Cochleovestibular Malformation Research Articles

Factors that influence performance in pediatric cochlear implant recipients with cochlear nerve deficiency

Objectives Children with cochlear nerve deficiency (CND) have wide variability in outcomes with cochlear implant (CI) use. The current study aims to report a large cohort of pediatric CI recipients with CND and to evaluate for factors that may predict improved performance. Methods The current study is a retrospective review of pediatric CI recipients with CND at a tertiary academic hospital. Variables including cochlear nerve status (hypoplasia vs aplasia), age at implantation, cochleovestibular malformation, bony cochlear nerve aperture, internal auditory canal aperture, and cognitive delay were evaluated for predictors of postoperative performance. A stepwise multinomial regression analysis was performed. Results Forty-seven CI recipients (54 ears) were included in the analysis. A majority (59%) showed auditory capabilities with their CI. Twenty percent of recipients achieved some level of open-set speech perception with their CI. The regression analysis identified cochlear nerve status and cognitive delay as predictors of performance. CI recipients with cochlear nerve hypoplasia had significantly improved performance compared to those with aplasia (p = 0.003). Recipients with cognitive delay had more limited benefit than those without cognitive delay (p = 0.033). Conclusions Children with CND can benefit from CI use, with outcomes spanning from non-use to development of spoken language. Predictive factors for improved performance include a lack of cognitive delay and cochlear hypoplasia rather than aplasia. These can be important considerations for parent counseling and decision making.

Sensorineural Hearing Loss and Cochleovestibular Malformations in Children in Côte d’Ivoire

Objective: to find the distribution of various malformations and to report the clinical features of commonly encountered IEMs in our practice. Methods: This was a retrospective cross-sectional study, over a period of 14 months (January 2019 to March 2020), in the ENT departments of University Hospitals and the Mother and Child Hospital in Bingerville. All patients, aged up 15 years with computed tomography and/or MRI available, who had a cochleovestibular malformation and sensorineural or predominantly sensorineural mixed hearing loss confirmed were included. Results: The prevalence of sensorineural hearing loss related to cochleovestibular malformations was 3.75% (6 cases out of 160 patients). Patients ranged in age from 2 to 14 years. The diagnosis was made before the age of 3.5 years in 5/6 cases. The sex ratio was 1. Language delay was noted in 5/6 cases. No contributing risk factors were identified. All patients had profound and bilateral hearing loss. Associated malformations were present in 2/6 cases. The most common abnormalities were semicircular canal hypoplasia, internal auditory canal abnormalities and cochlear malformations. There was an association between sensorineural hearing loss linked to malformation and the profound degree of hearing loss. Conclusion: Sensorineural deafness linked to cochleovestibular malformations in children appears to be uncommon. However, they are associated with a risk of profound sensorineural hearing loss. It is therefore important, in the face of any sensorineural hearing loss in the child, to systematically carry out an imaging assessment, even in our low income’s countries.

SMS classification of inner ear malformations: our experience with implantation in type II anomalies.

To study and analyse the radiological and surgical findings of 24 cochlear implantees with SMS type II cochleovestibular malformation and to compare their outcomes in terms of hearing and speech gains pre- and postoperatively. Retrospective analysis of cochlear implanted candidates over a period of 8year 6months from 3 institutions was undertaken and 24 patients suffering from prelingual profound congenital sensori-neural hearing loss along with the presence of SMS type II cochleovestibular malformation were studied. Preoperative radiology, surgical difficulties and complication, and postoperative hearing and speech outcomes up to a period of 2years, using IT-MAIS and SIR scores were noted. Statistical comparison pre- and postimplantation was done using Wilcoxon signed rank test and a p value of < 0.05 was considered statistically significant. In 24 cases, we encountered four cases of SMS type IIa malformation with a smaller modiolus, while the rest 20 were type SMS type IIb with partially defective modiolus. Intraoperatively, CSF leak was observed in 11 patients. Complete electrode insertion and good electrical response (NRT) was detected in all. No facial nerve anomaly was encountered. IT-MAIS and SIR scores increased from a preoperative mean of 5.6 and 1.16-34.56 and 3.88 after 2 years postsurgery, respectively and this difference was found to be statistically significant. Our experience with SMS Type II malformations show promising and motivating results with less chances of complications. Proper evaluation and surgical planning preoperatively can lead to an uneventful surgery with good outcomes. A simple and uniform classification system of these anomalies is a must for appropriate prognostication and right decision making.

Management of Meningitis Due to Cystic Cochleovestibular Malformation: a Stitch in Time.

Spontaneous CSF otorrhea alludes to those cases which have no known etiology like traumatic, iatrogenic, neoplastic or infectious. The presentation of the patient depends on the anatomical integrity of the eustachian tube as well as the tympanic membrane. Children with certain congenital inner ear malformations, including incomplete partition deformity, show a higher incidence of spontaneous CSF leaks and the resultant meningitis. Cystic vestibulocochlear deformity accounts for approximately 20% of inner ear malformations. In this case report, we discuss a child who presented with first episode of meningitis and with the help of thorough clinical otorhinolaryngological examination and radiology, was diagnosed with a congenital ear anomaly. With this paper, we stress upon the importance of keeping an open mind regarding the differential diagnosis of any condition, and the value of timely intervention.

New SMS Classification of Cochleovestibular Anomalies: Our Experience with 25 Cases of Type I Anomaly.

To study and analyse the radiological and surgical findings of 25 cochlear implantees with SMS type-I cochleovestibular malformation and to compare their outcomes in terms of hearing and speech gains pre- and post-operatively. Retrospective analysis of cochlear implanted candidates over a period of 8year from 3 institutions was undertaken and 25 patients suffering from pre-lingual profound congenital sensori-neural hearing loss along with presence of SMS type I cochleovestibular malformation were studied. Pre-operative radiology, surgical difficulties and complication, and post-operative hearing and speech outcomes upto a period of 2years, using IT-MAIS scores were noted. Statistical comparison pre- and post-implantation was done using Wilcoxon signed rank test and a p-value of < 0.05 was considered significant. In 25 cases, we encountered 2 posterior SCC dysplasia, 15 enlarged vestibular aqueducts (LVA), 2 dilated vestibules, 4 superior SCC dysplasia, 2 cases with both posterior and superior SCC dysplasia and of those 1 had a dilated vestibule also. Intra-operatively, CSF leak was observed in 10 patients. Complete electrode insertion and good electrical response (NRT) was detected in all. No facial nerve anomaly was encountered. IT-MAIS scores increased from a pre-operative mean of 4.32 to 34.56 two years post-operatively and this difference was found to be statistically significant. Our experience with SMS Type I malformations show promising and motivating results with less chances of complications, similar to implant candidates with normal anatomy. The outcomes in this group of patients are definitely not affected by any malformations in semicircular canals, vestibule or vestibular aqueduct.

Cochlear implantation in a child with cystic cochleovestibular malformation: a case report

Background: The inner ear and internal auditory canal (IAC) malformations account for 20% of congenital sensorineural hearing loss in children. The cystic cochleovestibular malformation is one of the congenital malformations in the cochlea, in which the cochlea is empty, unpartitioned, and cystic. The dimension of the cochlea is normal. This presents a significant challenge even to the most experienced clinicians because of difficulty in the surgery with facial nerve anomaly and gusher, choice and placement of electrode, and increased risk of meningitis after the procedure. This study aims to report the procedure of cochlear implantation in one case of cystic cochleovestibular (IP type I) malformation, which still became a challenge in the otology field.Case Presentation: We reported one case of 5 years old boy with bilateral cystic vestibular malformation who underwent cochlear implantation on the left ear. The patient was diagnosed with bilateral profound congenital sensorineural hearing loss. He already used hearing aids on both ears but gained limited improvements. Peri-operative perilymph gusher was happened and was stopped by plugging the cochleostomy hole using fascia. The patient showed good condition after the procedure. Although facial nerve lesion occurred after the operation, it was improved by giving steroids. There is no sign of cerebrospinal fluid leaks such as rhinorrhea, otorrhea, or meningitis.Conclusion: This report showed good outcomes following cochlear implantation in cystic cochleovestibular malformation.

FORM24 electrode array and perioperative cerebrospinal fluid leakage in cochlear implant recipients with cochleovestibular malformations

ABSTRACTBACKGROUND: Cochlear implant (CI) recipients with a cochleovestibular malformation (CVM) are at a higher risk of experiencing an intra-operative cerebrospinal fluid (CSF) gusher and, therefore are at greater risk of developing postoperative meningitis than are CI recipients with normal cochlear anatomy. To control CSF gushers, the FORM electrode array was developed.OBJECTIVES: To assess the ability of the FORM24 electrode array in managing intraoperative CSF gushers and preventing postoperative CSF leakage in a population of CI recipients.DESIGN: Retrospective.SETTING: Tertiary health care center.PATIENTS AND METHODS: All CIs in which a FORM24 was used between January 2014 and March 2018 were reviewed for demographic and safety results.MAIN OUTCOME MEASURES: Safety results were assessed as the intraoperative or postoperative presence of an episode of CSF leakage or meningitis.SAMPLE SIZE: 177 CI recipients.RESULTS: Thirty-six (20.3%) had a CVM and 141 had normal anatomy (79.7%). Of the 36 participants with a CVM, 20 (55.6%) experienced an intraoperative CSF gusher, all of which were resolved. No cases of postoperative leakage or meningitis were recorded after a mean follow-up time of 36 months.CONCLUSION: The FORM24 array is able to help surgeons stop intraoperative CSF gushers and prevent postoperative CSF leakage and meningitis in CI recipients with a CVM.LIMITATIONS: Further studies are needed.CONFLICT OF INTEREST: None.

Auditory Performance in Early Implanted Children with Cochleovestibular Malformation and Cochlear Nerve Deficiency.

This study aimed to report the auditory performance in children with cochleovestibular malformation (CVM)/cochlear nerve deficiency (CND) who were implanted early at the Universiti Kebangsaan Malaysia Medical Centre, using Categorical Auditory Performance (CAP)-II score and Speech Intelligibility Rating (SIR) scales, and to compare the outcome of their matched counterparts. A total of 14 children with CVM/CND with unilateral cochlear implant (CI) implanted before the age of 4 years old were matched and compared with 14 children with normal inner ear structures. Their improvement in auditory performance was evaluated twice using CAP-II score and SIR scales at 6-month intervals, with the baseline evaluation done at least 6 months after implantation. The average age of implantation was 31±8 and 33±7 months for the control group and the case (CVM/CND) group, respectively. Overall, there were no significant differences in outcome when comparing the entire cohort of case subjects and their matched control subjects in this study. However, the improvement in CAP-II scores and SIR scales among the case subjects in between the first and second evaluations was statistically significant (p=0.040 and p=0.034, respectively). With longer duration of CI usage, children with CVM/CND showed significant speech perception outcome evident by their SIR scales (p=0.011). Children with radiographically malformed inner ear structures who were implanted before the age of 4 years have comparable performance to their matched counterparts, evident by their similar improvement of CAP-II scores and SIR scales over time. Hence, this group of children benefited from cochlear implantation.

Application of the new SMS system of cochleovestibular anomalies: our experience with nine cases of type III anomaly.

To present and analyse the radiological findings, surgical findings, pre-implantation and post-implantation speech and hearing outcomes of nine children with SMS Type III cochleovestibular malformation. Retrospective case series of nine children with pre-lingual profound sensorineural hearing loss who underwent cochlear implantation (Jan 2012 to July 2019). These children had been classified as Type III malformation according to theSMS Classification of cochleovestibular anomalies. Facial nerve anomalies, CSF leaks and any other significant surgical finding were noted. Meaningful Auditory Integration Scale (MAIS) was used to report the child's pre-operative and two-year post-implantation auditory and speech abilities. Any significant improvement was assessed using the Wilcoxon signed rank test. P value < 0.05 was considered significant. Out of nine patients, five patients sustained CSF gushers, while three patients had mild CSF leak, which were plugged adequately. No facial nerve anomalies were encountered. Post-op course was uneventful for all nine patients. MAIS scores at a two-year follow-up showed significant statistical improvement (P < 0.05) when compared to pre-operative scores. Cochlear implant is the treatment of choice for children with type III cochleovestibular malformation. There are significant auditory and speech improvements expected. However, the surgeon should bear in mind the risk of CSF leak and subsequent meningitis.

New SMS classification of cochleovestibular malformation and its impact on decision-making.

To propose a new classification of inner-ear anomalies that is more clinically oriented and surgically relevant: the SMS (Sawai Man Singh) classification of cochleovestibular malformations. A retrospective multicentric study was conducted of 436 cochlear implantations carried out in 3 Indian tertiary care institutes. Patients with anomalous anatomy were included and classified, as per the new SMS classification, into cochleovestibular malformation types I, II, III and IV, based on cochlear morphology, modiolus and lamina cribrosa. There were 19, 23, 8 and 4 patients with cochleovestibular malformation types I, II, III and IV, respectively. Two-year post-operative Meaningful Auditory Integration Scale scores were statistically analysed. This new classification for inner-ear anomalies is a simpler, more practical, outcome-oriented classification that can be used to better plan the surgery. These merits make it a more uniform classification for recording results.

Contemporary surgical issues in paediatric cochlear implantation

Objective: To review the contemporary surgical issues in paediatric cochlear implantation (CI) based on published evidence. Design: Narrative literature review. Results: Surgical challenges in paediatric CI are discussed, with respect to post meningitic labyrinthitis ossificans; cochlear malformation; cochlear implantation in infants; auditory neuropathy and cochlear nerve deficiency; bilateral cochlear implantation; hearing preservation; otitis media; and device failure. Conclusion: Early CI is recommended if bacterial meningitis causes profound sensorineural hearing loss (SNHL). CI in cochleovestibular malformation requires pre-operative imaging to plan surgical technique, choice of electrode, and to anticipate complication. Children with congenital severe to profound SNHL should undergo early bilateral simultaneous implantation, preferably before 12 months of age, except those with auditory neuropathy spectrum disorder who should be implanted after one year. Soft surgical technique should be deployed in an attempt to preserve any residual hearing. Otitis media with effusion is not a contraindication to cochlear implantation, but active or recurrent acute otitis media requires resolution of infection with grommet insertion pre-operatively. Device failure in CI recipients requires a stepwise audiological, medical, radiological, and device integrity assessment to determine the need for reimplantation.

Effect of Cochleovestibular Malformation on Surgical and Auditory Outcomes in Pediatric Cochlear Implant Recipients: A Retrospective Study Using a Web-Based Database

Purpose Prior studies have suggested that cochleovestibular malformation (CM) may have an influence on surgical and auditory outcomes in pediatric cochlear implant (CI) recipients. The present stud...

A New Classification of Cochleovestibular Malformations and Implications for Predicting Speech Perception Ability after Cochlear Implantation

Objectives: The aims of this study were to introduce a new classification of cochleovestibular malformation (CVM) and to investigate how well this classification can predict speech perception ability after cochlear implantation in children with CVM. Methods: Fifty-nine children with CVM who had used a cochlear implant for more than 3 years were included. CVM was classified into 4 subtypes based on the morphology of the cochlea and the modiolus on temporal bone computed tomography (TBCT): normal cochlea and normal modiolus (type A, n = 16), malformed cochlea and partial modiolus (type B, n = 31), malformed cochlea and no modiolus (type C, n = 6), and no cochlea and no modiolus (type D, n = 6). Speech perception test scores were compared between the subtypes of CVM using analysis of covariance with post hoc Bonferroni test. Univariate and multivariate regression analyses were used to identify the significant predictors of the speech perception test scores. Results: The speech perception test scores after implantation were significantly better in children with CVM type A or type B than in children with CVM type C or type D. The test scores did not differ significantly between the implanted children with CVM type A or type B and those without CVM. In univariate regression analysis, the type of CVM was a significant predictor of the speech perception test scores in implanted children with CVM. Multivariate regression analysis revealed that the age at cochlear implantation, cochlear nerve size and preimplantation speech perception test scores were significant predictors of the postimplantation speech perception test scores. The chance of cochlear nerve deficiency increased progressively from CVM type A to type D. Conclusion: The new classification of CVM based on the morphology of the cochlea and the modiolus is simple and easy to use, and correlated well with postimplantation speech perception ability and cochlear nerve status. This simple classification of CVM using TBCT with cochlear nerve assessment by magnetic resonance imaging is helpful in the preoperative evaluation of children with CVM.

A stepwise algorithm for the management of cerebrospinal fluid gusher during cochlear implantation

Objectives The aim of this study was to assess the incidence of CSF gusher during cochlear implantation in children with and without congenital inner-ear malformation and to establish a simple stepwise algorithm for managing CSF gusher at the time of cochleostomy. Materials and methods A total of 54 congenitally deaf children were included in a retrospective study between January 2011 and December 2012. All cases underwent classical cochlear implantation surgeries via mastoidectomy and posterior tympanostomy approach. Results Nine patients developed gusher at the time of the cochleostomy. Among the nine cases, only one child did not show any preoperative radiologic evidence of any bony cochleovestibular malformation, whereas the remaining eight cases had different congenital inner-ear malformations with known risk for intraoperative gusher during surgery. Conclusion We concluded that the CSF gusher is a surgical difficulty or an intraoperative challenge rather than a bad prognostic determinate for the postoperative audiologic performance, and in cases of congenital cochleovestibular malformation that develop gusher, a high degree of congenital anomaly of the cochlea, and not the degree or the amount of gusher, is correlated to the poor patient performance. Finally, we were able to achieve a simple stepwise algorithm for the management of gusher during cochlear implantation.

Recurrent Meningitis in An Adult Secondary to An Inner Ear Malformation: Imaging Demonstration

Congenital labyrinthine dysplasia with a translabyrinthine cerebrospinal fluid (CSF) fistula may be an anatomic cause for recurrent meningitis. This condition is usually seen in children aged 5 to 10 years who present with sensorineural hearing loss (SNHL) and CSF discharge through the nose or ear, with or without recurrent meningitis. Multidetector-row computed tomography (MDCT) and high-resolution T2-weighted magnetic resonance imaging (MRI) of the petrous portion of the temporal bone can help to diagnose this abnormality. We report a case of translabyrinthine CSF fistula in an adult-a 30-year-old man-who presented with recurrent pneumococcal meningitis, a long history of a clear nasal discharge, and evidence of SNHL. MDCT and MRI of the temporal bone demonstrated a cystic-appearing cochleovestibular malformation (an incomplete partition type I) in the right inner ear. Imaging also showed an absence of the basal turn of the cochlea and the cribriform membrane at the lateral end of the right internal auditory canal, which was shorter and narrower than normal. Evidence of fluid in the right middle ear suggested a CSF fistula.

Cystic Cochleovestibular Malformation (Incomplete Partition Type 1)

A 5-year old female with bilateral profound hearing loss underwent computerized tomographic imaging of the temporal bone as part of the work-up to determine the etiology of her deafness, and to delineate middle and inner ear anatomy prior to cochlear implantation. The examination revealed an inner ear malformation which, based on the newest classification of cochleovestibular malformations by Sennaroglu and Saatci, is called an incomplete partition type I (IP-1) or cystic cochleovestibular malformation. This condition is characterized by (1) a cochlea that is lacking the entire modiolus and cribriform area, resulting in a cystic appearance, and (2) a large cystic vestibule.1 &nbsp; Temporal bone imaging is among the most useful examinations in the etiological investigation of idiopathic sensorineural hearing loss in children, with up to 30%2 of the imaging studies showing an abnormality. The detection of inner ear malformations is important, as some abnormalities are associated with an increased risk of meningitis or progressive hearing loss following head trauma.3 Likewise, the approach to cochlear implantation may be influenced by the type of malformation. In this particular patient, the use of a cochlear implant with a full-band electrode design may be more appropriate, as the location of the neural elements within the cystic cochlea is not definitely known.

Cystic Cochleovestibular Malformation (Incomplete Partition Type 1)

A 5-year old female with bilateral profound hearing loss underwent computerized tomographic imaging of the temporal bone as part of the work-up to determine the etiology of her deafness, and to delineate middle and inner ear anatomy prior to cochlear implantation. The examination revealed an inner ear malformation which, based on the newest classification of cochleovestibular malformations by Sennaroglu and Saatci, is called an incomplete partition type I (IP-1) or cystic cochleovestibular malformation. This condition is characterized by (1) a cochlea that is lacking the entire modiolus and cribriform area, resulting in a cystic appearance, and (2) a large cystic vestibule.1&#x0D; &#x0D; Temporal bone imaging is among the most useful examinations in the etiological investigation of idiopathic sensorineural hearing loss in children, with up to 30%2 of the imaging studies showing an abnormality. The detection of inner ear malformations is important, as some abnormalities are associated with an increased risk of meningitis or progressive hearing loss following head trauma.3 Likewise, the approach to cochlear implantation may be influenced by the type of malformation. In this particular patient, the use of a cochlear implant with a full-band electrode design may be more appropriate, as the location of the neural elements within the cystic cochlea is not definitely known.

Dysplasie cochléovestibulaire. À propos d’un cas

Cochleovestibular dysplasia or Mondini malformation is exceptional. Cochlear dysplasia is due to early cessation of the development of the inner ear during embryonic life. We report on the case of an infant who presented with perception deafness and repeated meningitis. CT allowed the diagnosis of Mondini malformation. We underline the importance of high-resolution CT in the diagnosis of Mondini malformation.

Intravestibular Lipoma

Lipomas constitute 0.1% of all intracranial tumors. 1 Very rarely they are located in the cerebellopontine angle (CPA) or the internal auditory canal, and even less frequently cerebellopontine angle (CPA) or the internal auditory canal, and even less frequently they have been described in an intravestibular location. 2-4 These lipomas should not be treated surgically because their adherence to nerves and surrounding brain structures often leads to neurological deficits when surgical removal of the lesion is attempted. 1 Therefore, it is important to distinguish inner ear and CPA lipomas from more common tumors in the cerebellopontine region, such as acoustic neuromas, which are often treated surgically. Thus, noninvasive diagnosis by radiological imaging is crucial. We report herein a case of intravestibular lipoma that is associated with CPA lipoma and cystic cochleovestibular malformation (incomplete partition type 1) of the inner ear. Diagnostic magnetic resonance imaging (MRI) and computed tomographic (CT) characteristics of lipomas are discussed in detail. Furthermore, this unique combination of intravestibular lipoma with cystic cochleovestibular malformation provides more understanding of the pathophysiologic characteristics of these rare tumors.

Surgical Results of Cochlear Implantation in Malformed Cochlea

To report the surgical aspects of cochlear implantation in malformed cochlea. Tertiary care center. Retrospective case review. Between November 1997 and October 2004, 20 patients with inner ear malformations were implanted in our department. The age range was between 2 and 37 years (average, 8.8 yr). The anomalies were classified according to Sennaroglu and Saatci classification. There were two patients with common cavity deformity, four cases of incomplete partition (IP) type I (cystic cochleovestibular malformation), four cases of IP type II (classical Mondini's deformity), nine patients with large vestibular aqueduct (LVA) syndrome, and one patient with X-linked deafness. Standard transmastoid facial recess approach was used in 17 patients (three patients with IP I, four patients with IP II, and nine patients with LVA syndrome). In the remaining patient with IP I, because of the dehiscent and anteriorly located facial nerve, the surgical approach had to be modified, and an anteroposterior approach was used. After elevating the tympanomeatal flap, the electrode was inserted through the ear canal and then transferred to the mastoid through a full-length cut produced in the ear canal. The flap then returned to its place. In the patients with common cavity deformity, the electrode was inserted by the transmastoid labyrinthotomy approach. Facial nerve had an abnormal course in four patients, but no patient had facial weakness postoperatively. Cerebrospinal fluid gusher was encountered in four patients, whereas oozing was present in five patients. It seems that a slightly larger cochleostomy may reduce postoperative rhinorrhea. The patient with common cavity deformity showed abnormal vestibular stimulation which decreased and was totally abolished during a 3-month period. Based on these findings, cochlear implantation is surgically feasible in patients with common cavity, IP types I and II, and LVA. The surgeon should be ready to make modifications in the surgical approach because of the abnormal course of the facial nerve and be ready to produce special precautions to cerebrospinal fluid gusher.