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Aural atresia

Janez Dolenšek, Erika Cvetko, Žiga Snoj, Marija Meznaric
Fusion of the atlas with the occipital bone is a rare congenital dysplasia known as occipitalization of the atlas, occipitocervical synostosis, assimilation of the atlas, or atlanto-occipital fusion. It is a component of the paraxial mesodermal maldevelopment and commonly associated with other dysplasias of the craniovertebral junction. External auditory canal atresia or external aural atresia is a rare congenital absence of the external auditory canal. It occurs as the consequence of the maldevelopment of the first pharyngeal cleft due to defects of cranial neural crest cells migration and/or differentiation...
February 18, 2017: Surgical and Radiologic Anatomy: SRA
Kenny H Chan, Dexiang Gao, Emily L Jensen, Gregory C Allen, Stephen P Cass
OBJECTIVE: To assess long-term complication rate and parental satisfaction of osseointegrated bone conduction hearing implants (OBCHIs). STUDY DESIGN: Retrospective chart review of children undergoing OBCHIs. METHODS: A retrospective chart review of children undergoing OBCHIs for the treatment of conductive, mixed, and single-sided sensorineural hearing loss in children. RESULTS: Forty-five subjects were identified with 0...
January 16, 2017: Laryngoscope
Rubens de Brito, Luzia Maria Pozzobom Ventura, José Carlos Jorge, Eduardo Boaventura Oliveira, Luiz Fernando Manzoni Lourencone
OBJECTIVE: To demonstrate the surgical technique and audiological results of a middle ear implant for restoring hearing in patients with bilateral aural atresia and microtia with conductive or mixed hearing loss. MATERIALS AND METHODS: In this prospective study, 12 subjects aged 12 years and older presenting with hearing loss and bilateral congenital aural atresia underwent surgical insertion of a middle ear implant. The patients underwent tympanomastoidectomy with a wide opening of the attic and preservation of the roof of the atresic tympanic bone to expose the middle ear and position the floating mass transducer of the implant...
December 2016: Journal of International Advanced Otology
Kimitaka Kaga, Hirotaka Asato
CONCLUSION: Each of eight patients with bilateral microtia and atresia underwent bilateral reconstruction of the auricles and external auditory canals and were fitted bilateral canal-type hearing aids in the operated ears to replace a bone conduction hearing aid. The ability to discriminate inter-aural intensity difference (IID) and even inter-aural time difference (ITD) was retained in all these patients. OBJECTIVE: This study studied the post-operative sound lateralization ability of patients with bilateral microtia and atresia after total reconstruction of both auricles and external auditory canals, followed by fitting of bilateral canal-type hearing aids...
November 11, 2016: Acta Oto-laryngologica
Elisa Tassano, Mariasavina Severino, Silvia Rosina, Riccardo Papa, Domenico Tortora, Giorgio Gimelli, Cristina Cuoco, Paolo Picco
BACKGROUND: Deletions of the long arm of chromosome 18 cause a common autosomal syndrome clinically characterized by a protean clinical phenotype. CASE PRESENTATION: We report on a 16-month-old male infant affected by fever attacks apparently unrelated with any infectious or inflammatory symptoms, growth retardation, bilateral vertical talus, congenital aural atresia, dysmorphisms, mild psychomotor delay, and peculiar neuroradiological features. Array-CGH analysis revealed one of the smallest 18q22...
2016: Molecular Cytogenetics
M Leinung, E Zaretsky, B P Ernst, B Trier, T Stöver, C Hey
No abstract text is available yet for this article.
October 11, 2016: Laryngo- Rhino- Otologie
Randall A Bly, Amit D Bhrany, Craig S Murakami, Kathleen C Y Sie
Microtia reconstruction is a challenging endeavor that has seen significant technique evolution. It is important to educate patients and their families to determine the best hearing rehabilitation and ear reconstructive options. Microtia is often associated with aural atresia, hearing loss, and craniofacial syndromes. Optimal care is provided by multiple disciplines, including a reconstructive surgeon, an otologic surgeon, an audiologist, and a craniofacial pediatrician. Microtia management includes observation, prosthetic ear, autologous cartilage reconstruction, or alloplastic implant placement...
November 2016: Facial Plastic Surgery Clinics of North America
Hong Chan Kim, Chul Ho Jang, Chung Man Sung, Eun Kyung Jung, Yong Beom Cho
No abstract text is available yet for this article.
September 12, 2016: Brazilian Journal of Otorhinolaryngology
Vânia Henriques, Rafaela Teles, Ana Sousa, Roberto Estevão, Jorge Rodrigues, Alexandra Gomes, Francisco Silva, Ângelo Fernandes, Fausto Fernandes
Congenital middle ear malformations are rare. Most part of them are usually associated with other malformations, such as aural atresia, microtia, and dysmorphic craniofacial features. A clinical case of a 24-year-old male with a right-sided conductive hearing loss since his childhood, without craniofacial malformation, is presented. He was proposed for exploratory tympanotomy under the suspicious diagnosis of otosclerosis. The surgery revealed an abnormal location of stapes' superstructure, which was attached to the promontory and had an isolated and mobile osseous footplate in the oval window...
2016: Case Reports in Otolaryngology
Shane Zim, Janet Lee, Brian Rubinstein, Craig Senders
OBJECTIVE:   The objective of this study was to determine whether patients with isolated microtia or aural atresia have an increased prevalence of renal or cervical vertebral anomalies. DESIGN:   The study design was a retrospective medical record review. SETTING:   The setting was the following four distinct institutions: an urban tertiary care children's hospital, two urban academic medical centers, and a staff-model health maintenance organization...
September 15, 2016: Cleft Palate-craniofacial Journal
Robert Reed, Matthew Hubbard, Bradley W Kesser
OBJECTIVES: To compare speech/language development and academic progress between children with right versus left congenital aural atresia (CAA). STUDY DESIGN: Case control survey and review of audiometric data. SETTING: Tertiary care academic practice. PATIENTS: Children with unilateral CAA. MAIN OUTCOME MEASURES: Demographic and audiometric data; rates of grade retention, use of any hearing or learning resource, and behavioral problems...
September 14, 2016: Otology & Neurotology
M Leinung, Y Zaretsky, B Ernst, B Vaerst, T Stöver, C Hey
BACKGROUND: The audiological treatment of children with aural atresia makes high demands on physicians and acousticians. Conventional hearing systems are often not tolerated by children and therefore do not meet the needs of the early and efficient therapy of hearing disorders. Aim of the present study was the evaluation of the audiological functional gain in children with uni- and bilateral aural atresia provided with the middle ear implant Vibrant Soundbridge(®) (VSB(®)) below the age of 6 years as well as the analysis of parents' satisfaction assessed with questionnaires...
September 2016: Laryngo- Rhino- Otologie
M Leinung, E Zaretsky, B P Lange, V Hoffmann, T Stöver, C Hey
The aim of this pilot study was to determine the acceptance and benefit from the middle ear implant system Vibrant Soundbridge(®) (VSB, MED-EL Corporation, Austria) by means of a questionnaire, compared to a previously used conventional bone conducting hearing device, in preschool children with unilateral congenital aural atresia. Prospective cohort study. All nine children with unilateral congenital aural atresia used the VSB and had previous experience with a bone conducting hearing device. The benefit from the VSB was evaluated by questionnaires concerning acceptance of hearing aids, handling, listening effort, behavior, quality of life, and the duration of daily use and compared to the experience with the bone conducting hearing device...
January 2017: European Archives of Oto-rhino-laryngology
Hetal Marfatia, Ratna Priya, Nilam U Sathe, Shampa Mishra
Baha has proven performance and advantages for patients with aural atresia or chronic ear drainage. Being a per cutaneous osseo integrated implant the skin around the fixture is in contact with a foreign body that leads to chronic inflammatory reaction and complications such as skin irritation and overgrowth occur frequently. Challenges that are faced during Baha surgery include those during candidate selection, work up, anesthesia related as well as intra-operative and post operative complications. Ours was a retrospective and observational study where case records of 10 patients who underwent Baha surgery in the Department of E...
September 2016: Indian Journal of Otolaryngology and Head and Neck Surgery
Danni Wang, Shouqin Zhao, Qingguo Zhang, Yi Li, Xiaobo Ma, Ran Ren
OBJECTIVES: To report an integrating surgical method of Vibrant SoundBridge (VSB) implant and auricle reconstruction. METHODS: 4 cases of congenital bilateral external/middle ear malformation were enrolled. All of them were diagnosed as bilateral bony atresia and grade III microtia. Stapes vibroplasty was performed for all subjects in one operation with auricle reconstruction stage 3. The clinical information and hearing outcomes were analyzed. RESULTS: Satisfied aesthetic outcomes were confirmed by sculptured outline and symmetric shape on both sides...
July 2016: International Journal of Pediatric Otorhinolaryngology
Ritu Verma, Manisha Jana, Ashu Seith Bhalla, Arvind Kumar, Rakesh Kumar
Aural atresia is a rare congenital malformation of the external and middle ear. There are several syndromic associations of this anomaly with those involving the first and second branchial arches. Occurrence of aural atresia with sclerosing skeletal dysplasia is unknown and has never been reported. The co-existence of a sclerosing dysplasia can make the surgical treatment in aural atresia difficult and risky; and the auditory improvement may not be as expected. Moreover, internal auditory canal narrowing and hence sensorineural hearing loss in sclerosing dysplasia might add to the already existing conductive hearing loss in such patients...
May 8, 2016: World Journal of Clinical Pediatrics
Keguang Chen, Dongming Yin, Huiying Lyu, Lin Yang, Tianyu Zhang, Peidong Dai
CONCLUSIONS: With the aggravation of the external auditory canal malformation, the size of extra-niche fossa became smaller, providing concrete data and valuable information for the better design, selecting and safer implantation of the transducer in the area of round window niche. Three-dimensional measurements and assessments before surgery might be helpful for a safer surgical approach and implantation of a vibrant soundbridge. OBJECTIVES: The aim of this study was to investigate whether differences exist in the morphology of the posterior tympanum related to the round window vibroplasty among congenital aural atresia (CAA), congenital aural stenosis (CAS), and a normal control group, and to analyze its effect on the round window implantation of vibrant soundbridge...
2016: Acta Oto-laryngologica
Kimitaka Kaga, Hirotaka Asato
CONCLUSION: In patients with unilateral microtia and atresia after reconstruction of the auricle and external canal and fitting of a canal-type hearing aid for the operated ear, the ability to discriminate the inter-aural intensity difference (IID) was acquired in all of the patients, whereas that to discriminate inter-aural time difference (ITD) was acquired in one-half of the patients. OBJECTIVE: To study the post-operative sound lateralization ability in patients with unilateral microtia and atresia after reconstruction surgery of the auricle and external canal and fitting of a canal-type hearing aid of the operated ear...
2016: Acta Oto-laryngologica
Kun Chen, Chenyan Jiang, Qingwei Wu, Yiyuan Sun, Runjie Shi
Atresiaplasty is still a challenge for otolaryngologist, although the operation technique has been modified several times over the past decades. This study describes a reliable flap technique for combining atresiaplasty with total auricular reconstruction by performing the total auricular reconstruction with the MEDPOR framework at the same time as the atresiaplasty for patients with microtia and congenital aural atresia. We performed a prospective study of the medical records of 18 consecutive patients with congenital aural atresia...
December 2015: Indian Journal of Surgery
Joong-Wook Shin, Sung Huhn Kim, Jae Young Choi, Hong-Joon Park, Seung-Chul Lee, Jee-Sun Choi, Han Q Park, Ho-Ki Lee
OBJECTIVES: Bone-anchored hearing aids (BAHA) occasionally cause soft tissue problems due to abutment. Because Sophono does not have abutment penetrating skin, it is thought that Sophono has no soft tissue problem relating to abutment. On the other hand, transcutaneous device's output is reported to be 10 to 15 dB lower than percutaneous device. Therefore, in this study, Sophono and BAHA were compared to each other from surgical and audiological points of view. METHODS: We retrospectively reviewed the medical records of 9 Sophono patients and 10 BAHA patients...
March 2016: Clinical and Experimental Otorhinolaryngology
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