A six-month-old baby with congenital patent ductus arteriosus (PDA), bilateral microtia and canal atresia was referred for hearing assessment. The audiology assessment revealed bilateral profound hearing loss, which is atypical for a case of pure canal atresia. Imaging was performed much earlier than usual and, as suspected, the patient also had bilateral severe inner ear anomaly. It is extremely rare for a person to have both external and inner ear anomaly because of the different embryological origin. The only suitable hearing rehabilitation option for this kind of patients is brainstem implant. However, the parents had opted for sign language as a form of communication. KEY WORDS: Microtia; canal atresia; cochleovestibular nerve aplasia; inner ear anomaly
Congenital Microtia

Superficial temporal artery (STA) based pedicled fascial flap plays a pivotal role in ear reconstruction for microtia patients. There is paucity of literature on the anatomy of the STA in microtia patients. The present study aimed to describe any possible anatomical variations seen in the STA of patients afflicted with microtia. Pre-operative carotid computer tomographic angiography images of patients under the microtia database of Plastic and Reconstructive Surgery Unit at a tertiary medical centre were selected and 3-dimensionally reconstructed. Measurements were made on the 3D reconstructed computed tomographic angiography images of the STA on both the sides of the microtic ear and the non-microtic ear to assess its various anatomical parameters. We managed to obtain a total of 39 computed tomographic angiography images of STAs for analysis. There was a significant difference in the number of main branches of STA between the two groups (P=0.006). The proportion of ears with 2 main branches was higher in the non-microtia group (89.5%) compared to the microtia group (45.0%). A significant difference was found in the STA diameter between the two groups (P=0.012). The mean diameter of STA in the non-microtia group was larger by 0.4 mm. Furthermore, the median angle of STA was larger on the side of the non-microtic ears compared to that of microtic ears by 24.5°, with a P-value of 0.011. The results of the study may be of clinical importance while planning and performing ear reconstructive surgeries using STA based pedicled fascial flaps.
Angiography ; Congenital Microtia* ; Ear ; Humans ; Plastics ; Temporal Arteries*

The complexity of the auricle makes its reconstruction technically difficult, owing to the convexities and concavities of the underlying cartilage with its tightly adherent and thin overlying skin. This article gives an overview of the current practice in the field of auricular reconstruction. The majority of surgeons who perform auricular reconstruction continues to employ the well-established techniques developed by Brent and Nagata. Surgery takes between two and four stages, with the initial stage being construction of a framework of autogenous rib cartilage which is implanted into a subcutaneous pocket. Several modifications of these techniques have been reported. Understanding the various treatment options and having sufficient practice to increase the surgeon's skills are crucial to providing the patients with the best outcomes possible.
Cartilage ; Congenital Microtia ; Costal Cartilage ; Humans ; Ribs ; Skin ; Surgeons

Congenital Microtia/surgery* ; Ear Auricle/surgery* ; Humans ; Reconstructive Surgical Procedures

Microtia is the second most common maxillofacial birth defect in neonates and has an prevalence rate of 3.06/10 000 in China, and 20%-60% of microtia cases is associated with a certain type of syndrome. This article elaborates on the clinical phenotypes and genetic characteristics of three microtia-associated syndromes (MASs) with high prevalence, high incidence rate of ear deformity, and definite genetic etiology, i.e., oculo-auriculo-vertebral spectrum, branchio-oto-renal spectrum disorder, and Treacher-Collins syndrome, and summarizes another three common MASs, so as to provide a reference for the genetic diagnosis of neonatal MAS.
China ; Congenital Microtia/genetics* ; Humans ; Infant, Newborn ; Phenotype ; Prevalence ; Syndrome

Preauricular sinus is a congenital malformation that is very commonly encountered among the general population and it has especially high prevalence among Asians when compared to other ethnic groups. It can often go unnoticed or easily overlooked by the patient or even by doctors because most of them are asymptomatic and is most of the time only a tiny pit that can be trivial in terms of aesthetics. We report a very rare and unique case that has no precedence what so ever; hence no reported case in the literature: an ectopic preauricular sinus in a facial cleft and microsomia patient.
Asian Continental Ancestry Group ; Branchial Region ; Congenital Microtia* ; Craniofacial Abnormalities ; Esthetics ; Ethnic Groups ; Humans ; Prevalence

Preauricular sinus is a congenital malformation that is very commonly encountered among the general population and it has especially high prevalence among Asians when compared to other ethnic groups. It can often go unnoticed or easily overlooked by the patient or even by doctors because most of them are asymptomatic and is most of the time only a tiny pit that can be trivial in terms of aesthetics. We report a very rare and unique case that has no precedence what so ever; hence no reported case in the literature: an ectopic preauricular sinus in a facial cleft and microsomia patient.
Asian Continental Ancestry Group ; Branchial Region ; Congenital Microtia* ; Craniofacial Abnormalities ; Esthetics ; Ethnic Groups ; Humans ; Prevalence

OBJECTIVETo investigate the characteristics and incidence of the thoracic deformities in patients with microtia.

METHODSIn Plastic Surgery Hospital, we conducted a retrospective study of the clinical and radiographical data of 300 patients with microtia from March 2013 to October 2014. Pearson χ2 test was used to analyze the relationship among deformities of ribs and spine, as well as microtia.

RESULTSA total of 78 (26.0%) patients were documented with rib deformities, 26 patients (8.7%) had spinal deformities, and 17 patients (5.7% )had both. The incidence of rib deformities in microtia I, II, and III was 7.1% (2/28), 26.7% (62/232) and 35.0% (14/40) respectively. The incidence of spinal deformities in microtia I, II, and III was 3.6% (1/28), 6.5% (15/232) and 25.0% (10/40 respectively. The patients with microtia III were found to have a higher incidence of ribs and spinal deformities than those with microtia II, patients with microtia II were found to have a higher incidence of ribs and spinal deformities than those with microtia I (P < 0.05).

CONCLUSIONSThe incidence of ribs and spinal deformities is high in patients with microtia. The poorer one auricle developed, the higher the incidence of thoracic deformities.

Biomedical Research ; Congenital Microtia ; epidemiology ; Humans ; Incidence ; Retrospective Studies ; Ribs ; abnormalities ; Spine ; abnormalities

Formation of an ideally-shaped tragus remains one of the most challenging issues during staged tragus reconstruction in microtia patients. The authors describe a new method used to treat a unique case of concha-type microtia in which the 10-year-old male patient had only a portion of pre-existing cartilage at the tragus site. An anomalous skin lump was also present. During the initial stages of the reconstruction, the two-stage Nagata method was used for surgical correction of the microtia. An autologous rib cartilage graft was used to form the ear framework. A temporoparietal fascia flap was also constructed. Remnant skin tags and anomalous cartilage that accompany microtia are usually removed during microtia repair. However, the cartilage and skin lump were preserved during the reconstruction. The skin lump was later used to form a vascularized chondrocutaneous island flap that supplemented the portion of cartilage during tragus formation. The result was a new tragus that was satisfactorily improved in both size and shape. Patients with concha-type microtia may benefit from the use of this new method for tragus formation.
Cartilage ; Child ; Congenital Microtia* ; Ear ; Ear Auricle ; Fascia ; Humans ; Male ; Methods ; Ribs ; Skin ; Surgical Flaps ; Transplants

OBJECTIVES: The aim of this study was to analyze the incidence of inner ear anomalies in patients with unilateral congenital aural atresia (CAA) combined with microtia. METHODS: We retrospectively reviewed 61 patients with unilateral CAA combined with microtia who underwent high-resolution temporal bone computed tomography (TBCT) and hearing examination. Inner ear anomalies were analyzed using TBCT and evaluated according to the Jahrsdoerfer grading system, Marx classification, and extent of inferior displacement of the mastoid tegmen. RESULTS: Inner ear anomalies were observed in 14 patients (23.0%). Lateral semicircular canal (LSCC) dysplasia was the most common inner ear anomaly, with an incidence of 16.4%. The incidence was significantly higher on the pathologic side than on the unaffected side (P=0.002). All vascular anomalies were observed in the high-riding jugular bulb, with an incidence of 24.6%. The incidence was significantly higher on the pathologic side than on the unaffected side (P<0.001). LSCC dysplasia was significantly more common in patients with a lower Jahrsdoerfer score (odds ratio, 0.66; P=0.004). CONCLUSION: The incidence of inner ear anomalies was relatively high in patients with unilateral CAA combined with microtia; LSCC dysplasia was the most common anomaly and the probability of coexistence was higher in patients with a lower Jahrsdoerfer score.
Classification ; Congenital Microtia ; Ear, Inner ; Hearing ; Humans ; Incidence ; Mastoid ; Retrospective Studies ; Semicircular Canals ; Temporal Bone