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Daniela V. Luquetti - One of the best experts on this subject based on the ideXlab platform.
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Risk factors and demographics for Microtia in South America
Birth defects research. Part A Clinical and molecular teratology, 2013Co-Authors: Daniela V. Luquetti, Jorge S. Lopez-camelo, Maria Da Graça Dutra, Babette S. Saltzman, Eduardo E. CastillaAbstract:BACKGROUND—The etiopathogenesis of Microtia is still unknown in the majority of the cases, particularly for individuals presenting with isolated Microtia. Our aim was to evaluate potential risk factors for this condition using a case-control approach. METHODS—We analyzed data from 1,194 livebirths with isolated Microtia enrolled in the ECLAMC study (Estudio Colaborativo Latino Americano de Malformaciones Congenitas) from 1982 to 2011 and their respective controls. Odds ratios were estimated with conditional logistic regression models along with 95% confidence intervals for the resulting odds ratio estimates controlling for the effects of potential confounders (sex, maternal age, hospital and year of birth) for an adjusted OR (aOR). RESULTS—Multiparity was associated with a higher risk of Microtia compared to primiparity (aOR 1.5, 95%CI 1.2–1.8), with women who had eight or more prior pregnancies having the highest risk (aOR 2.8, 95%CI 1.6–5.2). Women who presented with cold-like symptoms were at higher risk for Microtia (aOR 2.2, 95%CI 1.2–3.9) as well as those that used tobacco or alcohol during pregnancy (aOR 1.7, 95%CI 1.1–2.5 and aOR 1.4, 95%CI 0.9–2.1, respectively). The association with alcohol use appeared to be limited to those women who reported binge drinking during pregnancy (aOR 1.4, 95% 0.7–2.9). Cases from hospitals at low altitude (< 2,500 m) tended to have more severe types of Microtia than those from hospitals at high altitude. CONCLUSIONS—These results support the hypothesis that in addition to teratogens other nongenetic risk factors contribute to the occurrence of isolated Microtia.
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risk factors and demographics for Microtia in south america a case control analysis
Birth Defects Research Part A-clinical and Molecular Teratology, 2013Co-Authors: Maria Da Graça Dutra, Daniela V. Luquetti, Babette S. Saltzman, Jorge S Lopezcamelo, Eduardo E. CastillaAbstract:BACKGROUND—The etiopathogenesis of Microtia is still unknown in the majority of the cases, particularly for individuals presenting with isolated Microtia. Our aim was to evaluate potential risk factors for this condition using a case-control approach. METHODS—We analyzed data from 1,194 livebirths with isolated Microtia enrolled in the ECLAMC study (Estudio Colaborativo Latino Americano de Malformaciones Congenitas) from 1982 to 2011 and their respective controls. Odds ratios were estimated with conditional logistic regression models along with 95% confidence intervals for the resulting odds ratio estimates controlling for the effects of potential confounders (sex, maternal age, hospital and year of birth) for an adjusted OR (aOR). RESULTS—Multiparity was associated with a higher risk of Microtia compared to primiparity (aOR 1.5, 95%CI 1.2–1.8), with women who had eight or more prior pregnancies having the highest risk (aOR 2.8, 95%CI 1.6–5.2). Women who presented with cold-like symptoms were at higher risk for Microtia (aOR 2.2, 95%CI 1.2–3.9) as well as those that used tobacco or alcohol during pregnancy (aOR 1.7, 95%CI 1.1–2.5 and aOR 1.4, 95%CI 0.9–2.1, respectively). The association with alcohol use appeared to be limited to those women who reported binge drinking during pregnancy (aOR 1.4, 95% 0.7–2.9). Cases from hospitals at low altitude (< 2,500 m) tended to have more severe types of Microtia than those from hospitals at high altitude. CONCLUSIONS—These results support the hypothesis that in addition to teratogens other nongenetic risk factors contribute to the occurrence of isolated Microtia.
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Preferential Associated Anomalies in 818 Cases of Microtia in South America
American Journal of Medical Genetics Part A, 2013Co-Authors: Daniela V. Luquetti, Timothy C. Cox, Jorge S. Lopez-camelo, Maria Da Graça Dutra, Michael L. Cunningham, Eduardo E. CastillaAbstract:The etiology of Microtia remains unknown in most cases. The identification of patterns of associated anomalies (i.e., other anomalies that occur with a given congenital anomaly in a higher than expected frequency), is a methodology that has been used for research into the etiology of birth defects. We conducted a study based on cases of Microtia that were diagnosed from more than 5 million live (LB)- and stillbirths (SB) examined in hospitals participating in ECLAMC (Latin American Collaborative Study of Congenital Malformations) between 1967 and 2009. We identified 818 LB and SB with Microtia and at least one additional non-related major congenital anomaly (cases) and 15,969 LB and SB with two or more unrelated major congenital anomalies except Microtia (controls). A logistic regression analysis was performed to identify the congenital anomalies preferentially associated with Microtia. Preferential associations were observed for 10 congenital anomalies, most of them in the craniofacial region, including facial asymmetry, choanal atresia, and eyelid colobomata. The analysis by type of Microtia showed that for anomalies such as cleft lip and palate, macrostomia, and limb reduction defects, the frequency increased with the severity of the Microtia. In contrast, for other anomalies the frequency tended to be the same across all types of Microtia. Based on these results we will integrate data on the developmental pathways related to preferentially associated congenital anomalies for future studies investigating the etiology of Microtia.
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Microtia: epidemiology and genetics.
American journal of medical genetics. Part A, 2011Co-Authors: Daniela V. Luquetti, Michael L. Cunningham, Carrie L Heike, Anne V Hing, Timothy C. CoxAbstract:Microtia is a congenital anomaly of the ear that ranges in severity from mild structural abnormalities to complete absence of the ear, and can occur as an isolated birth defect or as part of a spectrum of anomalies or a syndrome. Microtia is often associated with hearing loss and patients typically require treatment for hearing impairment and surgical ear reconstruction. The reported prevalence varies among regions, from 0.83 to 17.4 per 10,000 births, and the prevalence is considered to be higher in Hispanics, Asians, Native Americans, and Andeans. The etiology of Microtia and the cause of this wide variability in prevalence are poorly understood. Strong evidence supports the role of environmental and genetic causes for Microtia. Although some studies have identified candidate genetic variants for Microtia, no causal genetic mutation has been confirmed. The application of novel strategies in developmental biology and genetics has facilitated elucidation of mechanisms controlling craniofacial development. In this paper we review current knowledge of the epidemiology and genetics of Microtia, including potential candidate genes supported by evidence from human syndromes and animal models. We also discuss the possible etiopathogenesis in light of the hypotheses formulated to date: Neural crest cells disturbance, vascular disruption, and altitude.
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Microtia-anotia: a global review of prevalence rates.
Birth defects research. Part A Clinical and molecular teratology, 2011Co-Authors: Daniela V. Luquetti, Emanuele Leoncini, Pierpaolo MastroiacovoAbstract:BACKGROUND There are few published studies on Microtia-anotia frequency. METHODS Using data from birth defects surveillance programs around the world, we conducted a systematic review on the frequency of Microtia-anotia to further explore the differences in prevalence across countries. Ninety-two birth defects surveillance programs were evaluated with a total of 8917 cases of Microtia-anotia. We computed the prevalence per 10,000 births for each surveillance program for total cases of Microtia-anotia (Microtia types I to IV), Microtia (types I to III), and anotia (type IV). Prevalence ratios were calculated by large geographic areas, race/ethnicity, and by surveillance methodologies. RESULTS The overall prevalences were: Microtia-anotia, 2.06 (confidence interval [CI], 2.02–2.10); Microtia, 1.55 (CI, 1.50–1.60); and anotia 0.36 (CI, 0.34–0.38). Higher prevalences were observed for the Americas, Northern Europe and Asia, among Hispanics and Asians, and among active ascertainment and hospital-based surveillance programs. CONCLUSIONS We observed marked variation in the prevalence of Microtia-anotia across surveillance programs and within countries. These results must be interpreted cautiously as this variability may be explained mainly by differences in surveillance methods. However, given the magnitude of some of the differences, other factors may also be involved. This study contributes to the knowledge of the prevalence of Microtia-anotia by providing a critical analysis of the existing data. In addition, it supports the need for a coding system that allows complete phenotype characterization of Microtia-anotia, including severity and laterality, as well as for further studies on the variation of its frequency related to race and ethnicity. Birth Defects Research (Part A), 2011. © 2011 Wiley-Liss, Inc.
Kimitaka Kaga - One of the best experts on this subject based on the ideXlab platform.
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hearing levels in patients with Microtia correlation with temporal bone malformation
Laryngoscope, 2007Co-Authors: Shinichi Ishimoto, Ken Ito, Shotaro Karino, Hideki Takegoshi, Kimitaka Kaga, Tatsuya YamasobaAbstract:OBJECTIVE: To evaluate the relationship between hearing level and temporal bone abnormalities in patients with Microtia. STUDY DESIGN: Retrospective case series study between 1992 and 2004. SETTING: Academic, tertiary care referral medical center. PATIENTS: We evaluated 115 ears of 89 patients (68 males, 21 females; mean age, 11 yr; range, 5-44 yr) with Microtia. MAIN OUTCOME MEASURES: Hearing level was examined in patients with Microtia. Developmental abnormalities of the temporal bone were evaluated by Jahrsdoerfer's computed tomography (CT) scoring system using high-resolution CT (HRCT) scans of the temporal bone. Temporal bone malformation scores were divided into four subgroups: ossicular development, windows connected to the cochlea, aeration of the middle ear cavity, and facial nerve aberration. Patients were divided into the stenosis and atresia groups on the basis of the appearance of the external auditory canal (EAC). We also evaluated the relationships between hearing level and four subtotal scores of the HRCT findings in the stenosis and atresia groups. RESULTS: There was no relationship between hearing level and total points of HRCT scoring system or between hearing level and severity of Microtia scored by Marx classification. With regard to subtotal points related to ossicles (4 points), the hearing level in ears with low scores ( or =2) (54.0 +/- 2.8 dB) in the stenosis group. In the atresia group, the hearing level was 64.3 +/- 2.2 dB in ears with low scores and 62.3 +/- 1.1 in ears with high scores (P > .5). As for subtotal points related to the windows connected to cochlea (2 points), the hearing level was 64.8 +/- 2.6 dB in ears with low scores (0) and 55.9 +/- 2.4 dB in ears with high scores (> = 1) in the stenosis group. In the atresia group, the hearing level was 67.7 +/- 2.3 dB in ears with low scores and 61.5 +/- 1.0 in ears with high scores. There was significant difference between ears with low and high scores in the stenosis group (P = .03) and atresia group (P = .009). There was no significant difference between ears with low and high scores with respect to the subtotal points related to aeration of the middle ear cavity and aberration of the facial nerve. CONCLUSION: The hearing level in microtic ears correlated with the formation of oval/round windows and ossicular development but not with the degree of middle ear aeration, facial nerve aberration, or severity of Microtia. The hearing level can also serve as an indictor, such as the HRCT findings, to determine whether a subject's hearing will likely improve after reconstructive surgery.
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cooperative reconstructive surgery for children with bilateral Microtia and atresia for esthetically and functionally better results
Practica oto-rhino-laryngologica, 2006Co-Authors: Kimitaka Kaga, Hirotaka AsatoAbstract:In two stages of total ear reconstruction surgery for children with Microtia and atresia, plastic surgeons and otologists have cooperated to reduce the number of surgeries and to achieve both esthetically and functionally better results. Ear elevation, external canal plasty and tympanoplasty are performed cooperatively in the second stage of surgery.In the past 5 years we have experienced 70 cases of combined surgery as the second stage of Microtia reconstruction: 59 boys and 11 girls, 12 cases of bilateral Microtia and atresia and 58 cases of unilateral Microtia and atresia. Analysis of the 3D CT scan was useful to plan total reconstruction surgery for Microtia and atresia, and was important for education and design of surgery. Esthetically and functionally combined surgery at the second stage is convenient to make a natural orifice of the external canal, elevate the external ear and reconstruct the ossicular chain.After bilateral reconstructive surgery, patients with bilateral Microtia and atresia can localize sounds in the right and left auditory spaces.
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correlation between Microtia and temporal bone malformation evaluated using grading systems
Archives of Otolaryngology-head & Neck Surgery, 2005Co-Authors: Shinichi Ishimoto, Ken Ito, Shotaro Karino, Hideki Takegoshi, Tatsuya Yamasoba, Kenji Kondo, Kimitaka KagaAbstract:Objective To evaluate the relationships between temporal bone abnormalities and the severity of Microtia in Japanese patients using objective grading systems. Design Retrospective case series study conducted between 1992 and 2003. Setting Academic, tertiary care, referral medical center. Patients One hundred forty-two ears of 109 Japanese patients (85 male and 24 female patients; mean age, 12.8 years [range, 2-36 years]) with Microtia. Main Outcome Measures The severity of Microtia was classified according to Marx classification. Developmental abnormalities of the temporal bone were evaluated by a computed tomographic (CT) scoring system modified after the system used by Jahrsdoerfer and colleagues, using high-resolution CT scans of the temporal bone. Correlations between the scores obtained from these 2 grading systems were evaluated using a nonparametric statistical method. Results Male preponderance and incidence of bilateral cases of approximately 30% were observed in our Japanese patients with Microtia. There was no significant difference in the severity of Microtia between unilateral and bilateral cases. The mean ± SEM total points in the CT scoring system (full marks, 10) was 7.9 ± 0.4 for grade I Microtia, 6.6 ± 0.6 for grade II, and 6.4 ± 0.3 for grade III; the total points correlated inversely with the Microtia grade. Development of the auricle correlated significantly with aeration in the middle ear spaces but not with ossicular development or formation of the oval/round windows. Proportion of acceptable surgical candidates according to the CT scoring system (>5 points) was 79% for grade I Microtia, 52% for grade II Microtia, and 65% for grade III Microtia. Conclusion The principle “the better developed the auricle, the better developed middle ear” was confirmed in Japanese patients with Microtia; however, even with grade II/III Microtia, more than half of the patients were considered suitable for atresia surgery.
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difference in facial canal anatomy in terms of severity of Microtia and deformity of middle ear in patients with Microtia
Laryngoscope, 2003Co-Authors: Hideki Takegoshi, Kimitaka KagaAbstract:Objective To study the difference in the facial canal anatomy in terms of the severity of Microtia and deformity of the middle ear in patients with Microtia using high-resolution computed tomography (HRCT). Study Design Forty-six ears with Microtia were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. Methods Forty-six ears with Microtia (unilateral Microtia, n = 12; bilateral Microtia, n = 34) were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings and age distribution of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. Results The mastoid portion of the facial nerve was 3 mm more anteriorly displaced in patients with grades II and III Microtia than in those with grade I Microtia (P <.01). From the scoring system that was used, the course of the facial nerve was not significantly different between patients with a score of 5 points or less and those with a score of 6 points or more. Conclusion The mastoid portion of the facial nerve in patients with grades II and III Microtia would be more anteriorly displaced because of hypoplasia of the second genu.
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Difference in facial canal anatomy in terms of severity of Microtia and deformity of middle ear in patients with Microtia.
The Laryngoscope, 2003Co-Authors: Hideki Takegoshi, Kimitaka KagaAbstract:Objective To study the difference in the facial canal anatomy in terms of the severity of Microtia and deformity of the middle ear in patients with Microtia using high-resolution computed tomography (HRCT). Study Design Forty-six ears with Microtia were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. Methods Forty-six ears with Microtia (unilateral Microtia, n = 12; bilateral Microtia, n = 34) were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings and age distribution of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. Results The mastoid portion of the facial nerve was 3 mm more anteriorly displaced in patients with grades II and III Microtia than in those with grade I Microtia (P
Jun Zhu - One of the best experts on this subject based on the ideXlab platform.
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epidemiologic characteristics and time trend in the prevalence of anotia and Microtia in china
Birth Defects Research Part A-clinical and Molecular Teratology, 2016Co-Authors: Kui Deng, Li Dai, Changfei Deng, Jun ZhuAbstract:BACKGROUND Previous studies have shown an inconsistent time trend on the prevalence of anotia and Microtia. Little has been reported on the epidemiologic characteristics of anotia and Microtia in the Chinese population. METHODS Data from 1996-2007 were obtained from the Chinese Birth Defects Monitoring Network in China. Birth prevalence of anotia and Microtia were assessed according to demographic characteristics and annual time trend. Poisson regression was used to calculate crude and adjusted prevalence ratios (APRs) and 95% confidence intervals (CIs) for selected demographic characteristics and subgroups of anotia and Microtia. RESULTS A total of 1933 cases with anotia/Microtia were identified among 6,308,594 live births, stillbirths, and terminations of pregnancy, yielding a rate of 3.06 per 10,000 births. Isolated anotia/Microtia had a prevalence of 2.25 per 10,000 births, whereas among nonisolated cases, the prevalence was 0.81 per 10,000 births. The prevalence rates of anotia/Microtia increased significantly during 1996-2007 (p < 0.05). Birth prevalence of isolated anotia/Microtia was significantly higher among western births (APR, 1.24; 95% CI, 1.10-1.40), mothers residing in urban areas (APR, 1.29; 95% CI, 1.15-1.46), mothers more than 35 years of age (APR, 1.26; 95% CI, 1.01-1.57), and males (APR, 1.38; 95% CI, 1.24-1.53). No significant associations were observed between nonisolated anotia/Microtia and geographic areas, maternal residence, and infant sex (except for maternal age). CONCLUSION An increasing trend of the birth prevalence of anotia and Microtia is observed in China. Higher prevalence risk of isolated anotia and Microtia is found among western births, mothers residing in urban areas, older mothers, and males.
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Epidemiologic characteristics and time trend in the prevalence of anotia and Microtia in China.
Birth defects research. Part A Clinical and molecular teratology, 2015Co-Authors: Kui Deng, Li Dai, Changfei Deng, Jun ZhuAbstract:BACKGROUND Previous studies have shown an inconsistent time trend on the prevalence of anotia and Microtia. Little has been reported on the epidemiologic characteristics of anotia and Microtia in the Chinese population. METHODS Data from 1996-2007 were obtained from the Chinese Birth Defects Monitoring Network in China. Birth prevalence of anotia and Microtia were assessed according to demographic characteristics and annual time trend. Poisson regression was used to calculate crude and adjusted prevalence ratios (APRs) and 95% confidence intervals (CIs) for selected demographic characteristics and subgroups of anotia and Microtia. RESULTS A total of 1933 cases with anotia/Microtia were identified among 6,308,594 live births, stillbirths, and terminations of pregnancy, yielding a rate of 3.06 per 10,000 births. Isolated anotia/Microtia had a prevalence of 2.25 per 10,000 births, whereas among nonisolated cases, the prevalence was 0.81 per 10,000 births. The prevalence rates of anotia/Microtia increased significantly during 1996-2007 (p
Eduardo E. Castilla - One of the best experts on this subject based on the ideXlab platform.
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Risk factors and demographics for Microtia in South America
Birth defects research. Part A Clinical and molecular teratology, 2013Co-Authors: Daniela V. Luquetti, Jorge S. Lopez-camelo, Maria Da Graça Dutra, Babette S. Saltzman, Eduardo E. CastillaAbstract:BACKGROUND—The etiopathogenesis of Microtia is still unknown in the majority of the cases, particularly for individuals presenting with isolated Microtia. Our aim was to evaluate potential risk factors for this condition using a case-control approach. METHODS—We analyzed data from 1,194 livebirths with isolated Microtia enrolled in the ECLAMC study (Estudio Colaborativo Latino Americano de Malformaciones Congenitas) from 1982 to 2011 and their respective controls. Odds ratios were estimated with conditional logistic regression models along with 95% confidence intervals for the resulting odds ratio estimates controlling for the effects of potential confounders (sex, maternal age, hospital and year of birth) for an adjusted OR (aOR). RESULTS—Multiparity was associated with a higher risk of Microtia compared to primiparity (aOR 1.5, 95%CI 1.2–1.8), with women who had eight or more prior pregnancies having the highest risk (aOR 2.8, 95%CI 1.6–5.2). Women who presented with cold-like symptoms were at higher risk for Microtia (aOR 2.2, 95%CI 1.2–3.9) as well as those that used tobacco or alcohol during pregnancy (aOR 1.7, 95%CI 1.1–2.5 and aOR 1.4, 95%CI 0.9–2.1, respectively). The association with alcohol use appeared to be limited to those women who reported binge drinking during pregnancy (aOR 1.4, 95% 0.7–2.9). Cases from hospitals at low altitude (< 2,500 m) tended to have more severe types of Microtia than those from hospitals at high altitude. CONCLUSIONS—These results support the hypothesis that in addition to teratogens other nongenetic risk factors contribute to the occurrence of isolated Microtia.
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risk factors and demographics for Microtia in south america a case control analysis
Birth Defects Research Part A-clinical and Molecular Teratology, 2013Co-Authors: Maria Da Graça Dutra, Daniela V. Luquetti, Babette S. Saltzman, Jorge S Lopezcamelo, Eduardo E. CastillaAbstract:BACKGROUND—The etiopathogenesis of Microtia is still unknown in the majority of the cases, particularly for individuals presenting with isolated Microtia. Our aim was to evaluate potential risk factors for this condition using a case-control approach. METHODS—We analyzed data from 1,194 livebirths with isolated Microtia enrolled in the ECLAMC study (Estudio Colaborativo Latino Americano de Malformaciones Congenitas) from 1982 to 2011 and their respective controls. Odds ratios were estimated with conditional logistic regression models along with 95% confidence intervals for the resulting odds ratio estimates controlling for the effects of potential confounders (sex, maternal age, hospital and year of birth) for an adjusted OR (aOR). RESULTS—Multiparity was associated with a higher risk of Microtia compared to primiparity (aOR 1.5, 95%CI 1.2–1.8), with women who had eight or more prior pregnancies having the highest risk (aOR 2.8, 95%CI 1.6–5.2). Women who presented with cold-like symptoms were at higher risk for Microtia (aOR 2.2, 95%CI 1.2–3.9) as well as those that used tobacco or alcohol during pregnancy (aOR 1.7, 95%CI 1.1–2.5 and aOR 1.4, 95%CI 0.9–2.1, respectively). The association with alcohol use appeared to be limited to those women who reported binge drinking during pregnancy (aOR 1.4, 95% 0.7–2.9). Cases from hospitals at low altitude (< 2,500 m) tended to have more severe types of Microtia than those from hospitals at high altitude. CONCLUSIONS—These results support the hypothesis that in addition to teratogens other nongenetic risk factors contribute to the occurrence of isolated Microtia.
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Preferential Associated Anomalies in 818 Cases of Microtia in South America
American Journal of Medical Genetics Part A, 2013Co-Authors: Daniela V. Luquetti, Timothy C. Cox, Jorge S. Lopez-camelo, Maria Da Graça Dutra, Michael L. Cunningham, Eduardo E. CastillaAbstract:The etiology of Microtia remains unknown in most cases. The identification of patterns of associated anomalies (i.e., other anomalies that occur with a given congenital anomaly in a higher than expected frequency), is a methodology that has been used for research into the etiology of birth defects. We conducted a study based on cases of Microtia that were diagnosed from more than 5 million live (LB)- and stillbirths (SB) examined in hospitals participating in ECLAMC (Latin American Collaborative Study of Congenital Malformations) between 1967 and 2009. We identified 818 LB and SB with Microtia and at least one additional non-related major congenital anomaly (cases) and 15,969 LB and SB with two or more unrelated major congenital anomalies except Microtia (controls). A logistic regression analysis was performed to identify the congenital anomalies preferentially associated with Microtia. Preferential associations were observed for 10 congenital anomalies, most of them in the craniofacial region, including facial asymmetry, choanal atresia, and eyelid colobomata. The analysis by type of Microtia showed that for anomalies such as cleft lip and palate, macrostomia, and limb reduction defects, the frequency increased with the severity of the Microtia. In contrast, for other anomalies the frequency tended to be the same across all types of Microtia. Based on these results we will integrate data on the developmental pathways related to preferentially associated congenital anomalies for future studies investigating the etiology of Microtia.
Hideki Takegoshi - One of the best experts on this subject based on the ideXlab platform.
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hearing levels in patients with Microtia correlation with temporal bone malformation
Laryngoscope, 2007Co-Authors: Shinichi Ishimoto, Ken Ito, Shotaro Karino, Hideki Takegoshi, Kimitaka Kaga, Tatsuya YamasobaAbstract:OBJECTIVE: To evaluate the relationship between hearing level and temporal bone abnormalities in patients with Microtia. STUDY DESIGN: Retrospective case series study between 1992 and 2004. SETTING: Academic, tertiary care referral medical center. PATIENTS: We evaluated 115 ears of 89 patients (68 males, 21 females; mean age, 11 yr; range, 5-44 yr) with Microtia. MAIN OUTCOME MEASURES: Hearing level was examined in patients with Microtia. Developmental abnormalities of the temporal bone were evaluated by Jahrsdoerfer's computed tomography (CT) scoring system using high-resolution CT (HRCT) scans of the temporal bone. Temporal bone malformation scores were divided into four subgroups: ossicular development, windows connected to the cochlea, aeration of the middle ear cavity, and facial nerve aberration. Patients were divided into the stenosis and atresia groups on the basis of the appearance of the external auditory canal (EAC). We also evaluated the relationships between hearing level and four subtotal scores of the HRCT findings in the stenosis and atresia groups. RESULTS: There was no relationship between hearing level and total points of HRCT scoring system or between hearing level and severity of Microtia scored by Marx classification. With regard to subtotal points related to ossicles (4 points), the hearing level in ears with low scores ( or =2) (54.0 +/- 2.8 dB) in the stenosis group. In the atresia group, the hearing level was 64.3 +/- 2.2 dB in ears with low scores and 62.3 +/- 1.1 in ears with high scores (P > .5). As for subtotal points related to the windows connected to cochlea (2 points), the hearing level was 64.8 +/- 2.6 dB in ears with low scores (0) and 55.9 +/- 2.4 dB in ears with high scores (> = 1) in the stenosis group. In the atresia group, the hearing level was 67.7 +/- 2.3 dB in ears with low scores and 61.5 +/- 1.0 in ears with high scores. There was significant difference between ears with low and high scores in the stenosis group (P = .03) and atresia group (P = .009). There was no significant difference between ears with low and high scores with respect to the subtotal points related to aeration of the middle ear cavity and aberration of the facial nerve. CONCLUSION: The hearing level in microtic ears correlated with the formation of oval/round windows and ossicular development but not with the degree of middle ear aeration, facial nerve aberration, or severity of Microtia. The hearing level can also serve as an indictor, such as the HRCT findings, to determine whether a subject's hearing will likely improve after reconstructive surgery.
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correlation between Microtia and temporal bone malformation evaluated using grading systems
Archives of Otolaryngology-head & Neck Surgery, 2005Co-Authors: Shinichi Ishimoto, Ken Ito, Shotaro Karino, Hideki Takegoshi, Tatsuya Yamasoba, Kenji Kondo, Kimitaka KagaAbstract:Objective To evaluate the relationships between temporal bone abnormalities and the severity of Microtia in Japanese patients using objective grading systems. Design Retrospective case series study conducted between 1992 and 2003. Setting Academic, tertiary care, referral medical center. Patients One hundred forty-two ears of 109 Japanese patients (85 male and 24 female patients; mean age, 12.8 years [range, 2-36 years]) with Microtia. Main Outcome Measures The severity of Microtia was classified according to Marx classification. Developmental abnormalities of the temporal bone were evaluated by a computed tomographic (CT) scoring system modified after the system used by Jahrsdoerfer and colleagues, using high-resolution CT scans of the temporal bone. Correlations between the scores obtained from these 2 grading systems were evaluated using a nonparametric statistical method. Results Male preponderance and incidence of bilateral cases of approximately 30% were observed in our Japanese patients with Microtia. There was no significant difference in the severity of Microtia between unilateral and bilateral cases. The mean ± SEM total points in the CT scoring system (full marks, 10) was 7.9 ± 0.4 for grade I Microtia, 6.6 ± 0.6 for grade II, and 6.4 ± 0.3 for grade III; the total points correlated inversely with the Microtia grade. Development of the auricle correlated significantly with aeration in the middle ear spaces but not with ossicular development or formation of the oval/round windows. Proportion of acceptable surgical candidates according to the CT scoring system (>5 points) was 79% for grade I Microtia, 52% for grade II Microtia, and 65% for grade III Microtia. Conclusion The principle “the better developed the auricle, the better developed middle ear” was confirmed in Japanese patients with Microtia; however, even with grade II/III Microtia, more than half of the patients were considered suitable for atresia surgery.
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difference in facial canal anatomy in terms of severity of Microtia and deformity of middle ear in patients with Microtia
Laryngoscope, 2003Co-Authors: Hideki Takegoshi, Kimitaka KagaAbstract:Objective To study the difference in the facial canal anatomy in terms of the severity of Microtia and deformity of the middle ear in patients with Microtia using high-resolution computed tomography (HRCT). Study Design Forty-six ears with Microtia were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. Methods Forty-six ears with Microtia (unilateral Microtia, n = 12; bilateral Microtia, n = 34) were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings and age distribution of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. Results The mastoid portion of the facial nerve was 3 mm more anteriorly displaced in patients with grades II and III Microtia than in those with grade I Microtia (P <.01). From the scoring system that was used, the course of the facial nerve was not significantly different between patients with a score of 5 points or less and those with a score of 6 points or more. Conclusion The mastoid portion of the facial nerve in patients with grades II and III Microtia would be more anteriorly displaced because of hypoplasia of the second genu.
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Difference in facial canal anatomy in terms of severity of Microtia and deformity of middle ear in patients with Microtia.
The Laryngoscope, 2003Co-Authors: Hideki Takegoshi, Kimitaka KagaAbstract:Objective To study the difference in the facial canal anatomy in terms of the severity of Microtia and deformity of the middle ear in patients with Microtia using high-resolution computed tomography (HRCT). Study Design Forty-six ears with Microtia were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. Methods Forty-six ears with Microtia (unilateral Microtia, n = 12; bilateral Microtia, n = 34) were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings and age distribution of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. Results The mastoid portion of the facial nerve was 3 mm more anteriorly displaced in patients with grades II and III Microtia than in those with grade I Microtia (P
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facial canal anatomy in patients with Microtia evaluation of the temporal bones with thin section ct
Radiology, 2002Co-Authors: Hideki Takegoshi, Kimitaka Kaga, Shigeru Kikuchi, Ken ItoAbstract:PURPOSE: To ascertain the location of the facial nerve in patients with Microtia by using thin-section computed tomography (CT). MATERIALS AND METHODS: Thin-section CT was performed in 66 ears of patients with Microtia (unilateral, n = 12; bilateral, n = 34) and mandibulofacial dysostosis (MFD, n = 20). Findings were compared with those in 22 ears with normal auricles (control group) by using the Dunnett two-sided t test. RESULTS: The facial nerve at the mastoid portion in patients with MFD was 2 mm more lateral and 3 mm more anterior than that in control subjects (P < .01). The same portion in patients with Microtia was 3 mm more anterior than that in the control subjects (P < .01). The distance between the facial nerve and the most lateral point of the temporal bone in patients with MFD was 10 mm shorter and that in patients with bilateral Microtia was 3 mm shorter than that in the control subjects (P < .01). CONCLUSION: The facial nerve in patients with Microtia was not more lateral from the Bill bar (...
