA: IQR = 8; ANSD: IQR = 1]. There was no connection between LEAQ growth and speech perception outcomes in a subsample [r(6) = 0.42; p = 0.30]. The LEAQ is a useful tool for monitoring initial auditory development in very young children and can inform early treatment decisions.

Using ASSR with narrow-band chirps to evaluate hearing in children and adults.

All studies concerning the reliability and threshold prediction of auditory steady-state responses (ASSR) focused on a particular group of patients. The present article evaluates the use of narrow-band, chirp-evoked ASSR for testing hearing in adults and children of all ages and with different types of hearing loss, as well as normal hearing. The aims are: to determine whether there are possible influencing factors, mainly the degree of hearing loss; and to validate the clinical value of using ASSR with chirp-stimuli. This is a retrospective study of 667 patients who had been diagnosed with and treated for hearing loss at our tertiary referral center. The following results were compared: ASSR to pure tone audiometry (PTA); click-ABRs to PTA; and click-ABRs to ASSR. We then calculated mean, median and standard deviation. A regression analysis was used to examine the correlation between: ASSR and click-ABRs; "estimated" audiogram and PTA; click-ABRs and PTA; and ASSR and PTA. We found significant correlations at all frequencies when comparing ASSR to click-ABRs, click-ABRs to PTA, and ASSR to PTA. Concerning the degree of hearing loss, there were significant differences between the patients with normal hearing and those with moderate-to-profound hearing loss. ASSR with narrow-band chirps are a reliable tool for estimating hearing thresholds in children and adults with all kinds of hearing loss. We have demonstrated that threshold differences between PTA and ASSR are negligible in the clinical routine. The "estimated" ASSR audiogram is a good approach for communicating ASSR results to the average user.

Correlation between brainstem evoked response audiometry with other audiological tests in different types of hearing loss

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Brainstem evoked response audiometry (BERA) is most specific and sensitive test for brain stem dysfunction. It is most important objective method for evaluating peripheral auditory system in neonates, infants, sedated and comatose patients and other person who doesn’t understand the language. Objective of the study was to evaluate correlation BERA with other audiological tests in different types of hearing loss as well as to study variations of wave forms in different types of hearing loss.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; Patients underwent a complete ENT check up to rule out any actively discharging gears, wax, infection or any middle ear problems. Different audiometric tests: pure tone audiometry (PTA), distortion product otoacoustic emissions, auditory steady-state response (ASSR) and BERA were applied to the patients. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; The majority of the patients (32 cases) belonged to the age group of 0-5 years. Maximum cases were of sensorineural hearing loss (60%). ASSR was highly sensitive (85.1%) for estimation of hearing threshold and specificity was 100% (p&amp;lt;0.001). BERA was also highly significant for estimation of hearing threshold (sensitivity: 83%; specificity: 92.3%; p value &amp;lt;0.001).&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; BERA has high degree of accuracy in detecting hearing threshold as an objective test but not as much accurate as ASSR. It is more valuable in terms of identification of site and size of the lesion in auditory pathway and identification for the type of the deafness.&lt;/p&gt;

Supra-Threshold Hearing Sensitivity Disorders and Mild Permanent Hearing Loss: Neglected Cause of Hidden Hearing Loss and Speech Defects.

Supra-threshold disorders in the form of auditory neuropathy (AN)/auditory dys-synchrony (AD) or central auditory processing disorders (CAPD), a special type of retrocochlear hearing loss; and also children with mild permanent hearing loss (PHL), may be missed on current hearing protocols. Otoacoustic emissions (OAE) and Brain stem evoked response audiometry (BERA), are tests, when used in combination, can indirectly help diagnose the different types of hearing loss. To correlate the parental awareness and the pattern of hearing loss (HL) in children with/without auditory and/or speech problems, using both OAE and BERA as hearing tests for indirect identification of suprathreshold disorders and mild PHL. An Observational Prospective study carried out on 100 children in the age group of 0-5years, for detection of type of HL by both OAE and BERA and correlating it with parental awareness of HL and speech defects. In 72.22% of children with speech problem only and without any complaints of HL, some form of HL (confirmed HL-OAE refer/BERA fail or auditory neuropathy/auditory dys-synchrony-OAE pass/BERA fail) was diagnosed, whereas 24.07% had no detectable HL on both OAE and BERA pass, and were considered as indirect evidence of CAPD. 3.7% with OAE refer and BERA pass were considered indicative of mild PHL. AN/AD/CAPD/Mild PHL are important cause of speech delay without parental awareness of HL. OAE and BERA together can be used as an indirect evidence of their presence.

Hallókészülékek, cochleáris implantátumok

Ez a gyűjtőmunka az emberi fül felépítéséről, a hallás működéséről, a halláskárosodás fajtáiról, valamint annak hallókészülékkel és implantátummal való javításáról szól. Ezen túl ismerteti az egyes hallókészülékek, implantátumok kiválasztását megelőző vizsgálatokat, a készülékek típusait, részeit, működését és egyéb funkcióit. Tárgyalja továbbá az implantátum beültetésének menetét és beüzemelésének folyamatát.

Contaminación acústica y su relación con las alteraciones auditivas en el personal de COPROBALAN EMA

Manta es una ciudad con mucha actividad productiva, esto hace que las personas que laboran en las empresas y fábricas vean en el ruido un factor para padecer de alteraciones auditivas, específicamente el ruido como riesgo laboral es mayor para quienes están en contacto directo con el mismo. La motivación para efectuar este trabajo que se pone en consideración tuvo como objetivo identificar los niveles de contaminación acústica en los empleados de Coprobalan Ema, para este fin se utilizó el instrumento de recolección de datos: Programa de Conservación de la Salud Auditiva, dirigido al personal que labora en el área de producción de la empresa, siendo 40 personas escogidas para la muestra. Las audiometrías que se realizaron en la empresa, fueron por vía aérea. Se observa en la interpretación de datos que, a pesar de la exposición a los ruidos dentro de la planta, existe protección dentro de cada una de las actividades que realizan los trabajadores dentro de la misma. Los aspectos más relevantes tomados en consideración, muestran la debida prevención en lo que respecta a al ruido.

The evidence of noninner ear involvement in the presence of depressed bone conduction threshold at 2 kHz frequency

Pure-tone audiometry is a standard clinical test for hearing diagnosis. Air–bone gaps derived from the difference between air conduction (AC) and bone conduction (BC) thresholds are important indicators to distinguish between different types of hearing losses. Even though BC thresholds are typically unaffected in conductive hearing loss (CHL) cases, a notch or depression at 2 kHz frequency (i.e., Carhart notch) could occur, which could be mistakenly considered as the involvement of sensorineural hearing loss (SNHL). In this article, it was clearly demonstrated that the BC notch at 2 kHz (that could exceed the normal limit) was in fact related to CHL (not SNHL). That is, in this case, both AC and BC thresholds became “perfectly” normal after the treatment (and the notch at 2 kHz disappeared). Several useful points were highlighted from the present case report to guide clinicians, medical trainees, and respective students to avoid misdiagnosis in clinical practice.

Optimizing Speech Recognition Using a Computational Model of Human Hearing: Effect of Noise Type and Efferent Time Constants

Physiological and psychophysical methods allow for an extended investigation of ascending (afferent) neural pathways from the ear to the brain in mammals, and their role in enhancing signals in noise. However, there is increased interest in descending (efferent) neural fibers in the mammalian auditory pathway. This efferent pathway operates via the olivocochlear system, modifying auditory processing by cochlear innervation and enhancing human ability to detect sounds in noisy backgrounds. Effective speech intelligibility may depend on a complex interaction between efferent time-constants and types of background noise. In this study, an auditory model with efferent-inspired processing provided the front-end to an automatic-speech-recognition system (ASR), used as a tool to evaluate speech recognition with changes in time-constants (50 to 2000 ms) and background noise type (unmodulated and modulated noise). With efferent activation, maximal speech recognition improvement (for both noise types) occurred for signal-to-noise ratios around 10 dB, characteristic of real-world speech-listening situations. Net speech improvement due to efferent activation (NSIEA) was smaller in modulated noise than in unmodulated noise. For unmodulated noise, NSIEA increased with increasing time-constant. For modulated noise, NSIEA increased for time-constants up to 200 ms but remained similar for longer time-constants, consistent with speech-envelope modulation times important to speech recognition in modulated noise. The model improves our understanding of the complex interactions involved in speech recognition in noise, and could be used to simulate the difficulties of speech perception in noise as a consequence of different types of hearing loss.

Modeling and Simulation of Frequency Response Masking FIR Filter Bank using Approximate Multiplier for Hearing Aid Application

The tremendous increase in the use of portable electronic devices is due to the development in the fields of signal processing and electronic technology. These battery operated devices needs reduction in power consumption with increased performance and long battery life. Since CMOS technology scaling fast approaches its physical limit of minimum supply voltage and smaller feature size, the hardware designer has to opt for new multiplier architectures for achieving low power and high speed performance. This paper proposes an area and power efficient approximate multiplier architecture. The error metrics are estimated to verify its performance advantage over other approximate multipliers. Using Frequency Response masking approach, a 6-band non-uniform digital FIR filter bank is developed using approximate multiplier for hearing aid application. Audiogram matching is done with audiograms of two different types of hearing losses and the matching error is computed. Simulation results show that the audiogram matching error falls within +/- 4 dB range.

Vowel production of Mandarin-speaking hearing aid users with different types of hearing loss.

In contrast with previous research focusing on cochlear implants, this study examined the speech performance of hearing aid users with conductive (n = 11), mixed (n = 10), and sensorineural hearing loss (n = 7) and compared it with the speech of hearing control. Speech intelligibility was evaluated by computing the vowel space area defined by the Mandarin Chinese corner vowels /a, u, i/. The acoustic differences between the vowels were assessed using the Euclidean distance. The results revealed that both the conductive and mixed hearing loss groups exhibited a reduced vowel working space, but no significant difference was found between the sensorineural hearing loss and normal hearing groups. An analysis using the Euclidean distance further showed that the compression of vowel space area in conductive hearing loss can be attributed to the substantial lowering of the second formant of /i/. The differences in vowel production between groups are discussed in terms of the occlusion effect and the signal transmission media of various hearing devices.

Review Studies of GJB2 Gene in Patients with Hearing Impairment in Pakistan

Deafness is inherited as one of the most frequent type of neurosensory disorder. The specific physiologic mechanisms of the different types of hearing loss are still unknown. Recent studies have listed numerous causative agents for hearing loss. Genetic factors contribute to a greater extent in hearing disability. GJB2 gene is one of the most promising candidates. 35delG is the most common mutation which accounts for about 50% of all GJB2 gene mutation. After collection of data and isolation of blood, extraction of DNA was performed for each sample. Direct sequencing of GJB2 gene was performed. Primary sequencing data of the representative sample concluded that GJB2 gene is not mutated in the studied family. There are other candidates for hearing disability; further investigations are needed for assessment of other members of the selected family or other genes that are responsible for hearing loss. Human GJB2 protein was compared with that of mouse and rabbit. The data from multiple alignment shows that there is only alteration at nine different points of rabbit compared to human GJB2 protein while this variation is sixteen times when we compared human GJB2 protein sequence with that of mouse.

An Appraisal of the Advancement of Emerging Technologies in Hearing Aids

Background/Objectives: To shed light on the history of development of hearing aid, along with the various pros and cons of each developed technological solution. Methods/Statistical Analysis: A historical survey has been conducted, which highlights some of the more renowned and widely deployed hearing aids within the market. The different types of hearing losses and the varying degree of assistance provided by the different technological solutions have been discussed. Over the years, the overall accuracy as well as practicality of the hearing aids have improved, ranging from cumbersome and heavy hearing aids in the early 1960-1970s to the portable and wearable ones available in the market at present. Findings: Technological innovation is spearheading massive improvement within the hearing aids. Some of the key features causing these changes include wireless technology, frequency lowering, directional microphones and digital noise reduction algorithms. Although DSP has provided significant changes in the hearing technology, limitations still exist, which require re-search contributions from different fields like clinical audiology, psychoacoustics and signal processing. Application/Improvements: Most of the portable and reliable hearing aid devices are very expensive. Therefore, there is a need to develop affordable pieces of equipment that is able to cater to basic hearing functionalities.

Diagnosis of hearing loss using automated audiometry in an asynchronous telehealth model: A pilot accuracy study

Introduction Standard criteria exist for diagnosing different types of hearing loss, yet audiologists interpret audiograms manually. This pilot study examined the feasibility of standardised interpretations of audiometry in a telehealth model of care. The aim of this study was to examine diagnostic accuracy of automated audiometry in adults with hearing loss in an asynchronous telehealth model using pre-defined diagnostic protocols. Materials and methods We recruited 42 study participants from a public audiology and otolaryngology clinic in Perth, Western Australia. Manual audiometry was performed by an audiologist either before or after automated audiometry. Diagnostic protocols were applied asynchronously for normal hearing, disabling hearing loss, conductive hearing loss and unilateral hearing loss. Sensitivity and specificity analyses were conducted using a two-by-two matrix and Cohen’s kappa was used to measure agreement. Results The overall sensitivity for the diagnostic criteria was 0.88 (range: 0.86–1) and overall specificity was 0.93 (range: 0.86–0.97). Overall kappa (k) agreement was ‘substantial’ k = 0.80 (95% confidence interval (CI) 0.70–0.89) and significant at p < 0.001. Discussion Pre-defined diagnostic protocols applied asynchronously to automated audiometry provide accurate identification of disabling, conductive and unilateral hearing loss. This method has the potential to improve synchronous and asynchronous tele-audiology service delivery.

Microtia: A Combined Approach by Genetics and Audiology

Abstract Microtia is a condition in which the external portion of the ear (the auricle) is malformed. In the strictest definition, there is also narrowing or absence of the external auditory canal (external auditory meatus). Microtia varies in severity from barely discernible to an external ear with major structural changes. Hearing loss is a common feature that can be associated with microtia. There are different types of hearing loss–conductive, sensorineural, or mixed–depending on which part of the ear is not working as it should. The present study was designed with the following aims: (i) identification of the genetic etiology and patterns of inheritance of microtia for proper genetic counseling; (ii) early detection and identification of associated hearing impairment for proper management including use of a hearing aid and surgical intervention. Twenty children with microtia ranging in age between 1 and 15 years (mean: 6.2 ± 3.68 years) irrespective of sex were included in this study: 14 males and six females. All children were subjected to a full assessment of medical history, a general examination, an ENT examination, tympanometry, pure tone audiometry, and an auditory steady-state response test for patients not responding to a conventional audiometric test. Karyotyping, fluorescence in-situ hybridization (FISH) for Treacher Collins cases, radiological investigations, and fundus examination were also performed. Syndromic microtia was more frequent than nonsyndromic microtia. Treacher Collins syndrome was the most clinically diagnosed syndrome, followed by Goldenhar’s syndrome. There was one case of Down’s syndrome and another single case of Johnson–McMillin syndrome. Meatal atresia and preauricular tags were frequently present in the microtia cases, whereas middle ear and inner ear anomalies were only found in some cases. The most common presenting symptom of microtia is hearing loss. Its degree and type differ according to the severity of the disease and frequencies affected. In total, 88.5% (23 ears) have conductive hearing loss and 11.5% (three ears) have mixed hearing loss. Karyotyping was performed for 10 cases; nine cases were normal, whereas one case was abnormal (47XY, +21) (Down’s syndrome), which represents 5% of all cases studied. FISH was performed for four cases of Treacher Collins syndrome using a probe for chromosome 5 with gene map locus 5q31q33, but no deletion was found in the chromosome 5 Treacher Collins–Franceschetti 1 (TCOF1) gene. Genetic predisposition for both autosomal dominant and autosomal recessive inheritance seems to be a strong determinant factor in the etiology of microtia than the environmental one. As for Treacher Collins, which is the most frequently clinically diagnosed syndrome in the current study, the FISH study showed that the 5q31-q33 locus may not carry the causative mutation as no single case was positive for this locus. Hearing impairment, of the conductive type, is the most frequent symptom that leads parents to seek medical advice and genetic counseling.