Computed tomography evaluation of the inner ear as a diagnostic, counselling and management strategy in patients with congenital sensorineural hearing impairment (original) (raw)
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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 .
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.
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
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...
Diagnostic Yield of High-Resolution Computed Tomography for Pediatric Sensorineural Hearing Loss
The Laryngoscope, 1999
In recent years, relatively subtle inner ear anomalies have become apparent using high-resolution computed tomography (CT). The purpose of this study was to determine the diagnostic yield of high-resolution CT for pediatric sensorineural hearing loss (HL) (SNHL). Methods: A review was performed on the records of all children ( < 18 y of age) who had undergone CT of the temporal bones over a 5-year period, since the introduction of current CT techniques. Results: Three hundred eighty-three studies were performed in 351 subjects. The indication for the CT was SNHL or mixed HL in 157 children. Forty-nine (31%) of these studies revealed significant inner ear findings. Large vestibular aqueducts (LVAs) were reported in 15%, commonly in association with cochlear modiolar deficiencies. Modiolar deficiencies (11%) and other cochlear dysplasias (12%) followed LVA in frequency. The incidence of inner ear dysplasia in children with perinatal or postnatal risk factors was only slightly lower than those without (22% vs. 32%, P > .05). The rate of dysplasias did not correlate with SNHL severity, pattern of HL, or type of HL (mixed vs. sensorineural). Conclusions: These findings suggest that radiographic imaging has a relatively high diagnostic yield in children with SNHL. These findings may be of value in counseling patients and guiding the management of their SNHL.
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
PARIPEX INDIAN JOURNAL OF RESEARCH, 2023
Objective: Hearing dysfunction is the most common sensory deficit in children. If hearing is lost, especially in children's, it may have a huge impact on life, interfering with the acquisition of spoken language and social development. It is therefore important to detect any abnormalities at an early age. Patients with complaint of inability to hear since birth attending ENT OPD, M.Y.H Hospital, Indore and patients referred to M.Y.H hospital from district hospitals with inability to hear since birth were evaluated clinically and audiometry was done. Patients with SNHL fulfilling the inclusion criteria were considered and their radiological evaluation was done. High resolution computed tomography scan (HRCT) of temporal bone were used in radiological evaluation of patients with congenital sensorineural. SUBJECTS AND METHODS: The study was conducted at a tertiary care centre Maharaja Yashwantrao Holkar (MYH) hospital and Mahatma Gandhi Memorial Medical College (MGMMC), Indore in the Department of otorhinolaryngology and head & neck surgery. Cases with hearing loss from 1 to 16 years of age were selected. A detailed medical history with primary focus on inability to hear was obtained with emphasis on previous hospital stays, developmental delay, maternal history, family history. Complete ENT examination was done. Our study included a total 30 children. All children were Results: between 1-16 years of age with sensorineural hearing loss. Majority 11(36.67%) of the participated in the study were between 5-8 years of age. Cochlear anomaly was found in 03(10%) patients. Cochlea was found normal in 27(90%) on both the ears, 03(10%) child had hypoplastic cochlea bilaterally, none were with aplastic cochlea. Vestibular anomaly was seen normal in 28(93.33%) patients on bilateral side. 02(6.67%) patients were present with dysplastic vestibule. SSC was found dysplastic/malformed in 04(13.33%) patients who had dysplastic/malformed semicircular canal. Conclusion: The selection of candidates for hearing aid trials and cochlear implantation requires consideration of a variety of clinical, audiological and radiological factors. Imaging is a fundamental part of the preoperative workup, it provides the surgeon with details which will lead him to a successful cochlear implantation with no or minimal complications. HRCT temporal bone provided us the information about the presence of various inner ear malformations including cochlea, vestibule, SCC, IAC. Mastoid, pneumatization, variations in vascular anatomy which included forward lying sigmoid sinus and high riding jugular bulb. All these findings are important for a detection of cause of SNHL in patients.
Indian Journal of Radiology and Imaging
External ear malformations are commonly associated with middle ear malformations due to their closely related embryogenesis. Inner ear anomalies are less consistently seen due to their independent development. Cross-sectional imaging has come to play a critical role in the workup of congenital deafness in the pediatric age group. To optimize speech and language development, early diagnosis and treatment are very crucial. Identification of these anomalies is additionally important when operative intervention is being planned because they may complicate or perhaps preclude the surgery.