Correlation of cochlear aperture stenosis with cochlear nerve deficiency in congenital unilateral hearing loss and prognostic relevance for cochlear implantation (original) (raw)
Related papers
Cochlear Implantation in Children With Congenital Unilateral Deafness: A Case Series
Otology & Neurotology, 2017
Objectives: Cochlear implant is regarded as a treatment option for hearing rehabilitation of adults with unilateral sensorineural hearing loss. A clear benefit has been experienced in regard to speech comprehension in noise, localization, and quality of life. The aim of this study was to investigate the benefit of cochlear implantation for children with congenital unilateral hearing loss. Study Design: Retrospective case series. Setting: Tertiary referral center; cochlear implant program. Patients: Ten children with congenital unilateral hearing loss. Intervention: After extensive consultation with the families and intensive counseling, the children received a cochlear implant. Main Outcome Measures: Categories of auditory performance, speech discrimination in open set, subjective assessment by Speech, Spatial and Qualities scale questionnaire. Conclusion: Eight of ten children use their cochlear implant consistently on a daily basis. Two children who were equipped with an implanted device at a later age tend to nonuse of the device. The evaluation of binaural hearing in small children is still difficult and methods have to be developed to allow objective assessment.
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 .
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.
Cochlear implantation in children with congenital inner ear malformations
The …, 2004
To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations. Study Design: Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002. Methods: Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the temporal bone were the subjects of review. Results of HRCT findings, intraoperative findings, postoperative complications, and objective measures of both closed-and open-set testing of speech perception were analyzed. Results: Patients with the constellation of an incompletely partitioned (IP) cochlea, enlarged vestibular aqueduct (EVA), and a dilated vestibule (i.e., Mondini's malformation) as well as those with an isolated EVA or partial semicircular canal aplasia have relatively good levels of speech perception. Patients with total semicircular canal aplasia, isolated IP, cochlear hypoplasia, or common cavity demonstrated lower levels of performance. Poor performance may be related to associated developmental delays rather than labyrinthine anatomy alone. Complications of surgery were relatively limited. Conclusions: Cochlear implantation can be successfully performed in children with inner ear malformations. These children and their parents can expect significant auditory benefits from this intervention. The various types of inner ear malformations may have quite different prognoses for good auditory performance.
2013
Purpose: Prior studies have associated gross inner ear abnormalities with pediatric sensorineural hearing loss (SNHL) using computed tomography (CT). No studies to date have specifically investigated morphologic inner ear abnormalities involving the contralateral unaffected ear in patients with unilateral SNHL. The purpose of this study is to evaluate contralateral inner ear structures of subjects with unilateral SNHL but no grossly abnormal findings on CT. Materials and methods: IRB-approved retrospective analysis of pediatric temporal bone CT scans. 97 temporal bone CT scans, previously interpreted as ''normal'' based upon previously accepted guidelines by board certified neuroradiologists, were assessed using 12 measurements of the semicircular canals, cochlea and vestibule. The control-group consisted of 72 ''normal'' temporal bone CTs with underlying SNHL in the subject excluded. The study-group consisted of 25 normal-hearing contralateral temporal bones in subjects with unilateral SNHL. Multivariate analysis of covariance (MANCOVA) was then conducted to evaluate for differences between the study and control group. Results: Cochlea basal turn lumen width was significantly greater in magnitude and central lucency of the lateral semicircular canal bony island was significantly lower in density for audiometrically normal ears of subjects with unilateral SNHL compared to controls. Conclusion: Abnormalities of the inner ear were present in the contralateral audiometrically normal ears of subjects with unilateral SNHL. These data suggest that patients with unilateral SNHL may have a more pervasive disease process that results in abnormalities of both ears. The findings of a cochlea basal turn lumen width disparity >5% from ''normal'' and/or a lateral semicircular canal bony island central lucency disparity of >5% from ''normal'' may indicate inherent risk to the contralateral unaffected ear in pediatric patients with unilateral sensorineural hearing loss. ß
2015
Introduction: The cochlear implantation in patients with inner ear malformation has always been a challenge even to the most experienced clinicians. We present the case of a child of 8 years old with profound bilateral sensorineural hearing loss and the absence of language development with indication of cochlear implantation as single solution for hearing and speech rehabilitation. Methods: The audiological assessment indicates the cochlear implantation. Preoperative CT scan revealed a bilateral inner ear malformation (cochlear common cavity deformity on the right side, cochlear aplasia on the left ear and bilateral vestibular malformation). Brain magnetic resonance showed the presence of auditory nerve only to the right side. The girl was implanted on the right ear in the common cavity with an Advanced Bionics device, HiRes90K with HiFocus1j electrode. Results: The insertion of the portelectrode inside the malformed inner ear was confirmed by X-ray. Audiological evaluation had show...
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
Single-Sided Deafness and cochlear implantation in congenital and acquired hearing loss in children
Clinical otolaryngology : official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery, 2018
While CI provision is a well-established and beneficial therapy in an increasing number of countries for adults with acquired SSD, there is less experience with this therapeutic option in children OBJECTIVE OF REVIEW: determine the audiological and clinical results of cochlear implantation in children below the age of 12 years old with congenital and acquired single-sided deafness. Observational, descriptive, transversal study, in children <12 implanted for congenital or acquired SSD. Speech reception thresholds, Cortical responses, Auditory Lateralization Test and SSQ questionnaire. All the children with congenital SSD showed positive cortical responses. Positive results were obtained in the Auditory Lateralization Test for the following modalities: 0º, 45º and 90º. With respect to the Speech Test, the children with acquired SSD showed the following results: 92% and 100% in recognition and 48% and 68% (Azimuth modalities), Signal CI side 52% and 68% and Signal normal hearing sid...