Abnormal Facial Nerve Research Articles

Close relationship with the glandular capsule: a highly sensitive diagnostic indicator of major salivary gland metastatic malignancies in ultrasound.

To investigate the ultrasound (US) characteristics of metastatic malignancies (MM) in the major salivary glands and to assess the diagnostic value of the close relationship with the glandular capsule in identifying MM. From January 2016 and April 2022, 122 patients with major salivary gland malignancies, including 20 patients with MM and 102 patients with primary malignancies (PM) confirmed by histopathological examination, were enrolled in this study. Their clinicopathologic and US data were recorded and analysed. The diagnostic performance of the close relationship with the glandular capsule for differentiating MM from PM was analysed. The mean age of MM were older than that of PM (59.50 ± 14.57 vs. 49.96 ± 15.73, P = .013). Compared with PM patients, MM were associated with a higher prevalence of local pain symptoms (P = .007) and abnormal facial nerve function (P < .001). MM were also more frequently characterized by unclear borders, rough margins, irregular shapes, heterogeneous internal echos, absence of cystic areas, presence of calcifications, close relationship with the glandular capsule, and US-reported positive cervical lymph nodes (all P < .05). The close relationship with the glandular capsule showed to be a good indicator in distinguishing between MM and PM, with an area under the receiver operating characteristic curve of 0.863, a sensitivity of 100%, a specificity of 72.5%, and an accuracy of 92.2%. Positive and negative predictive were calculated at 41.7% and 100%, respectively. The US finding of a close relationship with the glandular capsule is a highly sensitive diagnostic indicator for MM. Following this finding, US-guided needle biopsy should be recommended to further confirm the diagnosis.

Cochlear Implantation in Children with Congenital Inner Ear Malformations

AbstractBackground: Cochlear Implantation has proven beneficial in patients with profound congenital hearing impairments. Performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute as congenital deaf ears may have anatomical malformations and difficulties during operation, it is important to focus on problems and complications that may face the surgeon who is performing this operation.Objective: Evaluate the problems and surgical difficulties encountered in patients with congenital inner ear malformation, who underwent cochlear implantation.Patients and methods: A series of 21 pediatric cochlear implant (CI) patients with known inner ear malformations determined on computed tomography (CT) of the temporal bone who underwent cochlear implantation in the department of otolaryngology in Hospital Martyr Ghazi Al-Hariri and AL-Yarmouk Teaching Hospital, Baghdad, Iraq, during the period from January 2018 to January 2021 were the subjects of the study to determine intraoperative adverse effects of these anomalies regarding electrode array insertion depths, predisposition to cerebrospinal fluid (CSF) leak through the cochleostomy, and associated facial nerve anomalies that can create access problems for scala tympani.Results: The most common inner ear anomalies were wide vestibular aqueduct (15) patients with percentage of (71.4%), incomplete partitions (5) patients with percentage of (23.8%), and common cavity (1) patient with percentage of (4.8%). CSF leak, the most common was CSF ooze (11) patients with percentage of (52.4%), CSF gusher seen in (6) patients (28.6%), and no CSF leak in (4) patients (19%). Facial nerve anomalies, only one case has abnormal position (more superficial and not injured) with a percentage of (4.8%), while in 20 patients (95.2%) no anomaly was detected. Electrode insertion depth, full insertion was achieved in (17) patients with percentage of (81%), while incomplete insertion was occurred in (4) patients (19%).Conclusion: Cochlear implant can be successfully and safely done in patients with selected congenital inner ear anomalies. Surgery can be challenging in certain cases of gusher and facial nerve abnormalities, but proper radiology and good surgical technique will help avoid complications. Keywords: Cochlear Implantation, Congenital inner ear malformation, facial nerve anomalies.

Patterns of facial and blink reflex abnormalities in type 2 diabetes mellitus patients with short disease duration: a clue to subclinical cranial neuropathy

Abstract Background Cranial neuropathies occur in 3 to 14% of diabetic patients. Motor conduction study of the facial nerve and blink reflex study are electrophysiologic techniques used to assess the facio-trigeminal pathway in diabetic patients. The patterns of facial and blink reflex abnormalities are inconsistent among studies. This study aimed to assess the subclinical facial nerve and blink reflex abnormalities patterns in short-duration type 2 diabetes mellitus patients. This cross-sectional study included 30 type 2 diabetic patients with disease duration ≤ 5 years. We included only patients with the Toronto clinical neuropathy score ≤ 5. We enrolled 30 age- and sex-matched healthy subjects as a control group. We performed facial nerve motor conduction and blink reflex studies. Patients with prior history of cranial nerve lesions, stroke, or any other disease-causing polyneuropathy or drug-induced neuropathy were excluded from the study. Results Thirty diabetic patients were included, 20 females (66.7%) and ten males (33.3%). Their mean age was 52.63 ± 8.94 years. None of the patients had clinical evidence of neuropathy. There were significant differences between patients and controls in the distal latencies and amplitudes of facial nerve compound muscle action potentials and contralateral R2 late response latencies of the blink reflex. We detected subclinical cranial abnormalities in 6 diabetic patients (20%). One of them (3.3%) had facial nerve conduction abnormalities, four of them (13.4%) had blink reflex abnormalities, and one of them (3.3%) had both facial nerve and blink reflex abnormalities. Conclusion Subclinical cranial neuropathy can occur in short-duration type 2 diabetes mellitus patients. We detected different blink reflex patterns and facial conduction study abnormalities. We recommend blink reflex and facial nerve conduction studies as simple tests for the early evaluation of neurological subclinical affection in patients with short disease duration of T2DM as they may appear in the absence of peripheral neuropathy.

Facial Nerve Anomalies as an Obscure Co-occurrence With External Ear Malformations: A Case Report

This study reports an infant with a rare triad of congenital facial palsy, bilateral aural atresia, and middle ear malformations. We highlight the audiological test battery in detail that led to identifying this obscure co-occurrence in a 6-month-old infant. The challenges associated with identifying such rare conditions, especially in infants, can be overcome only by incorporating a meticulous and vigilant approach. The infant was subjected to a series of subjective and objective audiological evaluations, through which rare asymmetric facial grimaces were vigilantly observed. This observation warranted radiologic investigation, which confirmed the suspicion that the anomaly may not be restricted to auditory structures alone. As facial nerve anomalies were confirmed, diversified recommendations, including speech, language, and swallow evaluation, were made apart from the auditory management. Hence in cases of external ear anomalies, although rare, it is essential to rule out facial nerve abnormalities as they can be a concealed problem.

Association of Ear Anomalies and Hearing Loss Among Children With 22q11.2 Deletion Syndrome.

To identify inner and middle ear anomalies in children with 22q11.2 deletion syndrome (22q11DS) and determine associations with hearing thresholds. Retrospective study. Two tertiary care academic centers. Children presenting with 22q11DS between 2010 and 2020 were included. Temporal bone imaging with computed tomography or magnetic resonance imaging was reviewed by 2 neuroradiologists. Twenty-two patients (12 female, 10 male) were identified. Forty-four ears were evaluated on imaging. There were 15 (34%) ears with abnormal semicircular canals, 14 (32%) with abnormal vestibules, 8 (18%) with abnormal ossicles, 6 (14%) with enlarged vestibular aqueducts, 4 (9.1%) with abnormal facial nerve canals, and 4 (9.1%) with cochlear anomalies. There were 25 ears with imaging and audiometric data. The median pure tone average (PTA) for ears with any structural abnormality was 41.0 dB, as compared with 28.5 dB for ears without any structural abnormality (P = .21). Of 23 ears with normal imaging, 6 (26%) had hearing loss in comparison with 13 (62%) of 21 ears with abnormalities (P = .02). Total number of anomalies per ear was positively correlated with PTA (Pearson correlation coefficient, R = 0.479, P = .01). PTA was significantly higher in patients with facial nerve canal anomalies (P = .002), vestibular aqueduct anomalies (P = .05), and vestibule anomalies (P = .02). Semicircular canal, ossicular, vestibular aqueduct, and vestibular anomalies were detected in children with 22q11DS, especially in the setting of hearing loss. Careful evaluation of anatomic anomalies is needed prior to surgical intervention in these patients.

Midline Facial Nerve Monitoring: Single-Center Experience and Review of Literature.

Background: Facial nerve monitoring system has enabled facial muscle activity detection using electrodes placed over the target muscles. In an effort to enable the best and minimally invasive surgical approach, a midline facial nerve monitor was applied during bilateral cochlear implantation surgical intervention in our center and the feasibility of placing midline facial nerve monitor electrodes during bilateral cochlear implantation operations was evaluated.Methods: The medical records and operative notes of all patients who underwent bilateral cochlear implantation surgery between January 2017 and April 2018 in a quaternary care center were retrospectively reviewed and divided into 2 groups based on the facial nerve monitoring methods: facial nerve monitoring with the midline (midline facial nerve monitor) or bilateral (bilateral facial nerve monitor) electrode placements. Basic demographic information, comorbidities, and facial nerve status (pre- and postoperatively) were collected from patient electronic medical charts. The operative notes were reviewed for abnormal facial nerve findings, as well as for any reported difficulties with the identification or stimulation of facial nerve. The primary outcome was facial nerve identification postoperative function.Results: Seventy-eight patients met our inclusion criteria. Midline facial nerve monitor was used in 49 patients and bilateral facial nerve monitor was used in 29 patients. No documented difficulty was identified at the step of facial nerve identification in either group, and none of the patients developed facial nerve weakness postoperatively.Conclusion: Midline facial nerve monitor is a safe and reliable method that can be used in bilateral cochlear implantation surgeries and other surgeries requiring facial nerve monitoring.

Determination of Extra Craniofacial Abnormalities in Patients With Craniofacial Microsomia

Craniofacial microsomia (CFM) is caused by abnormalities in the development of the first and second pharyngeal arches. One-third to half of the patients with CFM also present with extra craniofacial (ECF) malformations. The knowledge of the visceral alteration related to CFM is vital for optimized care and a better prognosis. To describe the incidence of ECF malformations in patients with CFM and to infer if there was a correlation between CFM and ECF malformations. The authors analyzed medical records of patients diagnosed with CFM from 1996 to 2006. The data collected included age, gender, category of craniofacial alteration, and the type of ECF malformation when present. The sample was inspected to find possible correlations between craniofacial abnormalities and ECF malformations. The sample included 102 patients, with a mean age of 7 years and a predominance of males (61.8%). Ear malformations (93.1%) followed by mandible (59.8%) and facial nerve (10.8%) abnormalities were the most common CFM. Among patients with CFM, 37.2% had ECF involvement, mainly in vertebrae (20%), heart (11%), and limbs (9.8%). Multivariate analysis revealed that the presence of ear malformations was related to a higher incidence of nonspecific visceral malformations (P = 0.034) and that mandible malformation was related to an increased incidence of vertebral malformations (P = 0.008). A significant percentage of patients with CFM presented associated ECF impairment. Ear and mandible involvement may be predictors of nonspecific visceral malformation and vertebral malformations, respectively.

High-resolution computed tomography evaluation of congenital aural atresia - how useful is this?

To depict various temporal bone abnormalities on high-resolution computed tomography in congenital aural atresia patients, and correlate these findings with auditory function test results and microtia subgroup. Forty patients (56 ears) with congenital malformation of the auricle and/or external auditory canal were evaluated. Auricles were graded according to Marx's classification, divided into subgroups of minor (grades I and II) and major (III and IV) microtia. Other associated anomalies of the external auditory canal, tympanic cavity, ossicular status, oval and round windows, facial nerve, and inner ear were evaluated. Minor and major microtia were observed in 53.6 and 46.4 per cent of ears respectively. Mean hearing levels were 62.47 and 62.37 dB respectively (p = 0.98). The malleus was the most commonly dysplastic ossicle (73.3 vs 80.8 per cent of ears respectively, p = 0.53). Facial nerve (mastoid segment) abnormalities were associated (p = 0.04) with microtia subgroup (80 vs 100 per cent in minor vs major subgroups). Microtia grade was not significantly associated with mean hearing levels or other ear malformations, except for external auditory canal and facial nerve (mastoid segment) anomalies. High-resolution computed tomography is essential in congenital aural atresia, before management strategy is decided.

High Resolution Computed Tomography (HRCT) Imaging Findings of Oval Window Atresia with Surgical Correlation

Isolated oval window atresia (OWA) is a rare cause of congenital conductive middle ear deafness and may be overlooked owing to the normal appearance of the external ear. This anomaly has been previously described, although the published numbers with both imaging and surgical findings are few. Our aim is to correlate the imaging features of OWA with intraoperative findings. This is a single-centre retrospective evaluation of patients who were diagnosed with OWA and who received surgery from January 1999 to July 2006. No new case was diagnosed after 2006 to the time of preparation of this manuscript. High resolution computed tomography (HRCT) imaging of the temporal bones of the patients were retrospectively evaluated by 2 head and neck radiologists. Images were evaluated for the absence of the oval window, ossicular chain abnormalities, position of the facial nerve canal, and other malformations. Imaging findings were then correlated with surgical findings. A total of 9 ears in 7 patients (two of whom with bilateral lesions) had surgery for OWA. All patients had concomitant findings of absent stapes footplate with normal, deformed or absent stapes superstructure and an inferiorly displaced facial nerve canal. HRCT was sensitive in identifying OWA and associated ossicular chain and facial nerve abnormalities, which were documented surgically. OWA is a rare entity that can be diagnosed with certainty on HRCT, best visualised on coronal plane. Imaging findings of associated middle ear abnormalities, position of the facial nerve canal, which is invariably mal-positioned, and associated deformity of the incus are important for presurgical planning and consent.

Intraoperative Neurophysiological Monitoring during Microvascular Decompression Surgery for Hemifacial Spasm

Hemifacial spasm (HFS) is due to the vascular compression of the facial nerve at its root exit zone (REZ). Microvascular decompression (MVD) of the facial nerve near the REZ is an effective and curative treatment for HFS. In MVD for HFS, intraoperative neurophysiological monitoring (IONM) has two purposes. The first purpose is to prevent injury to neural structures such as the vestibulocochlear nerve and facial nerve during MVD surgery, which is possible through IONM of brainstem auditory evoked potentials and facial nerve electromyography (EMG). The second purpose is the unique feature of MVD for HFS, which is to assess and optimize the effectiveness of the vascular decompression. The purpose is achieved mainly through monitoring of abnormal facial nerve EMG that is called as lateral spread response (LSR) and is also partially possible through Z-L response, facial F-wave, and facial motor evoked potentials. Through IONM mentioned above, MVD can be developed as a more safe and effective treatment for HFS. Keywords: Hemifacial spasm; Microvascular decompression surgery; Intraoperative neurophysiological monitoring

Bilateral Facial Nerve (Bell’s) Palsy in a 24-Year-Old Woman: A Case Report

ABSTRACT&#x0D; Objective: To report a case of acute bilateral facial nerve palsy in a 24-year-old woman and to present the differential diagnoses, pathophysiology, management and prognosis&#x0D; Methods: &#x0D; Design: Case study&#x0D; Setting: Tertiary Private Hospital&#x0D; Patient: One (1)&#x0D; Result: A 24-year-old woman with fever, joint pains, cough, chest pain, difficulty ambulating and progressive facial muscle weakness was diagnosed with rheumatic fever. Bilateral facial nerve paralysis was noted, and Electromyography-Nerve Conduction Velocity (EMG-NCV) testing with special facial nerve study revealed abnormal facial nerve and blink reflex studies while EMG-NCV of the upper and lower limbs were normal. Audiometry and MRI of the brain and facial nerve were normal while Schirmer’s Test showed decreased tearing in both eyes. The rheumatic fever resolved within 5 days of antibiotics, while Prednisone and physiotherapy resulted in improvement of facial paralysis from House Brackmann V to House Brackmann II-III over a period of 6 months.&#x0D; Conclusion: Idiopathic facial paralysis or Bell’s Palsy is the most common cause of acute unilateral facial paralysis while bilateral facial nerve paralysis is a rare condition. Patients with facial palsy should undergo appropriate diagnostics to determine the underlying condition and to facilitate prompt management.&#x0D; Keywords: facial paralysis, idiopathic; Bell’s palsy

Aberrant course of facial nerve in the middle ear cavity

Facial nerve (FN) course anomaly is often found in patients with congenital aural atresia or other ear anomaly, but in this case report, we report an abnormal FN course with dehiscent facial canal found in a patient with congenital conductive hearing loss without any accompanying auricular deformity or cholesteatoma.

Displacement of the Facial Nerve by Deep Parotid Lobe Lesions in the Pediatric Population.

ObjectivesTo describe and investigate facial nerve displacement in deep lobe parotid lesions in children and to determine clinical and radiographic predictors of abnormal facial nerve position.MethodsRetrospective case review of children who underwent total parotidectomy for deep lobe parotid lesions at a tertiary care center between January 2014 and December 2017. Aberrant facial nerve trajectory was defined as ascension of the nerve at an angle of 45° or greater. Elongation was defined as the main trunk >2 cm in length. Patient demographics, radiographic, pathologic results, postoperative nerve weakness, and intraoperative nerve findings were collected. Wilcoxon rank‐sum test and Fisher's exact test were used to assess the associations between variables of interest and facial nerve position.ResultsA total of 20 patients were included. The mean age was 7.7 ± 5 years. The most common pathologies were lymphatic malformation, pleomorphic adenoma, and first branchial cleft cyst. Twelve out of twenty (60.0%) patients had abnormal intraoperative facial nerve position. There was no significant difference in distribution of pathologies between those with or without an abnormal intraoperative nerve position (P = .41). Neither radiographic lesion size nor distance between the lesion and proximal portion of the facial nerve (mastoid tip) were associated with abnormal facial nerve position intraoperatively.ConclusionPediatric deep lobe parotid lesions can displace the facial nerve and distort its anatomy in a posterior lateral direction, in approximately 60% of patients. Statistical analysis of increased numbers of patients to further define predictors of aberrant nerve course is warranted.Level of Evidence4.

Congenital middle ear malformation: clinical analysis and discussion of classification

Objective: To analyze the clinical characteristics and appropriate surgical procedures, and discuss the classification of congenital middle ear malformation. Methods: All cases were from the Center of Otorhinolaryngology, the Sixth Medical Center of Department of PLA General Hospital. All of these cases, including 26 male patients (ears) , 10 female patients (11 ears) , aged from 7 to 57 years old, had normal external auditory canal, tympanic membrane, conductive hearing loss, type A tympanogram and negative Gelle's test. Tympanoplasty was performed in all cases. The deformity was classified to three types,i.e., Type I (stapes foot plate mobility): Ⅰa, ossicular chain deformity with normal stapes suprastructure; Ⅰb, ossicular chain deformity with abnormal stapes suprastructure; Type Ⅱ (stapes foot plate fixation): Ⅱ a,normal ossicular chain, Ⅱ b, ossicular chain malformation; and Type Ⅲ: vestibular window osseous atresia or undeveloped, or with round window atresia. The malformation of type Ⅱ and Ⅲ may be accompanied with abnormal facial nerve. In addition, the papers on middle ear malformation published from 1982 to 2017 were analyzed retrospectively. The clinical data of 451 ears malformation were summarized. Results: According to the revisional classification criteria in 37 ear samples from our hospital, 20 ears were type I. 6 type Ⅰa cases were used PORP (partial ossicular replacement prosthesis) to reconstruct the ossicular chain; 14 type Ⅰb cases were used TORP (total ossicular replacement prosthesis) to reconstruct the ossicular chain. For the 5 ears of type Ⅱ, 2 of which were type Ⅱ a and 3 were type Ⅱ b. 4 ear samples of type Ⅱ were implanted with Piston ossicular prosthesis, 1 was implanted with TORP in which the ossificated foot plate was removed with periosteum preserved. 12 ear samples were type Ⅲ, with vestibular window osseous atresia, facial nerve malformation, and stapes suprastructure malformation. The pistons ossicular prosthesis were implanted in vestibular window in 3 ears with facial nerve covering vestibular window partially. The surgery had to be given up in 5 ears, and TORP was implanted in 4 ears at the opening with preserved periosteum at the beginning of the tympanic scala because of facial nerve covering vestibular window totally. 30 ears with complete follow-up data had no sensorineural hearing loss and the average air-bone conduction decreased 23.3±10.7 dB (P<0.05).There were 234 ears of type Ⅰ in 451 ears of congenital middle ear malformation reported in the literature. 113 of which were type Ⅰa, the basic surgery was ossicular chain shaking and artificial or autogenous PORP implantation. Type Ⅰb was 121 ears, with autogenous or artificial TORP and PORP. Type Ⅱ was125 ears, including type Ⅱa 22 ears, Ⅱb 60 ears, and no subclassification for 43 ears. The surgery of type Ⅱ was the same as otosclerosis. The vestibular window atresia of type Ⅲ was 92 ears, the surgery of 17 ears had to be abandoned, the other ears underwent vestibular window, promontory or semicircular canal opening to reconstruct hearing with Piston, autogenous or artificial TORP. Conclusion: Referring to the classification of congenital middle ear malformation combining with appropriate surgical materials and methods, otologists can better understand and choose appropriate surgical method to the middle ear malformation.

Intraoperative Neurophysiological Monitoring during Microvascular Decompression Surgery for Hemifacial Spasm.

Hemifacial spasm (HFS) is due to the vascular compression of the facial nerve at its root exit zone (REZ). Microvascular decompression (MVD) of the facial nerve near the REZ is an effective treatment for HFS. In MVD for HFS, intraoperative neurophysiological monitoring (INM) has two purposes. The first purpose is to prevent injury to neural structures such as the vestibulocochlear nerve and facial nerve during MVD surgery, which is possible through INM of brainstem auditory evoked potential and facial nerve electromyography (EMG). The second purpose is the unique feature of MVD for HFS, which is to assess and optimize the effectiveness of the vascular decompression. The purpose is achieved mainly through monitoring of abnormal facial nerve EMG that is called as lateral spread response (LSR) and is also partially possible through Z-L response, facial F-wave, and facial motor evoked potentials. Based on the information regarding INM mentioned above, MVD for HFS can be considered as a more safe and effective treatment.

Surgical Management for Dysplastic or Congenitally Absent Oval Window.

To evaluate surgical findings and hearing results for patient's undergoing the described surgical approach for congenitally absent or dysplastic oval window (OW). The Institutional Review Board approved retrospective review of patients with conductive hearing loss (CHL) operated on from 1992 to 2016. Academic tertiary center. Patients with CHL, an intact tympanic membrane (TM), and without history of chronic infection underwent middle ear exploration. Eleven patients and 13 ears underwent an oval window drill-out (OWD) procedure. Eleven patients presented, all with dysplastic or congenitally absent oval window (CAOW). CHL was identified using audiometry and tuning forks, many patients also had preoperative computed tomography temporal bones. A transcanal approach was used and an OWD was performed with a variety of prostheses placed. Audiometric studies before and after intervention were compared with 12 month and long-term follow-up (1-22 yr). Preoperative air-bone gaps ranged from 40 to 60 dB and averaged 55.1 dB. Postoperative air-bone gaps ranged from 0 to 60 dB and averaged 24.1 dB. The preoperative pure-tone average (PTA) ranged from 55 to 99 dB and averaged 71.3 dB. Postoperative PTA ranged from 21 to 108 dB and averaged 49.6 dB. Dysplastic and CAOW are uncommon congenital major ear anomalies. OWD is a viable treatment option, though careful counseling is critical, as significant complications are possible, especially with facial nerve (FN) abnormalities. This series demonstrates successful closure of the air-bone gap for many patients with this technique.

The Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome.

CHARGE syndrome is associated with a variety of temporal bone anomalies and deafness. The lack of surgical landmarks and facial nerve irregularities make cochlear implantation in this population a challenging endeavor. This study aims to describe a safe and efficacious transcanal approach for cochlear implantation that obviates the need to perform a mastoidectomy and facial recess. Three children with profound hearing loss secondary to CHARGE syndrome. Transcanal cochlear implantation with closure of the ear canal via a modified Rambo meatoplasty. Retrospective chart review of temporal bone anomalies associated with CHARGE syndrome, technical nuances of this transcanal approach, and cochlear implant outcomes. The mean patient age was 2.5 years (range 1.5-3.8 yr). Two were male and two were left ears. All patients had a hypoplastic mastoid, semicircular canal aplasia, and had some degree of cochlear dysplasia. A full cochlear implant insertion was achieved in all cases, even in the presence of grossly abnormal middle ear and facial nerve anatomy. There were no intraoperative or postoperative complications. The mean follow-up was 12.4 months (range, 3.9-25.2 mo). All three patients use their device daily. Their guardians report improved vocalization and environmental awareness. The modified Rambo transcanal approach provides a safe corridor for cochlear implantation in patients with CHARGE syndrome. This approach minimizes the anatomical variations associated with the syndrome and may reduce the risk of electrode extrusion. Implant outcomes in this patient population remain highly variable based on the patient's global cognitive capacity.

Classification and Current Management of Inner Ear Malformations.

Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.

Cochlear Implantation in Children with Anomalous Cochleovestibular Anatomy: Our Experience.

To report operative findings, postoperative course, and postimplantation performance in patients with cochlear malformations who underwent cochlear implantation. Seventeen patients with malformations which included enlarged vestibular aqueduct (n=6), Mondini's dysplasia (n=5) common cavity deformity (n=3) and incomplete partition type 2 (n=3) underwent cochlear implantation with Nucleus 22 straight array device at our center. Operative findings described facial nerve anatomy and cerebrospinal fluid leak. Standard tests of speech perception were used to evaluate the postoperative performance for each subject. Operative findings included cerebrospinal fluid leak (thirteen patients) all of which were repaired successfully with graft. None had abnormal facial nerve anatomy. No surgical complications occurred. All the patients except two with common cavity had complete insertion. Electrode thresholds and discomfort levels were variable for several months after implantation. All patients demonstrated improved performance after implantation. Patients with enlarged vestibular aqueduct fared better than patients with other inner ear malformations. Cochlear implantation can be a successful method of rehabilitation in patients with congenital deafness who have cochlear malformations.

Facial nerve anomalies in paediatric cochlear implant candidates: radiological evaluation.

Pre-operative radiological identification of facial nerve anomalies can help prevent intra-operative facial nerve injury during cochlear implantation. This study aimed to evaluate the incidence and configuration of facial nerve anomalies and their concurrence with inner-ear anomalies in cochlear implant candidates. Inner-ear and concomitant facial nerve anomalies were evaluated by magnetic resonance imaging and temporal high-resolution computed tomography in 48 children with congenital sensorineural hearing loss who were cochlear implant candidates. Inner-ear anomalies were present in 11 out of 48 patients (23 per cent) and concomitant facial nerve anomalies were present on 7 sides in 4 patients (7 per cent of the total). Facial nerve anomalies were accompanied by cochlear or vestibular malformation. Potential facial nerve abnormalities should always be considered in patients with inner-ear anomalies. Pre-operative facial nerve imaging can increase the surgeon's confidence to plan and perform cochlear implantation. Magnetic resonance imaging should be used to detect inner-ear anomalies; if these are identified, temporal high-resolution computed tomography should be used to evaluate the facial nerve.