Congenitally deaf infants and children commonly suffer vestibular failure in both ears, and impairment of postural control, locomotion, and gait. The development of gross motor functions, such as head control, sitting, and walking is likely to be delayed, but fine motor function is usually preserved unless disorders of the central nervous system are present. These children can eventually catch up with their normal peers in terms of development and growth as a result of central vestibular compensation. The visual and somatosensory systems, pyramidal and extrapyramidal motor system (cerebellum, basal ganglia, cerebrum) and intellectual development, compensate for vestibular failure in infants and children with congenitally hypoactive or absent function of the semicircular canals and otolith organs.

CONCLUSIONS: The horizontal portion of the petrous carotid artery (HPCA) of mandibulofacial dysostosis (MFD) patients had anterior-inferior displacement or was aplasia at birth. Our findings indicate that most MFD patients have aplasia of the internal carotid artery. This information may be very important for safe reconstruction surgery in atresia. OBJECTIVE: MFD rarely has malformations of the cardiovascular system such as those seen in Goldenhar syndrome. This study aimed to compare MFD patients and normal subjects with respect to the length and diameter of the HPCA. SUBJECTS AND METHODS: We radiographically studied 22 MFD patients (44 sides) using high-resolution computed tomography (HRCT), measuring the length and diameter of the HPCA and the angle between the right and left HPCA using computer software. Findings were compared with those in 86 ears with normal auricles (control group) using the nonparametric test. RESULTS: The HPCA in MFD patients averaged 23.2 mm in length and 4.8 mm in diameter. The angle between the right and left HPCA averaged 100.2 degrees. In MFD patients, on average, HPCA were 2.0 mm shorter (p=0.001) and 0.4 mm narrower (p=0.016) than in control subjects. Mean angle between right and left HPCA was 5.4 degrees more acute in MFD patients than in control subjects (p=0.026). Moreover, the genu of the vertical and horizontal petrous ICA in the MFD group was on average 3.2 mm more inferior than in controls (p<0.001).

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 (<2)(64.7 +/- 1.6 dB) was significantly different (P =.03) from that in ears with high scores (>/=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.

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 mandibulofacial dysostosis using high-resolution computed tomography. STUDY DESIGN: Retrospective analyses. SETTING: The study was carried out at the Department of Otorhinolaryngology, University of Tokyo, Tokyo, Japan. PATIENTS: Thirty-six ears of 18 patients with mandibulofacial dysostosis were examined by high-resolution computed tomography. These ears were graded based on the Marx classification and Jahrsdoerfer scoring systems. MAIN OUTCOME MEASURES: The high-resolution computed tomography findings and age distribution of each group were compared with those of other groups by multiple comparison using Tukey's honestly significant difference test. RESULTS: The course of the facial nerve was not significantly different in terms of the severity of microtia and deformity of the middle ear. The bony cochlea in the patients with mandibulofacial dysostosis was displaced by a mean value of 2 mm more anteriorly and a mean value of 0.7 mm shallower than that in the cases with normal auricles. CONCLUSION: The facial nerve of patients with mandibulofacial dysostosis is displaced more anterolaterally than that of the cases with normal auricles; however, the auricle anomaly is not severe.

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.

Seven patients with Treacher-Collins syndrome were studied. All of patients were children or teenagers. Helical CT scanner (Toshiba) was used to reconstruct zygomatic arch and temporomandibular joint capsule on lateral aspect of temporal bone in five patients of microtia and atresia of both ears and two patients of narrow ear canals of both ears without microtia.Three-dimensional reconstructions of computed tomography on lateral aspect of temporal bone demonstrated various congenital abnormality including aplasia of zygomatic arch in seven patients and dislocation of temporomandibular joint capsule in seven patients.

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 (vertical crest) than that in control subjects. The facial nerve in patients with MFD was different from that in patients with microtia who had no other head anomalies.

We have developed a special purpose adaptor making it possible to use a commercially available high-speed camera to observe vocal fold vibrations during phonation. The camera can capture dynamic digital images at speeds of 600 or 1200 frames per second. The adaptor is equipped with a universal-type attachment and can be used with most endoscopes sold by various manufacturers. Satisfactory images can be obtained with a rigid laryngoscope even with the standard light source. The total weight of the adaptor and camera (including battery) is only 1010 g. The new system comprising the high-speed camera and the new adaptor can be purchased for about $3000 (US), while the least expensive stroboscope costs about 10 times that price, and a high-performance high-speed imaging system may cost 100 times as much. Therefore the system is both cost-effective and useful in the outpatient clinic or casualty setting, on house calls, and for the purpose of student or patient education.

CONCLUSIONS: Our results show that among the patients with inner ear malformations, there were two patients with sensory cells of sacculus and inferior vestibular neurons and at least five patients with inferior vestibular neurons only, but no patients without sensory cells and vestibular neurons, as determined on the basis of vestibular evoked myogenic potentials (VEMPs). OBJECTIVE: The aim of this study was to investigate whether sensory cells of sacculus or inferior vestibular neurons or both are present in cochlear implant patients with inner ear malformations, particularly common cavity (CC) deformity, using VEMPs. PATIENTS AND METHODS: Seven children with inner ear malformation who underwent cochlear implantation (CI) participated in this study. The patients had CC deformity (n=2), incomplete partition type I (n=2), incomplete partition type II (n=1), or a narrow internal auditory canal (IAC)(n=2). It was possible to record VEMPs before and after CI in three patients and not before but after CI in four patients. After surgery, VEMPs were recorded with the cochlear implant device switched both off and on. RESULTS: Before the CI, two patients showed VEMPs and one patient showed no VEMPs, whereas in four patients no VEMPs could be recorded. All the patients showed VEMPs with the cochlear implant on.

We have developed a special purpose adaptor making it possible to use a commercially available high-speed camera to observe vocal fold vibrations during phonation. The camera can capture dynamic digital images at speeds of 600 or 1200 frames per second. The adaptor is equipped with a universal-type attachment and can be used with most endoscopes sold by various manufacturers. Satisfactory images can be obtained with a rigid laryngoscope even with the standard light source. The total weight of the adaptor and camera (including battery) is only 1010 g. The new system comprising the high-speed camera and the new adaptor can be purchased for about $3000 (US), while the least expensive stroboscope costs about 10 times that price, and a high-performance high-speed imaging system may cost 100 times as much. Therefore the system is both cost-effective and useful in the outpatient clinic or casualty setting, on house calls, and for the purpose of student or patient education.

OBJECTIVE: To study the epidemiologic characteristics of microtia in China and to investigate the possible risk factors with respect to the classification of microtia. METHODS: A total of 345 patients with microtia were studied. All patients were taken an intentional physical examination and classified into five types. A detailed questionnaire concerning the maternal conditions during pregnancy was filled out by patient's mother. The frequencies of the relative factors were counted and the variables were statistically analyzed using Chi Square and Fisher's tests in the five types of microtia. RESULTS: Most cases of microtia (88.12%) were sporadic and 72.75% of all cases occurred in males. It was more seen unilateral, especially affected on the right side (55.94%). A total of 195 patients (56.52%) were isolated microtia. In the rest of non-isolated microtia patients, 37.97% of the cases had hemifacial microsomia, which was the most common associated deformity. Thirty-four patients (9.86%) belonged to typical familial microtia. Three maternal factors showed significant differences in the five types of microtia, which were perinatal virus infection, high prior miscarriages and prevention treatments for threatened abortion. CONCLUSIONS: The majority of microtia cases in China are sporadic and usually more common in males. Mothers who have prior miscarriages over 3 times or perinatal virus infection seem to be more likely to have severe microtia infants.

The auricle derives from six hillocks arising from the first and second branchial arches. Different hillocks give rise to different parts of the pinna. In the course of embryonic development, the auricle migrates postero-cranially as the mandible enlarges. Auricular malformations, such as microtia, are thought to be related to cell death of the first and second arch derivatives. The prevalence and characteristics of microtia vary in different populations. The prevalence ranges from 0.83 to 17.4 per 10,000. Microtia is more common in males, and right-sided dominance varies from 57 to 67%. The prevalence of aural atresia or stenosis varies from 55 to 93%. Microtia has been associated with numerous risk factors including race and gender. Genetic factors are likely to have an effect at least in some patients with microtia.

OBJECTIVE: To present in detail a reconstruction technique using the remnant ear in lobule-type microtia. METHODS: By comparing the location of residual ear and the contralateral normal ear, we classified 3 different types of the remnant ear. Three techniques of transposition were applied to adjust the location of the reconstructed ear. RESULTS: The reconstructed earlobes not only looked real but also were symmetrical with the contralateral normal ears. CONCLUSION: The remnant ear is a key factor-in addition to the framework and covering skin-in ear reconstruction in microtia.

INTRODUCTION AND OBJECTIVES: Microtia is a major malformation of the auricle, comprising a clinical spectrum ranging from a slight reduction in the size of the auricle or one of its parts to the complete absence of the pinna (anotia). Its prevalence varies according to the region of the world it is evaluated in. We analyzed a range of maternal, neonatal, and familial variables in a case group and a control group, and compared them with the existing literature. METHODS: We collected information from the Latin-American Collaborative Study on Congenital Malformations (ECLAMC) gathered between 2001 and 2006, where we found 27 cases of isolated microtia; we also collected information from 103 control subjects. Data were analyzed using Student's t and odds ratio (OR). RESULTS: Microtia distribution was 3 (11.1 %) patients with grade I microtia, 19 (70.4 %) with grade II microtia, 2 (7.4 %) with grade III microtia. We found no patients with anotia. Regarding laterality, the right side was involved more often. Male-to-female ratio was 1.7:1. Birthweight pound 2,500 g produces an OR of 3.25 (95 % CI, 1.11-9.58) for the development of microtia. CONCLUSIONS: Microtia may be directly or indirectly associated with the early onset of labour. Future studies should include long-term follow up of the patients in order to detect possible anomalies of the oculo-auriculo-vertebral spectrum. It is also important to take anthropometric measurements to increase the likelihood of detecting cases of grade I microtia and mid-face hypoplasia, and to define with greater accuracy whether isolated microtia is the mildest form of the oculo-auriculo-vertebral syndrome.

Congenital malformations of the external ear are relatively rare, however gradual increase in their frequency has been observed in the last years. These defects can occur as isolated congenital malformations, but they can coexist with congenital malformations of facial skeleton and also with congenital defects of distant organs. The purpose of this study was to determine coexisting facial skeleton defects and congenital defects of distant organs in a group of patients with unilateral or bilateral congenital malformations of the external ear. 37 patients age 1 to 30 [mean age 12.4 years] with anotia, microtia or aplasia of external ear canal were part of this study. In 17 examined patients [46.0% of all examined persons] microtia could be treated as an isolated malformation, however hypoplasia of other elements formed from branchial arches, including mandibula, cheek or oral cavity, was found in 15 examined patients [40.5%]. In 11 [29.7%] patients congenital malformations of the external ear were found together with the congenital defects of distant organs (kidney, heart, muscular and skeletal system), and in two patients [5.4%] the defects involved even two different distant organs. Defects of the distant organs were found more frequently in patients with bilateral malformations of the external ear than in patients with unilateral ear malformation. In authors opinion each patient, even with seemingly isolated microtia, should be closely examined in order to exclude probable congenital defects of the distant organs.

OBJECTIVE: To determine the proportion of hemifacial microsomia (HFM) in patients with unilateral or bilateral "isolated" microtia. DESIGN: Prospective cohort clinical study. SETTING: University-affiliated, tertiary referral clinic for patients with microtia. PATIENTS: One hundred consecutive patients with isolated microtia. INTERVENTIONS: All the patients underwent a clinical examination and audiologic evaluation. The OMENS classification system was used to grade the severity of craniofacial features: orbital deformity, mandibular hypoplasia, ear deformity, nerve (cranial nerve VII) involvement, and soft-tissue deficiency. Each anatomical abnormality was graded from 0 (normal) to 3 (most severe)(score range, 0-15). MAIN OUTCOME MEASURES: The OMENS scores, percentage of patients with isolated microtia and undiagnosed HFM, and isolated microtia as an early clinical marker for HFM. RESULTS: Forty patients (40%) with microtia were determined to have HFM (31 unilateral and 9 bilateral). Mean patient age was 9.2 years (range, 6 weeks to 41 years), with male predominance (27 males and 13 females). The OMENS scores were less than 5 in 24 patients and 6 to 10 in 16 patients. Thirty patients had cranial nerve deficits, and 37 had mandibular asymmetry. Thirty-seven patients demonstrated conductive hearing loss, and 1 had sensorineural hearing loss. CONCLUSIONS: Isolated microtia served as an early clinical marker for asymmetrical facial growth in 40% of the patients. Isolated microtia and HFM could represent a spectrum of expression of the same developmental phenomenon.

OBJECTIVE: To compare the characteristics of microtia in Finland and in other populations. METHODS: Retrospective case series and patient questionnaire of 190 microtia patients referred for reconstruction of the earlobe to the Helsinki University Central Hospital during the years 1980-2005. RESULTS: The prevalence in Finland is 4.34/10,000 and varied in other populations from 0.83 to 17.4/10,000. Microtia is seen more in males (58%), as unilateral (88.4%), right-sided (59.5%) and it is almost always associated with aural atresia or stenosis (93%). There is conductive hearing loss in 96% and sensorineural hearing loss in 8% of the affected ears. 11% of the patients had congenital heart defects, and 5% had anomalies of extremities. CONCLUSIONS: There is variation in the prevalence and characteristics of microtia in different populations.