Common Cavity Malformation Research Articles

A Case Report of Cochlear Malformations: A Rare Case of Unilateral Type I Incomplete Partition with Cochlear Nerve Aplasia and Contralateral Common Cavity Malformation with Normal Vestibulocochlear Nerve

A Case Report of Cochlear Malformations: A Rare Case of Unilateral Type I Incomplete Partition with Cochlear Nerve Aplasia and Contralateral Common Cavity Malformation with Normal Vestibulocochlear Nerve

Orthodontic treatment needs: parents and child perception

Background: Malocclusion is one of the most common oral cavity malformations. It has a variety of effects on dental health, function, aesthetics, and psychosocial condition. The suggested treatment for such a condition is orthodontic treatment. This study aims to assess the perceptions of orthodontic treatment needs of parents and their children with relation to their perception in Riyadh, Saudi Arabia.Methods: This is a cross-sectional study that was conducted to study the perception of children and their parents about the need for orthodontic treatment. The data were collected at the pediatric clinics of Riyadh Elm university in Riyadh, Saudi Arabia, and included 379 parents and their school children aged 4-12 years. An interviewed questionnaire was used to collect the data from the children and their parents separately.Results: The study found that the perception towards the need for orthodontic treatment of children and their parents was 44.6%, and 34.8% respectively. Also, a statistically significant difference was shown between the perception of children and their parents (p>0.05). The main factors affecting the perception was age, as the desire to have orthodontic treatment increased among children above age of 10 years. Other factors including satisfaction about chewing, teeth appearance, and social media altered the perception for orthodontic treatment need.Conclusions: These findings could be essential for orthodontic treatment planning and increasing patient compliance. The difference between children and parents’ perceptions can affect drive toward initial orthodontic visit.

Cerebrospinal fluid gusher in cochlear implant and its associated factors

Introduction: Cochlear implants (CI) is considered a safe procedure with overall complication rate of 12.5% amongst adult and pediatric population. Cerebrospinal fluid (CSF) gusher is regarded as a common CI intraoperative complication.Objective: In this study, we determined the association between probable associated factors and occurrence of intraoperative CSF gusher.Method: In a retrospective survey, 394 patients with severe to profound sensorineural hearing loss who underwent primary cochlear implantation surgery were evaluated. Patients with incomplete electronic or manual file and those with revision, explantation or reimplantation surgery were excluded.Result: three hundred and ninety-four CI patients with a mean age of 8.74 ± 12.21 years were reviewed 49.62% of them were female. CSF gusher developed in 22 patients (5.58%). Patients with CSF gusher had more structural abnormalities in their CT scans’ report. Common cavity malformation and Mondini dysplasia were the most common abnormal reports amongst those with intra-operative CSF gusher.Conclusion: In conclusion, cochlea structural abnormalities affect the incidence of CSF gusher in CI recipients. Common cavity malformation and Mondini dysplasia were associated with an increased incidence of CSF gusher. We insist on more precise pre-operative imaging of those with abnormal cochlea structures to provide the needed management.

Application of “banana cochleostomy” and looped electrode insertion for cochlear implantation in children with common cavity malformation and cystic forms of cochlear hypoplasia

ObjectivesPatients with profound hearing loss due to inner ear malformations may benefit from cochlear implantation; however, the surgery may present a substantial problem for the cochlear implant surgeon due to anatomical variations.The authors describe a new surgical and technical advancement for implantation in patients with small inner ear cavities that make the surgery easier and safer. On the basis of experience involving five consecutive surgeries performed in four patients with inner ear malformations, we present the advantages and application possibilities of the technique. MethodsThe technique does not change the surgical approach in general; however, modification of the cochleostomy shape and looping of the cochlear implant electrode enables safe advancement of the electrode with optimal positioning in the cavity. Additionally, these modifications protect against the insertion of the electrode into the internal auditory canal minimizing the risk of gushing and extracochlear stimulation. ResultsThe present technique has been used in five cases of cystic implantable inner ear spaces in three independent institutions by different surgeons. It has proven to be a reliable, relatively easy and safe procedure performed with very good anatomic and initially functional effects (positive intraoperative neural response telemetry measurements). ConclusionsWe hope that utilization of the “banana cochleostomy” and insertion of the looped cochlear implant electrode in the implantable cystic spaces of children with malformed inner ears will facilitate and simplify the surgical technique in this difficult procedure and additionally, in revision surgical cases. To our knowledge, the looped insertion and banana-shaped cochleostomy have not been reported previously.

Evaluation of embryological sequences of ear anomalies and its radiological relevance

Aim: To correlate the sequence of embryological development of ear with radiological imaging. Materials and Methods: The study enrolled 23 patients of age group 11 months to 27 years with malformed external ear/microtia and hearing loss. The children with postoperative changes, acute hearing loss (such as infection, trauma) were excluded from the study. We used high-resolution computed tomography, with axial and coronal sections to examine the temporal bones of patients. Results: Of the 23 cases, 12 cases had external ear anomaly and 11 cases had cochlear anomaly. In patients with the external ear anomaly, seven cases had associated middle ear malformations, one patient had associated middle and inner ear anomaly, one had anomalous course of facial nerve, and three cases had isolated external ear anomalies. In patients with inner ear anomalies, one patient had complete labyrinthine aplasia, one had cochlear dysplasia with incomplete cochlear turns, four had common cavity malformations, one had cystic featureless cochlea with dilated and cystic vestibule, two patients had small cochlea with the middle and apical turns coalescing to form a cystic apex, one patient had small rudimentary cochlea, and the other one had dilated vestibule and enlarged endolymphatic duct and sac with cochlear dysmorphism. Conclusion: Most of the children with malformed pinna had external auditory canal atresia with associated middle ear anomalies. Though the inner ear development is independent of external and middle ear development, we insist on the fact that insult during the 1 st month of embryogenesis can result in associated abnormalities involving external, middle, and inner ear. Developmental arrest at various stages of inner ear development results in various types of cochlear anomalies with associated vestibular, semicircular canal abnormalities, and rarely associated with middle and external ear anomalies. Hence, clear knowledge about embryology will help to guide the management.

Cochlear implantation in 21 patients with common cavity malformation

Conclusion: The facial recess approach is preferred in common cavity (CC) malformation with an incomplete basal turn, and the transmastoid single-slit labyrinthotomy approach in classic CC malformation. Patients with CC benefit from cochlear implantation (CI) over time, but the audiological and speech development is poorer than in cases with normal cochleas. Objectives: To discuss the surgical aspects and performance of CI in 21 patients with CC malformation. Methods: Twenty-one CC malformations were classified into 2 types: classic CC malformation and CC malformation with an incomplete basal turn. Twenty-one patients without inner ear malformation were set as the control group. Thus, data for 42 patients were analyzed. Results: The facial recess approach was used in 3 patients with CC malformation with an incomplete basal turn, and the transmastoid single-slit labyrinthotomy approach in 18 patients with classic CC malformation. After follow-up for 36 months, the average free-field hearing threshold was higher, and the scores for the CAP, SIR, IT-MAIS, and closed-set/open-set auditory speech perception tests were lower than in the control group (p < 0.05).

Recurrent Pneumococcal Meningitis in a Child With Congenital Common Cavity Malformation of the Inner Ear

Recurrent Pneumococcal Meningitis in a Child With Congenital Common Cavity Malformation of the Inner Ear

Facial canal bifurcation with inner ear anomalies in a case of Klippel–Feil syndrome: A case report

We present a case of Klippel–Feil syndrome in a 2-year-old Chinese girl who presented with global developmental delay and bilateral profound hearing loss. High resolution computed tomography (HRCT) of temporal bones showed bilateral common cavity malformations with hypoplastic internal auditory canals and anomalous labyrinthine segments of both facial canals. The labyrinthine segment of the right facial canal was duplicated. Her brain magnetic resonance imaging (MRI) showed aplasia of both vestibulocochlear nerves, while the cisternal and intracanalicular segments of both facial nerves were of normal caliber. These congenital anomalies, coupled with global developmental delay, precluded her candidacy for cochlear implantation. This case report is aimed at highlighting the association of inner ear malformations and in particular facial canal anomalies in Klippel–Feil syndrome.

Auditory maturity and hearing performance in inner ear malformations: a histological and electrical stimulation approach

The objective of this study was to assess the auditory performance of the neural structures in response to controlled electrical stimulation period. A prospective cohort study focused on the intracochlear electrical stimulation parameters and hearing performance of patients suffering different cochlear malformations who were treated by cochlear implants constituted the study design. The study sample constituted 16 patients, suffering profound prelingual hearing impairment, diagnosed on the basis of radiological criteria as having an inner ear malformation, and who underwent cochlear implantation and were followed for 24 months. Patients with common cavities, characterized by fewer nerve structures involved, less epithelial penetration, and deficient cochlear tonotopy distribution showed have higher thresholds and electrical charges than patients with cochlear hypoplasia, who in turn have higher thresholds than patients with minor malformations (p < 0.05). Furthermore, word perception was severely compromised in patients with a common cavity malformation and was also poor in patients with cochlear hypoplasia, who were unable to discriminate more than 50% of the words and relied on visual cues as a necessary aid to communication. Better results were reached by minor malformed inner ears. To conclude, the number of nerve structures involved, epithelial penetration and deficient cochlear tonotopy are responsible of inner ear functionality.

Cochlear Implantation of the Common Cavity Malformation may be Performed During or Before CSF Leak Repair

Cochlear Implantation of the Common Cavity Malformation may be Performed During or Before CSF Leak Repair

Radiographic diagnosis of trans-stapedial cerebrospinal fluid fistula

Objective To report the high-resolution computed tomography and magnetic resonance imaging (MRI) findings of a bulging oval window in children with recurrent meningitis and congenital cerebrospinal fluid fistula. Study Design Case series. Setting Academic medical center children's hospital. Subjects and Methods A series of eight ears in four children with profound, bilateral sensorineural hearing loss and perilymphatic hydrops were evaluated. Two patients presented with recurrent meningitis. All children were assessed with high-resolution computed tomography, and two children also underwent MRI. Results Seven of eight ears had a common cavity malformation. The vestibular compartment showed severe dysplasia (n = 5), moderate dysplasia (n = 1), or a single semicircular canal (n = 2). The lamina cribrosa was clearly absent in four of eight ears. Its presence was difficult to assess in ears with small internal auditory canals. A bulging oval window, present in six of eight ears, was defined as a fluid density on high-resolution computed tomography or a hyperintense mass demonstrated by T2-weighted MRI protruding from the vestibule into the middle ear cavity. When present, this herniation of a fluid-filled sac could be seen on both MRI and computed tomography. This imaging finding was surgically confirmed in two patients. Conclusion The bulging oval window, which represents a fluid-filled sac, can be identified by both high-resolution computed tomography and MRI. Communication between the middle ear and the subarachnoid space through the inner ear is an important etiology for recurrent meningitis in children with sensorineural hearing loss.

Real-Time Intraoperative Computed Tomography to Assist Cochlear Implant Placement in the Malformed Inner Ear

Cochlear implantation is increasingly being performed in children with inner ear malformations. In severe cochleovestibular anomalies, such as severe partitioning defects and common cavity dysplasia, positioning of the electrode array can be hazardous, with inadvertent placement into the internal auditory canal (IAC) or carotid canal being well known. We describe a case in which real-time intraoperative computed tomographic scanning was used to help achieve proper electrode positioning in a child with a severe malformation. Child with common cavity malformations undergoing cochlear implantation. Intraoperative computed tomography used during implantation procedure. Use of technique in determining electrode position. A 10-year-old patient with bilateral common cavity malformations presented with declining performance in a functioning implant placed 7 years earlier. The family elected implantation of the contralateral ear. Via a posterior labyrinthotomy approach, a straight array was placed into the common cavity. Intraoperative computed tomographic scanning was immediately performed on the operating room table, showing that the array was in the IAC. A second attempt with a different insertion angle also resulted in IAC placement. In a third attempt, the electrode was advanced as a loop, grasping the tip through an adjacent second labyrinthotomy. Computed tomography confirmed good position against the outer wall of the cavity. Real-time intraoperative computed tomography is a new technology with many potential applications in surgery. In our patient, it allowed rapid and accurate determination of electrode position and helped achieve ideal placement in a severely malformed inner ear.

Audiological performance after cochlear implantation: a 2-year follow-up in children with inner ear malformations

Conclusions: Open-set speech perception in children with an inner ear malformation is equal to that of other congenitally deaf children after an average of 2 years follow-up. Objective: To analyze audiological performance after cochlear implantation in a sample of children with radiographically detectable malformations of the inner ear compared to performance in prelingually deafened children at large. Materials and methods: Nine children with osseous inner ear malformations were compared to 22 congenitally deaf children, all of whom underwent cochlear implantation. All subjects were tested on their electrical evoked compound action potential. Speech perception tests were performed using the monosyllabic trochee polysyllabic test without visual support and the open-set monosyllabic wordlist. Results: In all, 20% of the congenitally deaf children in our center study have inner ear abnormalities. Inner ear malformations were limited to incomplete partition of the cochlea; none of the subjects had common cavity malformations. Electrical compound action potentials were successfully recorded in both groups intraoperatively. Speech perception tests on open-set speech yielded an average of 48.8% (SD 21.2%) in the group of children with inner ear malformations vs 54.5% (SD 21.1%) in congenitally deaf children. In four of nine cases with an inner ear malformation we encountered a minor CSF leak.

Endoscopically guided placement of prefabricated cochlear implant electrodes in a common cavity malformation

To devise a safe and effective method of optimal customized electrode placement in the common cavity of children with cochleovestibular malformations. Specialized electrodes were manufactured on the basis of three-dimensional data obtained from the high resolution computed tomography (HRCT) scans of the temporal bones of these two children. Electrode positioning was achieved with direct endoscopic view of the cavity utilizing a three-hole common cavity technique. Optimal electrode positioning in apposition to the medial neuroepithelium in the common cavity was verified visually intraoperatively. Postoperatively, minimal stable electrical current levels were found to be required. Custom-designed electrodes have the potential to offer improved results in children with common cavity malformations. Intraoperative direct positioning may further improve these results.

Inner Ear Anomalies Causing Congenital Sensorineural Hearing Loss: CT and MR Imaging Findings

Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cases had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss.

Intraoperative evoked potentials monitoring for cochlear implantation in common cavities

Objectives: To determine the efficacy of intraoperative evoked compound action potential (ECAP) monitoring for optimal electrode placement and postoperative programming in cochlear implantation for patients with common cavity malformations. Methods: Retrospective case review of 1 child with a common cavity malformation undergoing revision cochlear implantation with a Clarion HiRes 90K™ implant. Outcome measures included neural response amplitude curves from intraoperative ECAP for each of 16 independent output circuits. Postoperative ECAP aided in mapping configuration to adjust threshold (T) and most comfortable loudness (M) levels across hearing frequencies. Results: Intraoperative ECAP confirmed proximity of the electrode to the neural response elements in this child with a common cavity defect by demonstrating expected neural response amplitudes across 10 of the 16 independent output circuits. No intraoperative repositioning of the electrode was required. Postoperative computed tomography imaging confirmed the location of electrodes with present ECAP responses to be along the medial most wall of the cavity. Postoperative ECAP aided the adjustment of mapping configuration to optimize threshold levels across frequencies for this child. Conclusion: Intraoperative ECAP can aid placement of a cochlear electrode in patients with common cavity malformations, particularly when the location of the neural response elements within the malformed otic capsule are unknown. ECAP can also assist in optimizing the postoperative mapping configurations in these patients.

A new classification for cochleovestibular malformations.

The report proposes a new classification system for inner ear malformations, based on radiological features of inner ear malformations reviewed in 23 patients. The investigation took the form of a retrospective review of computerized tomography findings relating to the temporal bone in 23 patients (13 male and 10 female patients) with inner ear malformations. The subjects were patients with profound bilateral sensorineural hearing loss who had all had high-resolution computed tomography (CT) with contiguous 1-mm-thick images obtained through the petrous bone in axial sections. The CT results were reviewed for malformations of bony otic capsule under the following subgroups: cochlear, vestibular, semicircular canal, internal auditory canal (IAC), and vestibular and cochlear aqueduct malformations. Cochlear malformations were classified as Michel deformity, common cavity deformity, cochlear aplasia, hypoplastic cochlea, incomplete partition types I (IP-I) and II (IP-II) (Mondini deformity). Incomplete partition type I (cystic cochleovestibular malformation) is defined as a malformation in which the cochlea lacks the entire modiolus and cribriform area, resulting in a cystic appearance, and there is an accompanying large cystic vestibule. In IP-II (the Mondini deformity), there is a cochlea consisting of 1.5 turns (in which the middle and apical turns coalesce to form a cystic apex) accompanied by a dilated vestibule and enlarged vestibular aqueduct. Four patients demonstrated anomalies involving only one inner ear component. All the remaining patients had diseases or conditions affecting more than one inner ear component. Eight ears had IP-I, and 10 patients had IP-II. Ears with IP-I had large cystic vestibules, whereas the amount of dilation was minimal in patients with IP-II. The majority of the semicircular canals (67%) were normal. Semicircular canal aplasia accompanied cases of Michel deformity, cochlear hypoplasia, and common cavity. In 14 ears, the IAC had a defective fundus at the lateral end. In two ears the IAC was absent. In all seven cases of common cavity malformations, there was a bony defect at the lateral end of the IAC. In five of them the IAC was enlarged, whereas in two the IAC was narrow. All patients with IP-I had an enlarged IAC, whereas in patients with type II disease, four had a normal IAC and 10 had an enlarged IAC. All cases of IP-II had an enlarged vestibular aqueduct, whereas this finding was not present in any of the cases of IP-I. In all cases, the vestibular aqueduct findings were symmetrical on both sides (simultaneously normal or enlarged). No patient demonstrated enlargement or any other abnormalities involving the cochlear aqueduct. Radiological findings of congenital malformations in the present study suggested two different types of incomplete partition. Cystic cochleovestibular malformation (IP-I) and the classic Mondini deformity (IP-II). The type I malformation is less differentiated than the type II malformation. Classic Mondini deformity has three components (a cystic apex, dilated vestibule, and large vestibular aqueduct), whereas type I malformation has an empty, cystic cochlea and vestibule without an enlarged vestibular aqueduct. Mondini deformity represents a later malformation, so the amount of dysplasia is much less than in type II. Therefore, it is more accurate and useful for clinical purposes to classify these malformations (in descending order of severity) as follows: Michel deformity, cochlear aplasia, common cavity, IP-I (cystic cochleovestibular malformation), cochlear hypoplasia, and IP-II (Mondini deformity). Only in this way can these complex malformations be grouped precisely and the results of cochlear implantation compared.