Congenital Anomaly of Cochlea: A Case Report (original) (raw)
Related papers
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
Congenital Malformation of the Inner Ear and Pediatric Cochlear Implantation
Otology & Neurotology, 2004
Objectives: To study the surgical aspects and performance outcome of cochlear implantation in children with malformed inner ears. Study Design: Clinical and audiometric evaluation in 13 patients. Methods: Patient data concerning surgery, postoperative follow-up, and pre-and postimplantation audiometry were obtained from the cochlear implant center's database and evaluated. A review of the literature has been included. Setting: Tertiary referral center. Patients: The patients had a variety of inner ear malformations and profound hearing loss. One patient with recurrent meningitis had a severe cochlear malformation (common cavity). Results: Major complications did not occur. In one patient with an abnormal position of the cochlea and concurring middle ear disease, it was difficult to find the scala tympani during surgery. A cerebrospinal fluid gusher was encountered in two patients and an aberrant facial nerve in another, which did not lead to any complications. The patients with mild cochlear malformation such as an incomplete partition demonstrated a good performance in speech perception tests. Even the child with the common cavity deformity had some open-set speech perception 1 year after implantation. Conclusions: Viewing the patients from this study and patients from a review of the literature concerning cochlear implantation in children with malformed inner ears including severe cochlear malformations, the occurrence of an aberrant facial nerve was 17%, which increases to 27% if one reviews the surgical findings in children with severe malformed cochleae such as a common cavity or a severe cochlear hypoplasia. In the latter patients, results in speech perception vary. Although the result of cochlear implantation may be promising, as in our patient with a common cavity, during preoperative counseling the child's parents must be informed that the result is uncertain.
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.
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.
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 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.
Pakistan Journal of Medical Sciences
Objective: To determine the prevalence and features of inner ear anomalies in children with congenital profound hearing loss who presented at our cochlear implant center based on imaging studies. Methods: This retrospective study reviewed charts of children with congenital SNHL, who presented to Department of Otolaryngology & Auditory Implant Centre, Capital Hospital Islamabad over a period of 2 years from 1st May 2017 to 30th April 2019. These included 481 cases of both genders aged between 1 to 12 years. After gathering demographic data, audiological data, computed tomography findings of the temporal bone were analyzed. Data was analyzed using SPSS 22. Results: The Inner Ear Malformations were identified in 48(10%) children including 28 (58.33%) males and 20 (41.67%) female. Most 20(41.67%) presented at >3-5 years of age followed by 19(39.58%) at 2-3 years. However, no significant association of gender (p=0.57, p=0.076) and age of presentation (p=0.344, p=0.697) for right and l...
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 .
Nature reviews. Disease primers, 2017
Congenital hearing loss (hearing loss that is present at birth) is one of the most prevalent chronic conditions in children. In the majority of developed countries, neonatal hearing screening programmes enable early detection; early intervention will prevent delays in speech and language development and has long-lasting beneficial effects on social and emotional development and quality of life. A diagnosis of hearing loss is usually followed by a search for an underlying aetiology. Congenital hearing loss might be attributed to environmental and prenatal factors, which prevail in low-income settings; congenital infections, particularly cytomegalovirus infection, are also a common risk factor for hearing loss. Genetic causes probably account for the majority of cases in developed countries; mutations can affect any component of the hearing pathway, in particular, inner ear homeostasis (endolymph production and maintenance) and mechano-electrical transduction (the conversion of a mech...