Congenital defects of the middle ear - uncommon cause of pediatric hearing loss (original) (raw)
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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.
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
Surgical management of conductive hearing loss in children
Otolaryngologic Clinics of North America, 2002
Congenital middle ear anomalies pose a challenge to clinicians because they present in a myriad of ways; however, advances in the diagnosis and treatment of these relatively uncommon ear disorders over the past decade have simplified the care of patients who suffer from them. This article focuses on the evaluation of congenital middle ear anomalies (excluding congenital cholesteatoma and aural atresia) as well as their surgical management.
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
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.
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.
Prevalence of middle ear pathologies in children with bilateral sensorineural hearing loss
2006
Objectives: In poor countries, hearing aids are too expensive for sensorineural hearing loss (SNHL) children's parents to offer for their children. These children may have middle ear problem, this will aggravate the level of hearing loss which may lead to delay in their ability to speak. This study is to highlight the prevalence of middle ear pathology in SNHL children. Methods: Two hundred children with bilateral sensorineural hearing loss (SNHL) were selected in our study from the outpatient clinic of ENT department of Sohag University Hospital, Egypt. Children were classified into three categories according to their middle ear status. They were normal middle ear, middle ear with unhealthy tympanic membrane or otitis media with intact drum and chronic suppurative otitis media with perforation. Results: Seventy percent of cases were normal, 25% had middle ear problem with intact tympanic membrane and 5% had chronic suppurative otitis media with perforation. Conclusion: Thirty percent of SNHL children have middle ear pathology which aggravate the degree of hearing loss. Regular evaluation of SNHL children to treat those having middle ear pathology medically and/or surgically and this may help those having no ability to have hearing aids to learn language early.
Modified Surgical Technique for Combined Congenital External and Middle Ear Malformations
International Bulletin of Otorhinolaryngology, 2008
Hypothesis: There is a positive correlation between type and intensity of combined congenital ear malformations and results of surgical reconstruction. Background: Combined congenital malformations of external and middle ear pose a lot of surgical problems and demand adequate skill and planned, fractioned therapy. The goals of these operations are: limited number of operations with minimal complications, well cosmetic result, hearing improvement and easier use of hearing aid, removal of congenital cholesteatoma, preservation of labyrinth and facial nerve. Methods: The aim of this work is to present the experience in treating patients with combined malformations of external and middle ear. In the period between 1992 and 2001 seventeen patients with this type of congenital malformations were operated. Classical dilemma who operates first, plastic surgeon or otosurgeon is solved here by complete otosurgical treatment with reduced number of operations. Results: Totally 36 operation was performed, two reoperations were made in four patients, and also three operations in three patients for the reconstruction of deformity of auricle. In seven patients costal rib cartilage was used for auricular framework. Reoperations were done during the course of auricular reconstruction (14 operations), or because of meatal restenosis (five operations), or cartilage graft necrosis (one case). Atresia was adequately solved in 14 patients, while in three cases connective tissue meatal restenosis was found. The results of reconstruction of auricular malformation were aesthetically good in 13 patients and sufficient in four patients. Conclusion: We found positive correlation between external ear and middle ear deformity, and the results of reconstruction, as well. Two thirds of patients have hearing threshold level under 30 dB, while the threshold in other patients is 30-40 dB. Residual airbone gap amounting about 20 dB was universally present.
Results of Management of Patients with Congenital Developmental Defects of the External Ear
Central Asian Journal of Medical and Natural Science, 2023
Rehabilitation of patients with congenital defects is a challenge for a specialist of any profile. The key to success in this difficult matter is a multidisciplinary approach, a trusting relationship between doctor and patient, and the participation of loved ones. The need for information, support and often surgical intervention is high, some of the procedures required are very complex, and the results obtained are variable. However, to date there is no centralized national program for the rehabilitation of this group of patients and standards for providing them with medical care. This work is an attempt to generalize our experience of monitoring patients with congenital malformations of the external and middle ear, to determine the forms and timing of assistance provided, and to evaluate the effectiveness of treatment.
Congenital Anomalies in Otorhinolaryngology
A number of factors like genetic, intrauterine infection and maternal nutritional deficiencies are involved in complex development of brachial apparatus and cochlea formation. A clinician should examine the child thoroughly for other organ developmental anomalies.. The counseling and children management shouldbe considered hand by hand.