Imaging in a Rare Case of Unilateral External and Middle Ear Anomalies Associated with Inner Ear Malformation (original) (raw)
Related papers
American Journal of Roentgenology, 1985
The external auditory canal, middle ear, and bulk of the ossicular chain develop from the first branchial groove, first and second branchial arches, and first pharyngeal pouch. Embryologic development of these structures is complex and only rarely are two anomalies identical. Development of the inner ear structures occurs independently of external ear structures, and concomitant involvement is unusual. This study includes 11 cases of unilateral external auditory canal atresia and two cases of bilateral atresia. Eight cases (four bilateral) of isolated congenital ossicular anomalies are also included. Emphasis is placed on findings of surgical import. All patients were studied with computed tomography only, because it was believed that the bony and soft-tissue detail achieved is superior to that with conventional multidirectional tomography.
Classification of Congenital Middle and External Ear Malformations: CT Study
The Medical Journal of The Islamic Republic of Iran, 1991
The authors used high-resolution computed tomography (HRCT) for studying 52 congenitally malformed ears in 45 children between five and 10 years of age. In six children the malformations were bilateral. The malforma tions clinically manifested as microtia, atresia of the external auditory canal and conductive deafness. Analyzing anatomical details and pathological changes on HRCT axial sections the authors established three groups of malformations. In the first group, auditory assicles were malformed in almost all cases, in the second group besides deformed auditory ossicles in majority of cases, the mastoid was apneumatised, and in the third group there were malformed auditory ossicles and the mastoids were apneumatised and in more than half of cases cavum tympani was malformed or filled with mesenchyme. These features have great importance in surgical reconstruction of congenital middle ear malformations. MJIRI, Vo1.5, No.3 & 4,105-109,199
Clinical Course of Pediatric Congenital Inner Ear Malformations
The Laryngoscope, 2000
To determine any factors that could improve the early detection and management of congenital inner ear malformations. Study Design: A retrospective review was performed of all patients with a diagnosis of inner ear malformation at Loyola University Medical Center (LUMC) and the Hospital for Sick Children (HSC) between 1987 and 1995. Clinical records and audiometric data were accumulated. One neuroradiologist reviewed every temporal bone computed tomography (CT) scan. Methods: Forty-six pediatric patients with congenital inner ear anomalies evaluated at two tertiary care hospitals. Results: The average patient age at initial assessment was 25.7 months. A family history of hearing loss was noted in only five patients (12.8%). A major nonotological deformity was seen in 41% of patients. The average hearing threshold was 88 dB. All three patients with sudden hearing loss had vestibular aqueduct enlargement. Two of the three patients with common cavity anomalies had a history of recurrent meningitis. Twenty-seven patients had a vestibular aqueduct deformity, the most frequent radiographic abnormality in the series. Conclusions: Because inner ear malformation was diagnosed after 24 months of age in a significant percentage of patients, we recommend increased parental education and vigilance by primary care practitioners. Universal newborn screening may be the key to earlier detection of these infants. For children with idiopathic sensorineural hearing loss, we recommend a temporal bone CT scan. Patients with vestibular aqueduct enlargement must be counseled about the risk of progressive sensorineural hearing loss, meningitis, and the need to avoid contact sports. Patients with common cavity abnormalities should be considered for exploratory tympanotomy and also educated about the risk for meningitis.
Congenital sensorineural hearing loss due to Inner ear malformations: Spectrum on CT and MRI
Introduction Inner ear malformations is an important cause of congenital sensorineural hearing loss .Cross sectional imaging playing a crucial role not only in the diagnosis but also for preoperative evaluation in candidates deemed fit for cochlear implant. Aim To study the various causes of SNHL on HRCT temporal bone and MRI cochlea. Material And Methods A prospective study was conducted to study spectrum of causes in 30 cases evaluated over a period of 2 years. Results Out of 30 patients, 18 were female and 12 were males with a mean age 4 years. All patients presenting with congenital SNHL were evaluated with HRCT/MRI or both also includinga T2WI axial screening of the brain to evaluate brainstem or any intracranial abnormality. Audiometry and BERA testswere also performed in all cases. Around 2/3 rd of the cases were positive for varying types of inner ear malformations as per the Sennaroglu classification. Conclusion : As imaging plays a key role in the evaluation and management of congenital SNHL ,knowledge about the anatomy and classification is a must for accurate diagnosis .
Congenital defects of the middle ear - uncommon cause of pediatric hearing loss1,2
Brazilian Journal of Otorhinolaryngology, 2014
Introduction: in children, hypoacusis, or conductive hearing loss, is usually acquired; otitis media with effusion is the most common etiology. However, in some cases this condition is congenital, ranging from deformities of the external and middle ear to isolated ossicular chain malformations. The non-ossicular anomalies of the middle ear, for instance, persistent stapedial artery and anomaly of the facial nerve, are uncommon but may accompany the ossicular defects. Objective: This study aimed to describe the clinical presentation, diagnostic tests, and therapeutic options of congenital malformations of the middle ear. Methods: This was a retrospective study of cases followed in otolaryngologic consultations since 2007 with the diagnosis of congenital malformation of the middle ear according to the Teunissen and Cremers classification. A review of the literature regarding the congenital malformation of the middle ear and its treatment is presented. Conclusion: Middle ear malformations are rarely responsible for conductive hearing loss in children. As a result, there is often a late diagnosis and treatment of these anomalies, which can lead to delays in the development of language and learning.
CT and MR imaging of congential abnormalities of the inner ear and internal auditory canal
European Journal of Radiology, 2001
The embryology of the inner ear must be known as many of the inner ear malformations present as a result of the arrest during the various stages of embryology. These malformations are described in this 'embryologic' perspective and specific names for certain malformations are no longer used. Both CT and MR can be used to look at inner ear malformations but often both techniques are complementary. However, CT is preferred when associated middle-or external ear malformations must be excluded. Magnetic resonance is preferred when subtle changes in the membranous labyrinth or abnormalities of the nerves in the internal auditory canal must be visualised. The CT and MR technique must however be adapted as more and more subtle congenital malformations can only be seen when the right technique is used. The heavily T2-weighted gradient-echo or fast spin-echo MR techniques are mandatory if malformations of the inner ear must be excluded. The purpose of this paper is to describe the techniques used to study these patients and to give an overview of the most frequent and important congenital malformations which can be found in the inner ear and internal auditory canal/cerebellopontine angle. : S 0 7 2 0 -0 4 8 X ( 0 1 ) 0 0 3 7 7 -1
Congenital defects of the middle ear - uncommon cause of pediatric hearing loss
Brazilian Journal of Otorhinolaryngology, 2014
Introduction: in children, hypoacusis, or conductive hearing loss, is usually acquired; otitis media with effusion is the most common etiology. However, in some cases this condition is congenital, ranging from deformities of the external and middle ear to isolated ossicular chain malformations. The non-ossicular anomalies of the middle ear, for instance, persistent stapedial artery and anomaly of the facial nerve, are uncommon but may accompany the ossicular defects. Objective: This study aimed to describe the clinical presentation, diagnostic tests, and therapeutic options of congenital malformations of the middle ear. Methods: This was a retrospective study of cases followed in otolaryngologic consultations since 2007 with the diagnosis of congenital malformation of the middle ear according to the Teunissen and Cremers classification. A review of the literature regarding the congenital malformation of the middle ear and its treatment is presented. Conclusion: Middle ear malformations are rarely responsible for conductive hearing loss in children. As a result, there is often a late diagnosis and treatment of these anomalies, which can lead to delays in the development of language and learning.
Congenital malformations of the ear
The Indian Journal of Pediatrics, 1992
Tile external ear develops from I and II branchial arches commencing on 38th day of fetal life. The middle ear is formed from the ends of Ist pharyngeal puch and the surrounding mesenchyme, which also is part of the I and II branchial arches. The congenital defects of the external and middle ear usually occurs in combination, and many times with congenital defects of other systems. The cochlear functions i.e. bone conduction is normal in 50% of these cases, thus rehabilitation of these patients with congenital anomalies of external and middle ear is possible. The coexistence of congenital aural atresia with varying degrees of malformation of inner ear may be more frequent than generally assumed. Moderate and severe forms of congenital aural atresia area encountered in about 1 in 10,000 to 20,000 individualJ Tile more severe forms of congenital auricular malformation are always associated with meatal atresia, whereas meatal atresia may, in a few cases be seen in patients with a normal pinna. Atresia of the meatus may be membranous or osseus, membranous atresia is much less common and is characterised by rudimentary cartilagenous canal sep~ated from the middle ear by a dense structure of con
Unclassified congenital deformities of the external ear
Indian Journal of Plastic Surgery
ABSTRACTCongenital ear deformities are a common entity. They are found in isolation or as a part of syndrome in patients. They may involve the external, middle or inner ear or in any of these combinations. Three patients of different ages presented with deformities including mirror image duplication of the superior auricle, unclassified deformities of ear lobule (wavy lobule) and deformity of superior auricle with unclassified variety of lateral ear pit. This article highlights that there are further cases of ear deformities that are noticed in the general population who come for cosmetic correction, and hence, there is a need for further modifying the classification of ear deformities.
Clinical Otolaryngology and Allied Sciences, 1999
The value of computed tomography "CT# of the petrous bone in the investigation of congenital sensorineural hearing impairment has been questioned[ We have conducted a study to establish the usefulness of CT of the temporal bone in the evaluation and management of a consecutive series of unselected adolescent patients with congenital sensorineural hearing impairment of greater than 49 dB HL[ Seventy!one patients "031 ears# were identi_ed and images reviewed to establish the incidence of inner ear malformations[ Fifteen ears were found to be abnormal in eight patients "seven bilateral and one unilateral abnormality#[ Three patients had Mondini abnormalities and one of these also had dilatation of the lateral semicircular canals[ There were _ve patients with dilatation of the vestibular aqueduct[ One patient had a unilateral dysplasia of the middle and external ear[ A variety of incidental intracranial abnormalities were also discovered[ We conclude that CT does have a valuable role in the management of SNHI[ Keywords petrous computed tomo`raphy con`enital ear malformation sensorineural hearinì mpairment