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Acta Otolaryngol 2022 Jun 23:1-6. Epub 2022 Jun 23.

Department of Ear Reconstruction, Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Background: Retrosigmoid sinus (RS) approach was not the dominant choice for BONEBRIDGE implantation.

Objective: To investigate the efficacy and safety of auricular reconstruction concomitant with BONEBRIDGE implantation using retrosigmoid approach for aural atresia patients.

Materials And Methods: A retrospective analysis was conducted of 15 children (28 ears) who underwent auricular reconstruction using a completely expanded postauricular flap with a skin expander concomitant with retrosigmoid BONEBRIDGE implantation from July 2019 to September 2020. Read More

Laryngoscope Investig Otolaryngol 2022 Jun 20;7(3):863-869. Epub 2022 Apr 20.

Division of Pediatric Otolaryngology, Department of Otolaryngology-Head & Neck Surgery University of California San Francisco California USA.

Objectives: To describe the prevalence and significance of first branchial cleft anomalies in children with congenital aural atresia.

Methods: Retrospective cohort study and case series. Patients were included if they had ICD-10 code Q16. Read More

Acta Otorhinolaryngol Ital 2022 Apr;42(2):182-188

King Abdullah Ear Specialist Center (KAESC), College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Objectives: To evaluate the safety, speech performance in noise and subjective satisfaction of patients with congenital aural atresia (CAA) implanted with the active middle ear implant.

Methods: This retrospective study included 13 patients (15 ears) implanted with middle ear implants with different methods of floating mass transducer attachment. In 6 ears, the floating mass transducer (FMT) was coupled with the short process of incus; in 8 ears, a clip coupler was used; and in one ear, a round window coupler was used. Read More

Facial Plast Surg 2022 May 17. Epub 2022 May 17.

Otolaryngology- Head and Neck Surgery, University of Washington School of Medicine, Seattle, United States.

Microtia techniques have evolved to improve aesthetic outcomes, reduce donor site morbidities, and reduce complications. Patients with microtia commonly have aural atresia associated with conductive hearing loss. We present the evolution of our technique for microtia reconstruction and considerations for hearing management in these patients. Read More

Otolaryngol Head Neck Surg 2022 Apr 19:1945998221094230. Epub 2022 Apr 19.

Department of Otolaryngology-Head and Neck Surgery, McGill University, Montreal Children's Hospital, Montreal, Canada.

Objective: Patients with congenital external auditory canal (EAC) abnormalities are at risk of developing cholesteatoma and often undergo surveillance imaging to detect it. The aims of this systematic review are to determine the incidence of cholesteatoma in patients with congenital aural atresia (CAA) and patients with congenital EAC stenosis and to investigate the most common age of cholesteatoma diagnosis. This information will help clinicians decide which patients require surveillance scanning, as well as the timing of imaging. Read More

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2022 Mar;57(3):338-344

Department of Facial Plastic and Reconstructive Surgery, ENT institute, Eye & ENT Hospital, NHC Key Laboratory of Hearing Medicine (Fudan University), Shanghai 200031, China.

To investigate whether the contralateral normal external auditory canal (EAC) skin graft can maintain the ear canal health after EAC reconstruction in unilateral congenital aural atresia (CAA) cases. A Zelen design randomized controlled study was used to collect unilateral CAA patients for EAC reconstruction prospectively (clinical trial registration number: ChiCTR2000032103). The patients were randomly divided into the control group and the trial group. Read More

Ear Hear 2022 Mar 23. Epub 2022 Mar 23.

Department of Otolaryngology - Head and Neck Surgery, University of California - San Francisco, San Francisco, California, USA Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel School of Medicine - Health Faculty, University of Angers, Angers, France Center for Craniofacial Anomalies, University of California - San Francisco, San Francisco, California, USA These authors contributed equally to this work.

Objective: To describe risk factors for speech and language delay in a diverse population of children with aural atresia.

Methods: Retrospective chart review was performed from 2012 to 2020 at UCSF Benioff Children's Hospital to identify children with aural atresia evaluated for speech, language, or auditory skills delays. Ninety-five children with aural atresia, conductive hearing loss, and assessment of speech, language, or auditory skills delay were included. Read More

Int J Pediatr Otorhinolaryngol 2022 May 17;156:111114. Epub 2022 Mar 17.

Department of Otolaryngology-Head & Neck Surgery, University of California, San Francisco, CA, USA. Electronic address:

Objective: To assess the outcomes of pediatric Osia 2® System placements.

Methods: We performed a retrospective chart review of primary and revision Osia 2® System surgical cases at two tertiary academic children's hospitals. Operative details and post-operative surgical and audiologic outcomes were recorded. Read More

Int J Pediatr Otorhinolaryngol 2022 Apr 16;155:111075. Epub 2022 Feb 16.

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

Objective: To determine whether children with unilateral sensorineural hearing loss (USNHL) and unilateral conductive hearing loss (UCHL) have higher levels of fatigue than literature reported normal hearing (LRNH) children.

Methods: This was a cross-sectional survey utilizing the PedsQL™ Multidimensional Fatigue Scale administered to children with unilateral hearing loss (UHL) and their parents at two tertiary care academic medical centers and a nationwide microtia/atresia conference. The PedsQL™ Multidimensional Fatigue Scale was used to compare child and parental proxy reports of fatigue among USNHL, UCHL, and LRNH children. Read More

J Surg Case Rep 2022 Feb 11;2022(2):rjac037. Epub 2022 Feb 11.

Department of Anatomy, Medical School, National and Kapodistrian University of Athens, 75 Mikras Asias str., 11527 Goudi, Greece.

Congenital aural atresia refers to abnormal embryological development of the external auditory canal. The treatment of this anatomical malformation is only surgical repair. This article reports a case of unilateral congenital aural atresia, the surgical procedure performed and the post-operative findings. Read More

Clin Otolaryngol 2022 05 11;47(3):478-482. Epub 2022 Feb 11.

Department of Otolaryngology Head and Neck Surgery, Key Laboratory of Otolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

Acta Otolaryngol 2022 Jan 31;142(1):23-29. Epub 2021 Dec 31.

Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul, Korea.

Background: Treatment options for congenital aural atresia (CAA) include canaloplasty and implantation of an osseointegrated bone conduction device (OBCD). Few studies have compared hearing outcomes in these two treatment methods.

Objectives: Hearing outcomes and revision surgery rates were compared in CAA patients managed by canaloplasty and surgically implanted OBCD. Read More

Laryngoscope Investig Otolaryngol 2021 Dec 10;6(6):1429-1435. Epub 2021 Nov 10.

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

Objectives: Providing hearing compensation to patients with aural atresia is considerably challenging. Hearing aid transducers vibrating the aural cartilage (cartilage conduction; CC) have been devised, and hearing aids utilizing them (CC hearing aids) have quickly become a beneficial option for aural atresia in clinical applications. However, it remains unclear which placement (on the aural cartilage or mastoid) is beneficial to signal transmission. Read More

Otol Neurotol 2022 03;43(3):320-327

Department of Otolaryngology - Head and Neck Surgery, University of Virginia, Charlottesville, Virginia.

Objective: Compare surgical and audiological outcomes of patients with congenital aural stenosis (CAS) with cholesteatoma to patients with CAS without cholesteatoma and patients with complete congenital aural atresia (CCAA).

Study Design: Retrospective case series.

Setting: Tertiary care hospital. Read More

Cleft Palate Craniofac J 2021 Oct 29:10556656211050006. Epub 2021 Oct 29.

Division of Molecular Genetics and Cancer, Nitte University Centre for Science Education and Research (NUCSER), 231912Nitte (Deemed to be University), Mangaluru, India.

Objective: Facial dysostosis is a group of rare craniofacial congenital disabilities requiring multidisciplinary long-term care. This report presents the phenotypic and genotypic information from South India.

Design: The study is a case series. Read More

Audiol Res 2021 Sep 30;11(4):508-523. Epub 2021 Sep 30.

Department of Communication Design Science, Faculty of Design, Kyushu University, Fukuoka 815-0032, Japan.

Sound localization in daily life is one of the important functions of binaural hearing. Bilateral bone conduction devices (BCDs), middle ear implants, and cartilage conduction hearing aids have been often applied for patients with conductive hearing loss (CHL) or mixed hearing loss, for example, resulting from bilateral microtia and aural atresia. In this review, factors affecting the accuracy of sound localization with bilateral BCDs, middle ear implants, and cartilage conduction hearing aids were classified into four categories: (1) types of device, (2) experimental conditions, (3) participants, and (4) pathways from the stimulus sound to both cochleae. Read More

Ann Med Surg (Lond) 2021 Oct 22;70:102880. Epub 2021 Sep 22.

Department of Otorhinolaryngology-Head and Neck Surgery, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin General Hospital, Bandung, Indonesia.

Background: External auditory canal (EAC) cholesteatoma is a lesion lined with stratified squamous epithelium containing proliferative keratin with bony erosion in EAC which can spread to the tympanic cavity, mastoid, and surrounding organ. External cholesteatoma can occur in patients with congenital abnormalities such as congenital aural atresia (CAA).

Method: This case series was reported using the 2020 PROCESS Guideline. Read More

Laryngoscope 2022 05 23;132(5):1088-1092. Epub 2021 Oct 23.

Department of Otolaryngology-Head and Neck Surgery, Hospital for Sick Children, Toronto, Canada.

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2021 Sep;56(9):936-942

Department of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.

To evaluate the auditory efficacy and subjective satisfaction of adhesive bone conduction hearing aid in children with unilateral congenital aural atresia (UCAA). Ten subjects (5 males and 5 females) diagnosed with UCAA with an average age of 8.3 years old (ranged from 5 to 15) were included in Beijing Tongren Hospital, Capital Medical University from January to August 2019. Read More

Otol Neurotol 2021 12;42(10):1527-1533

Otorhinolaryngology Department, Hospital das Clínicas, University of São Paulo, São Paulo, Brazil.

Objective: To describe the audiological results and complications following active middle ear implant (AMEI) surgery in users with bilateral ear atresia.

Study Design: Observational and retrospective longitudinal follow-up.

Setting: A tertiary referral hospital for hearing rehabilitation and craniofacial malformations. Read More

Med Sci Monit 2021 Sep 25;27:e933915. Epub 2021 Sep 25.

World Hearing Center, Institute of Physiology and Pathology of Hearing, Warsaw/Kajetany, Poland.

BACKGROUND The aim of this study was to assess the effectiveness of bone conduction hearing aids in children under 2 years old who have congenital microtia and atresia. MATERIAL AND METHODS This prospective study involved 42 children under 2 years old with congenital microtia and atresia who were divided into 2 groups: 21 with unilateral defect and 21 with bilateral defect. All children were provided with bone conduction hearing aids on a softband. Read More

Curr Opin Otolaryngol Head Neck Surg 2021 Dec;29(6):526-533

Department of Otolaryngology-Head and Neck Surgery.

Purpose Of Review: The management of patients with unilateral microtia and aural atresia is complex. Recent literature suggests significant strides in hearing habilitation and ear reconstruction.

Recent Findings: Several options of hearing management are available and are associated with improved outcomes. Read More

JNMA J Nepal Med Assoc 2020 Dec 31;58(232):1080-1082. Epub 2020 Dec 31.

Department of ENT and Head & Neck Surgery, TU Teaching Hospital, Maharajgunj Medical Campus, Institute of Medicine, Kathmandu, Nepal.

Congenital external canal atresia is one of the congenital ear anomalies that can occur in patients. Similarly, congenital cholesteatoma is also another congenital disease that is often diagnosed in early adulthood. Both the above-mentioned diseases can occur independently but the presence of both these entities is a rare occurrence and needs a high degree of suspicion aided by a computed tomography scan to make the diagnosis. Read More

Eur Arch Otorhinolaryngol 2022 Jul 31;279(7):3371-3378. Epub 2021 Aug 31.

Department of Otorhinolaryngology, Ghent University Hospital, Corneel Heymanslaan 10, 9000, Ghent, Belgium.

Purpose: Most developed countries have implemented some form of universal newborn hearing screening program. Early identification and rehabilitation of congenital hearing loss is important in functional outcome, and the need to identify the cause of hearing impairment has become clear. We aimed to evaluate audiological and etiological outcomes in a large group of patients with failed neonatal hearing screening. Read More

Audiol Res 2021 Aug 13;11(3):410-417. Epub 2021 Aug 13.

Nara Medical University, Kashihara 634-8522, Nara, Japan.

Hearing improvement represents one of the may valuable outcomes in microtia and aural atresia reconstruction surgery. Most patients with poor development in their hearing function have had a severe microtia. Conventional methods to improve hearing function are bone conduction and bone anchored hearing aids. Read More

ORL J Otorhinolaryngol Relat Spec 2021 25;83(6):471-477. Epub 2021 Aug 25.

ColIege of Otolaryngology Head and Neck Surgery, Chinese PLA G General Hospital, Beijing, China.

Cochlear implantation (CI) is a safe and beneficial surgery for children with congenital inner ear malformations, with the exception of cochlear nerve aplasia. The combination of microtia with middle and inner ear abnormalities is extremely uncommon and sufficiently severe to make a surgical approach to the cochlea difficult. We report herein the case of a 2-year-old girl who presented with profound bilateral sensorineural hearing loss, congenital aural atresia, microtia, and inner ear malformations. Read More

Int Arch Otorhinolaryngol 2021 Jul 24;25(3):e374-e378. Epub 2020 Sep 24.

Department of ENT, Universidad Nacional de Cordoba, Sanatorio Allende, Cordoba, Córdoba, Argentina.

 The transmastoid approach is the most recommended technique to Bonebridge surgery, while in patients with bad anatomy or in the canal wall down technique, retrosigmoid or Middle Fossa Approaches are the alternative surgical options.  To describe a novel alternative approach called inverted middle fossa approach (IMFA) and its technique and audiological outcomes.  Seven patients submitted to the IMFA were included. Read More

Int J Audiol 2021 Aug 9:1-7. Epub 2021 Aug 9.

MBT (Medicine-Based Town) Institute, Nara Medical University, Kashihara, Japan.

Objective: Congenital aural atresia causes severe conductive hearing loss disturbing auditory development. The differences in speech recognition were investigated between bilateral and unilateral aural atresia.

Design: The maximum speech recognition scores (SRSs) were compared between patients with bilateral and unilateral aural atresia. Read More

Curr Probl Diagn Radiol 2022 Jul-Aug;51(4):599-616. Epub 2021 Jul 12.

Department of Radiodiagnosis, Maulana Azad Medical College and Associated Lok Nayak, G.I.P.M.E.R. and G.N.E.C. Hospitals, New Delhi, DL, India.

Congenital aural atresia (CAA) is characterized by hypoplasia or aplasia of external auditory canal associated with auricular deformity. It also embodies a spectrum of temporal bone abnormalities including anomalies of middle ear, ossicles, facial nerve, oval window, round window, mastoid pneumatization, temporomandibular joint and occasionally inner ear and vascular anomalies. The management in such patients is variable and consists of rehabilitative hearing aids or surgical correction depending on various patient factors like associated temporal bone abnormalities, degree of hearing impairment and laterality. Read More

Front Neurol 2021 8;12:687070. Epub 2021 Jul 8.

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