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Int J Pediatr Otorhinolaryngol 2019 Apr 3;122:117-125. Epub 2019 Apr 3.

Department of Otorhinolaryngology, Head and Neck Surgery, St. Elisabeth-Hospital, Ruhr University Bochum, Bochum, Germany.

Objectives: Bone conduction hearing devices integrated in softbands (BCDSs) are frequently not well accepted by children with conductive hearing loss due to pressure on the head, sweating, or cosmetic stigma. A non-surgical hearing system (ADHEAR) uses a new bone conduction concept consisting of an audio processor connected to an adhesive adapter fixed behind the ear. This study is the first to evaluate the audiological and clinical outcome of this novel system, comparing it with conventional BCDSs in a short- and mid-term follow-up in children under 10 years of age. Read More

Otolaryngol Clin North Am 2019 Apr;52(2):195-210

Department of Otolaryngology, Long Island Jewish Medical Center, Hearing and Speech Center, Northwell Health, 430 Lakeville Road, New Hyde Park, NY 11042, USA.

Implantable auditory devices (IADs) are a viable hearing restoration option for patients with hearing loss. Conditions such as chronic otitis externa, congenital aural atresia, and chronic otitis media can be treated with a variety of implants. Progressive disease are also amenable to restoration with IADs, providing stabilized hearing. Read More

J Formos Med Assoc 2019 Feb 20. Epub 2019 Feb 20.

School of Medicine, Chang Gung University, Taoyuan, Taiwan; Craniofacial Research Center, Division of Craniofacial Surgery, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Taoyuan, Taiwan. Electronic address:

Background/purpose: The Bonebridge (BB) is a newly designed transcutaneous bone conduction hearing implant. We describe, for the first time, simultaneous BB implantation and different surgical techniques of auricular reconstruction for microtia patients with aural atresia/stenosis.

Methods: Ten patients with unilateral or bilateral microtia underwent BB implantation combined simultaneously with either total auricular reconstruction using bespoke hand-carved Medpor framework or second stage auricular projection using autologous costal cartilage framework. Read More

J Prev Alzheimers Dis 2019 ;6(2):112-120

Yakeel T. Quiroz, PhD Assistant Professor, Harvard Medical School, Departments of Psychiatry and Neurology, Massachusetts General Hospital, 100 1st Avenue, Building 39, Suite 101, Charlestown, MA 02129, Phone (617) 643-5944; Fax: (617) 726-5760, E-mail:

The study of individuals with autosomal dominant Alzheimer's disease affords one of the best opportunities to characterize the biological and cognitive changes of Alzheimer's disease that occur over the course of the preclinical and symptomatic stages. Unifying the knowledge gained from the past three decades of research in the world's largest single-mutation autosomal dominant Alzheimer's disease kindred - a family in Antioquia, Colombia with the E280A mutation in the Presenilin1 gene - will provide new directions for Alzheimer's research and a framework for generalizing the findings from this cohort to the more common sporadic form of Alzheimer's disease. As this specific mutation is virtually 100% penetrant for the development of the disease by midlife, we use a previously defined median age of onset for mild cognitive impairment for this cohort to examine the trajectory of the biological and cognitive markers of the disease as a function of the carriers' estimated years to clinical onset. Read More

Auris Nasus Larynx 2019 Apr 2;46(2):151-159. Epub 2019 Feb 2.

Department of Otolaryngology-Head and Neck Surgery, Nara Medical University, Kashihara, Japan.

It has been long considered that air and bone are the two major mediators that conduct sounds to the inner ear. In 2004, Hosoi found that vibration of aural cartilage, generated by placing gently a transducer on it, could create audible sound with the same level of clarity as air- and bone-conduction sound. He thus proposed the term "cartilage conduction" for this concept. Read More

Dermatol Online J 2019 Jan 15;25(1). Epub 2019 Jan 15.

Department of Dermatology, Indiana University School of Medicine, Indianapolis, Indiana.

A 26-year-old man with a history of congenital bilateral microtia, unilateral renal agenesis, left aural atresia, and right external auditory canal occlusion admitted for right rib cartilage graft harvest and left ear re-construction. Following surgery, an ulceration with violaceous borders and a yellow fibrinous base unresponsive to broad-spectrum antibiotics developed at the harvest site. The wound was expanding and not responsive to systemic broad-spectrum antibiotics. Read More

Auris Nasus Larynx 2019 Jan 19. Epub 2019 Jan 19.

Department of Otolaryngology-Head and Neck surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan.

Objective: Cartilage conduction (CC) is a new transduction form, and hearing devices that utilize CC present a new option for patients with aural atresia. However, in occluded ears, low-tone sounds are transmitted very effectively, resulting in excessive low-tone sound emphasis and speech recognition reduction. This study aimed to clarify low-tone speech recognition for CC in occluded ears, and determine if excessive low-tone sound emphasis decreases maximum speech recognition scores. Read More

Int J Pediatr Otorhinolaryngol 2019 Apr 14;119:54-58. Epub 2019 Jan 14.

Sanatorio Allende, Cordoba, Argentina.

Objectives: The objective of this study is to evaluate the safety and efficacy of the transcutaneous Bone Conduction Implant, the Bonebridge, in patients with congenital aural atresia.

Methods: Audiometry, speech recognition test and free field audiometry were performed. Word recognition scores and speech perception was evaluated using Spanish phonetically-balanced disyllables word list. Read More

Int J Pediatr Otorhinolaryngol 2019 Mar 7;118:165-169. Epub 2019 Jan 7.

Department of Otorhinolaryngology, Head and Neck Surgery "Otto Koerner", Rostock University Medical Center, Rostock, Germany.

Objectives: Foreign body incorporation in children is often a serious situation. Attention deficit hyperactivity disorder (ADHD) could be a risk factor for self-insertion of foreign bodies. Large cohort analyses are missing. Read More

Otolaryngol Clin North Am 2019 Apr 3;52(2):265-272. Epub 2019 Jan 3.

House Clinic, 2100 West 3rd Street, Suite 111, Los Angeles, CA 90057, USA. Electronic address:

Bonebridge is an active bone conduction device that consists of a bone conduction-floating mass transducer (BC-FMT) and magnet internally and an audio processor externally. Surgery for implantation can be performed under local anesthesia but requires surgical planning for adequate bone depth for the BC-FMT well. Bonebridge does not require osseointegration to function, so the device can be activated early. Read More

Indian J Otolaryngol Head Neck Surg 2018 Dec 29;70(4):587-590. Epub 2018 May 29.

Department of ENT, Sri Devaraj Urs Academy of Higher Education and Research, Kolar, India.

The term congenital aural atresia is used to describe failure of external auditory canal to open. Thus it results in conductive hearing loss. There are ways to improve the transmission of sound to the healthy inner ear, we have performed surgery to open the ear canal and restore the natural sound-conducting mechanism to the inner ear. Read More

Clin Exp Otorhinolaryngol 2019 May 7;12(2):176-180. Epub 2018 Nov 7.

Department of Otolaryngology-Head and Neck Surgery, Korea University College of Medicine, Seoul, Korea.

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. Read More

Int J Pediatr Otorhinolaryngol 2018 Nov 8;114:175-179. Epub 2018 Sep 8.

Department of Otorhinolaryngology, University Medical Center Utrecht, Heidelberglaan, 100, 3584, CX, Utrecht, The Netherlands; Cochrane Netherlands, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands. Electronic address:

Background: Little is known about the academic performance of children with unilateral congenital aural atresia (CAA).

Objective: of review: Our objective was to summarize what is known about the academic performance of children with hearing loss by unilateral congenital aural atresia, in order to provide pragmatic recommendations to clinicians who see children with this entity.

Type Of Review: Systematic review. Read More

Auris Nasus Larynx 2019 Apr 7;46(2):204-209. Epub 2018 Sep 7.

Sen-En Rifu Otologic Surgery Center, Miyagi-gun, Miyagi, Japan.

Objective: A method of Vibrant Soundbridge (VSB) placement to the round window (RW) via the retrofacial approach with preoperative evaluation of the relationship between the facial nerve (FN) and RW by 3D-CT reconstruction was proposed for the treatment of congenital aural atresia (CAA) patient.

Methods: A fenestration to the mesotympanum was made mastoid portion of the FN. During the approach, part of the stapedial muscle was encountered and removed. Read More

Hear Res 2018 Dec 26;370:238-247. Epub 2018 Aug 26.

Department of Biophysics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen, Nijmegen, The Netherlands; Department of Otorhinolaryngology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre Nijmegen, Nijmegen, The Netherlands.

Different amplification options are available for listeners with congenital unilateral conductive hearing loss (UCHL). For example, bone-conduction devices (BCDs) and middle ear implants. The present study investigated whether intervention with an active BCD, the Bonebridge, or a middle ear implant, the Vibrant Soundbridge (VSB), affected sound-localization performance of listeners with congenital UCHL. Read More

Otolaryngol Head Neck Surg 2018 Oct 7;159(4):601-602. Epub 2018 Aug 7.

2 Department of Head and Neck Surgery, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Intraoperative facial nerve monitoring (IOFNM) has evolved from requiring sophisticated electromyography equipment to a self-contained monitor with an auditory signal. Subspecialty ear surgeons currently use IOFNM in most otologic and temporal bone procedures as it improves facial nerve outcomes. Our competency and near-universal adoption of IOFNM notwithstanding, otolaryngologists are rarely reimbursed for this procedure. Read More

J Laryngol Otol 2018 Aug 16;132(8):693-697. Epub 2018 Jul 16.

Department of Otorhinolaryngology - Head and Neck Surgery,Sarawak General Hospital,Malaysia.

Objectives: To investigate the surgical and audiological outcomes of the Bonebridge transcutaneous bone conduction hearing implant among children with congenital aural atresia.

Methods: Six children were recruited and underwent Bonebridge transcutaneous bone conduction implant surgery. The patients' audiometric thresholds for air conduction, bone conduction and sound-field tests were assessed pre-operatively and at six months post-operatively. Read More

JAMA Otolaryngol Head Neck Surg 2018 Aug;144(8):704-709

Division of Pediatric Otolaryngology-Head and Neck Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.

Importance: Patients with aural atresia typically have maximal conductive hearing loss, which can have negative academic and social consequences. Transcutaneous osseointegrated implants (TOIs) can potentially restore hearing on the affected side.

Objectives: To review the demographic, audiological, and surgical outcomes of TOI placement in pediatric patients with aural atresia and to describe a modification in incision technique in anticipation of later auricular reconstruction. Read More

Int J Pediatr Otorhinolaryngol 2018 Jul 5;110:118-122. Epub 2018 May 5.

Department of Otolaryngology-Head & Neck Surgery, Emory University School of Medicine, 1365 Clifton Road, Atlanta, GA 30322, USA. Electronic address:

Objectives: The right jugular foramen is usually larger than the left in Homo sapiens, but it is unknown if right-sided jugular dominance is also more common amongst those with unilateral congenital aural atresia. We hypothesized that the dominant (i.e. Read More

Oral Surg Oral Med Oral Pathol Oral Radiol 2018 Oct 7;126(4):e233-e239. Epub 2018 May 7.

Professor and Director, Division of Oral and Maxillofacial Radiology, Diagnostic Sciences, Texas A&M University College of Dentistry, Dallas, TX, USA.

Background: Goldenhar syndrome, also known as oculo-auriculo-vertebral spectrum, is a variant of hemifacial microsomia, a congenital condition affecting primarily aural, oral, and mandibular development, with vertebral anomalies, ocular abnormalities, and epibulbar dermoids. As a complex and heterogeneous condition, this disorder varies from mild to severe, and involvement is unilateral in many cases, but bilateral involvement is also known to occur. It presents several complex problems in treatment, requiring an integrated, multidisciplinary approach. Read More

Otol Neurotol 2018 Jun;39(5):602-608

Department of Otorhinolaryngology-Head and Neck Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine.

Objective: The aim of this study was to correlate the postoperative hearing outcomes with regard to the length of prosthesis of the partial ossicular replacement prosthesis (PORP) in patients with congenital aural atresia.

Study Design: Retrospective review of medical records.

Setting, Patients, Intervention, Main Outcome Measure: The medical records of 131 patients (132 ears) who underwent canaloplasty with PORP by a single surgeon from 2011 to 2016 were reviewed for demographic data, Jahrsdoerfer score, grade of microtia, length of prosthesis, and audiometric outcomes. Read More

Arch Oral Biol 2018 Jul 6;91:23-34. Epub 2018 Apr 6.

Operative Unit of Anthropology, Department of Medicine and Aging Sciences, 'G. d'Annunzio' University of Chieti-Pescara, Piazza Trento e Trieste 1, 66100 Chieti, Italy.

Background: This study is based in an analysis of the skeletal remains of an adult male from the Teramo Sant'Anna archaeological site (7th-12th centuries of the Common Era, Teramo, Italy).

Results And Discussion: The individual shows distinct abnormalities that principally involve asymmetric hypoplasia and dysmorphogenesis of the facial skeleton. The combination of these findings and the absence of abnormalities of the spine strongly suggest diagnosis of the congenital malformation known as hemifacial microsomia. Read More

Acta Otorrinolaringol Esp 2019 Jan - Feb;70(1):32-35. Epub 2018 Mar 20.

Otoneurology Department, Sanatorio Allende, Cordoba, Argentina.

Introduction: Congenital Aural Atresia (CAA) or microtia is a malformation that results in esthetic and functional problems. There is little information on prevalence, considering that Latin American is the most affected region in the world.

Objective: To determine the prevalence of microtia, considering the different ethnical structure of the population. Read More

Hear Res 2019 Feb 17;372:88-98. Epub 2018 Feb 17.

Neurosciences & Mental Health, The Hospital for Sick Children, Toronto, ON, Canada; Institute of Medical Sciences, The University of Toronto, Toronto, ON, Canada; Collaborative Program in Neuroscience, The University of Toronto, Toronto, ON, Canada; Department of Otolaryngology - Head & Neck Surgery, The Hospital for Sick Children, Toronto, ON, Canada; Department of Otolaryngology - Head & Neck Surgery, The University of Toronto, Toronto, ON, Canada.

This longitudinal study aimed to identify auditory plasticity promoted by a cochlear implant in children with asymmetric hearing loss. Participants included 10 children who experienced (mean ± SD) 3.1 ± 3. Read More

Int J Pediatr Otorhinolaryngol 2018 Apr 1;107:176-182. Epub 2018 Feb 1.

Department of Otolaryngology, Head and Neck Surgery, Starship Children's Health, Park Road, Grafton, Auckland 1023, New Zealand. Electronic address:

Introduction: To compare the audiological results of Bone Conduction Hearing Aid (BCHA) on hard test band and Bonebridge (BB) implant among children with microtia and atresia.

Methods: This is a retrospective review of patients with microtia and atresia who underwent BB implant insertion from September 2014 to February 2017 in Starship Children's Hospital. Preoperative audiological testing using a powered BCHA (Oticon Medical Ponto Pro Power) on a hard test band was used to compare post-operative hearing assessments with the BB. Read More

Cochlear Implants Int 2018 07 17;19(4):234-238. Epub 2018 Feb 17.

a Department of Otolaryngology , Northwell Health , New Hyde Park , NY , USA.

Objective And Importance: Reports of patients with concurrent middle and inner ear anomalies are rare. These patients present a surgical challenge for cochlear implantation. The surgical risk must be weighed against the predicted benefit of the patient's hearing outcome and subsequent development of speech and language as well as their quality of life. Read More

Int J Pediatr Otorhinolaryngol 2018 Mar 3;106:10-15. Epub 2018 Jan 3.

Department of Pediatric Plastic Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address:

Objectives: The aim of our study was to investigate subjective information concerning parental experiences and preferences with regard to the initial information that is provided right after the birth of a child with microtia. The analysis of these data is intended to help professionals improve the way in which such conversations are conducted. As a result, future parents may feel better informed and, hence, better fit to cope with challenges they may encounter having a child with microtia. Read More

Acta Otolaryngol 2018 Jul 9;138(7):621-624. Epub 2018 Feb 9.

a Department of Otorhinolaryngology , Tampere University Hospital, and School of Medicine, University of Tampere , Tampere , Finland.

Objectives: To evaluate long-term benefits of atresiaplasty on hearing and the impact of surgery on quality of life (QoL) in congenital aural atresia (CAA) patients.

Methods: We evaluated the long-term hearing results, the impact of atresiaplasty on QoL, the meatal diameter of the operated ear canal, and the cumulative number of post-operative hospital visits in 14 CAA patients, on average, 12 years (range: 4-17 years) post-operatively.

Results: The mean preoperative pure tone average (PTA) was 61 dB HL. Read More

Am J Case Rep 2018 Jan 23;19:82-89. Epub 2018 Jan 23.

Audiology Unit, Department of Clinical Science and Community Health, University of Milano, IRCCS Fondazione Cà Granda, Ospedale Maggiore Policlinico, Milano, Italy.

BACKGROUND Implantable devices have been proposed as an alternative to hearing aids and auditory canal reconstruction in patients with microtia (congenital aural atresia), which includes a malformation of the external and middle ear. This report is of two rare cases of microtia associated with congenital stapes ankylosis treated with an implantable device and describes the treatment outcomes. CASE REPORT Two siblings from Ecuador, a 29-year-old woman, and her 35-year-old brother, were born with unilateral type II microtia with bilateral external auditory canal atresia and conductive hearing loss. Read More

Lancet Neurol 2018 03 10;17(3):197-198. Epub 2018 Jan 10.

Stroke Research Centre, Department of Brain Repair and Rehabilitation, University College London Institute of Neurology, National Hospital for Neurology and Neurosurgery, London WC1N 3BG, UK. Electronic address:

Ear Nose Throat J 2017 Dec;96(12):E38-E43

Department of Otolaryngology-Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, No. 1 Dong Jiao Min Xiang Rd., Dong Cheng District, Beijing, China 100730.

We conducted a prospective study to assess the value of high-resolution computed tomography (HRCT) in identifying facial nerve variations in patients with congenital aural atresia and to determine how they affect otologic reconstruction surgery. Our study population was made up of 65 patients (69 ears) aged 6 to 22 years (mean: 13.7) without regard to sex. Read More

Otol Neurotol 2018 01;39(1):65-72

Department of Otolaryngology-Head and Neck surgery.

Objective: To assess the benefits of a new type of hearing aid using cartilage conduction (CC) in patients with severe conduction hearing loss and evaluate its potential for practical use.

Study Design: Consecutive, prospective case series.

Patients: Forty-one subjects (21 with bilateral aural atresia; 15 with unilateral aural atresia; and 5 others) participated in this study. Read More

AJNR Am J Neuroradiol 2018 Feb 7;39(2):355-361. Epub 2017 Dec 7.

Otolaryngology-Head and Neck Surgery (D.S.R., B.W.K.), University of Virginia, Charlottesville, Virginia.

Background And Purpose: Middle ear space is one of the most important components of the Jahrsdoerfer grading system (J-score), which is used to determine surgical candidacy for congenital aural atresia. The purpose of this study was to introduce a semiautomated method for measuring middle ear volume and determine whether middle ear volume, either alone or in combination with the J-score, can be used to predict early postoperative audiometric outcomes.

Materials And Methods: A retrospective analysis was conducted of 18 patients who underwent an operation for unilateral congenital aural atresia at our institution. Read More

Sci Rep 2017 Dec 4;7(1):16900. Epub 2017 Dec 4.

Institute of Medical Sciences, The University of Toronto, Toronto, ON, M5S 1A8, Canada.

Early treatment of single sided deafness in children has been recommended to protect from neurodevelopmental preference for the better hearing ear and from social and educational deficits. A fairly homogeneous group of five young children (≤3.6 years of age) with normal right sided hearing who received a cochlear implant to treat deafness in their left ears were studied. Read More

World Neurosurg 2018 Feb 28;110:386-390. Epub 2017 Nov 28.

Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA; Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA. Electronic address:

Background: Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma-multiple myeloma (EP-MM) and the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder.

Case Description: A 59-year-old woman presented with 4 months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia. Read More

Facial Plast Surg Clin North Am 2018 Feb;26(1):87-96

Department of Otolaryngology-Head and Neck Surgery, University of Virginia School of Medicine, PO Box 800713, Charlottesville, VA 22908, USA. Electronic address:

Patients with microtia and congenital aural atresia should have a comprehensive hearing assessment early in life. Options for hearing habilitation should be presented, and children with bilateral aural atresia should be fitted with a bone conducting hearing device to support normal speech and language development. If atresia surgery is pursued, the microtia surgeon must be aware of certain principles. Read More

Int J Pediatr Otorhinolaryngol 2017 Nov 28;102:174-179. Epub 2017 Sep 28.

Department of Neurosciences, ENT Clinic, Padua University Hospital, Via Giustiniani 2, 35128 Padua, Italy. Electronic address:

Objectives: Congenital Aural Atresia (CAA) is a deformity of the external ear and it is commonly associated with malformations of middle and inner ear and, in some cases, with other facial deformities. Very few assessment measures exist for evaluating the functional impairment in children with CAA. Purpose of this study is to introduce and describe an assessment Checklist, (nominated FOS Checklist) that covers feeding abilities (F), oralmotor skills (O), communication/language development (S) in children with CAA. Read More

Ann Otol Rhinol Laryngol 2017 Dec 27;126(12):835-838. Epub 2017 Oct 27.

1 The George Washington University School of Medicine and Health Sciences, Washington, DC, USA.

Introduction: Soft tissue occlusion of the external auditory canal (EAC) can cause intense pruritis, recurrent foul smelling otorrhea, recurrent otitis externa, and conductive hearing loss. Occlusion of the EAC can be challenging to treat as the area is prone to circumferential scarring.

Methods: We describe the novel use of serial bipolar radiofrequency ablation (coblation) to treat 3 children with complete EAC occlusion from congenital and acquired conditions including lymphedema (1), microcystic lymphatic malformation (1), and venolymphatic malformation (1). Read More

Otol Neurotol 2017 12;38(10):1470-1475

*University of New Mexico Health Sciences Center †Presbyterian Ear Institute, Albuquerque, New Mexico.

Objective: To compare temporal bone thickness along a three-dimensional arc of potential osseointegrated implant sites for bone-anchored hearing aids in children with and without aural atresia using computed tomographic imaging (CT).

Study Design: Retrospective case review.

Setting: Tertiary children's hospital. Read More

J Clin Diagn Res 2017 Aug 1;11(8):MD01-MD03. Epub 2017 Aug 1.

Professor, Department of Otorhinolaryngology and Head-Neck Surgery, Medical College and Hospital, College Street Kolkata, Kolkata, West Bengal, India.

Acquired atresia of External Auditory Canal (EAC) is seldom encountered in routine otolaryngology practice. Apart from resulting in moderate-to-severe conductive hearing impairment, it is a potentially dreaded condition which might lead to canal cholesteatoma. Suspected to develop as a consequence of a pre-existing chronic otitis externa/media, the EAC atresia leads to proximal (medial) accumulation of desquamated epithelium and denatured keratin (the canal cholesteatoma) that further leads to aggravation of the chronic otitis, thereby initiating a vicious cycle. Read More

Int J Pediatr Otorhinolaryngol 2017 Oct 18;101:230-234. Epub 2017 Aug 18.

ENT Institute, Eye & ENT Hospital of Fudan University, China; Hearing Medicine Key Laboratory, National Health and Family Planning Commission, China. Electronic address:

Objectives: Our aims were to measure and compare anatomical parameters of the ossicles in normal, congenital aural stenosis (CAS), and congenital aural atresia (CAA) ears.

Methods: This retrospective study was performed using three-dimensional reconstructed images derived from computed tomography scans of 20 normal subjects, 20 CAS patients, and 20 CAA patients.

Results: The lengths of the malleus handle and long process of the incus were greater in normal ears than in CAS and CAA ears (all P < 0. Read More

Int J Pediatr Otorhinolaryngol 2017 Oct 4;101:164-166. Epub 2017 Aug 4.

ENT Institute, Eye & ENT Hospital of Fudan University, Shanghai 200031, China; Hearing Medical Key Laboratory, National Health and Family Planning Commission, Shanghai 200031, China. Electronic address:

Objective: To investigate the deformations of temporal bone and mandible combined with congenital aural atresia.

Methods: A total of 158 patients with congenital aural atresia were included in the study. The raw CT data of the temporal bone was imported into MIMICS v 12 and threshold dissection, region growing and three-dimensional (3D) calculation were used to calculate 3D models. Read More

Indian J Otolaryngol Head Neck Surg 2017 Sep 11;69(3):363-369. Epub 2017 May 11.

Department of Otorhinolaryngology, All India Institute of Medical Sciences, Patna, Phulwarisharif, Patna, 801507 India.

External auditory canal atresia (EACA) is a common otologic condition. Etiology can vary from congenital to acquired causes. It causes considerable difficulty to the patient. Read More

Semin Plast Surg 2017 Aug 9;31(3):127-133. Epub 2017 Aug 9.

Department of Otolaryngology, Michael E. DeBakey Department of Surgery, Baylor College of Medicine.

Microtia is a congenital auricular deformity that commonly presents with associated congenital aural atresia. The most acute concern in these patients is concomitant hearing loss at birth. A team-based approach by plastic surgeons and otologists is necessary to address both the otologic and audiologic concerns of microtia and atresia. Read More

Otol Neurotol 2017 09;38(8):1140-1144

*Department of Otorhinolaryngology-Head and Neck Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine †Biostatics Team, Samsung Biomedical Research Institute, Seoul, Korea.

Objective: The aim of this study was to determine the most important factors in preoperative imaging, including components of Jahrsdoefer score (J score), and favorable prognostic factors for postoperative hearing results after canaloplasty for congenital aural atresia.

Study Design: Retrospective review of medical records.

Setting, Patients, Intervention, Main Outcome Measure: One hundred eight patients who underwent canaloplasty by a single surgeon between January 2011 and July 2014 were included. Read More

Laryngoscope 2018 05 3;128(5):1191-1195. Epub 2017 Jul 3.

Russell H. Morgan Department of Radiology and Radiological Science, Division of Pediatric Radiology, Johns Hopkins University School of Medicine, Baltimore, Maryland, U.S.A.

Objectives/hypothesis: We hypothesized that children with isolated nonsyndromic unilateral congenital aural atresia have subclinical mandibular condylar hypoplasia ipsilateral to the atretic ear, and that the Jahrsdoerfer score is associated with the degree of condylar hypoplasia.

Study Design: Retrospective self-controlled case series.

Methods: We reviewed high-resolution computed tomography scans of the temporal bones of 68 children with isolated nonsyndromic unilateral congenital aural atresia. Read More

Int J Pediatr Otorhinolaryngol 2017 Apr 29;95:5-8. Epub 2017 Jan 29.

Department of Otolaryngology-Head & Neck Surgery, Emory University School of Medicine, 1365 Clifton Road, Atlanta, GA, 30322, USA. Electronic address:

Objectives/hypothesis: We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia.

Methods And Materials: Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Read More

Otol Neurotol 2017 08;38(7):996-1004

*Department of Otorhinolaryngology, Head and Neck Surgery †Department of Otorhinolaryngology, Head and Neck Surgery, Division of Phoniatrics and Pediatric Audiology, Ruhr University Bochum, St. Elisabeth-Hospital, Bochum, Germany.

Objective: To evaluate the safety and effectiveness of coupling an active middle ear implant to the short process of the incus in subjects with meatal pathologies.

Study Design: Retrospective study.

Setting: Tertiary referral center. Read More

Acta Otolaryngol 2017 Sep 22;137(9):935-939. Epub 2017 May 22.

b Department of Radiology , Beijing Tongren Hospital, Capital Medical University , Beijing , China.

Objective: The objective of this study is to retrospectively analyze the imaging characteristics of patients with congenital aural atresia who underwent Vibrant Soundbridge implantation, and to investigate the importance of preoperative evaluation of vibroplasty.

Materials And Methods: The study included 16 patients with bilateral congenital aural atresia aged 6-25 years (mean age, 14.7 years). Read More

Acta Otorhinolaryngol Ital 2017 Oct;37(5):355-367

Department of Neuroscience, Institute of Otorhinolaryngology, University Hospital of Padua, Italy.

In the last 20 years, neonatal survival has progressively increased due to the constant amelioration of neonatal medical treatment and surgical techniques. Thus, the number of children with congenital malformations and severe chronic pathologies who need rehabilitative care has progressively increased. Rehabilitation programs for paediatric patients with disorders of voice, speech and language, communication and hearing, deglutition and breathing are not widely available in hospital settings or in long-term care facilities. Read More