Anomalous Facial Nerve Research Articles

Cochlear Implantation in Goldenhar Syndrome.

Goldenhar syndrome is a rare genetic condition characterized by hemifacialmicrosomia, mandibular hypoplasia, auricular malformations, and epibulbardermoids. The syndrome has both sporadic and familial occurrence.Incidence of congenital hearing loss in these patients is 1:1000 in children with a male to female ratio of 3:2. In our case study we report a case of Goldenhar Syndrome who underwent cochlear implantation. The patient had right side microtia, right hemifacial microsomia and right side torticollis, pterygium in her right eye, right hypoplastic thumb and unilateral right side kidney. Radiologically, there wasnarrow duplicated internal auditory canal on right side with absent right cochlear nerve with normal anatomy on left side and the left side showed malformed facial nerve at tympani segment and second genu. Therefore, the patient was planned for left side cochlear implantation. Intraoperatively, there were malformed ossicles with anomalous facial nerve covering whole of oval window and partially the round window. Thus, a separate cochleostomy was done.Impedance was < 5 Hertz in all electrodes and electrically evoked action potential (ECAP) thresholds were obtained on all electrodes.

Intratemporal variations of facial nerve course

Facial nerve (FN) is the seventh cranial nerve with a unique complex anatomical course. Knowledge about the normal predictable course within the temporal bone with its possible variations is crucial to avoid accidental injuries to the FN, preventing its devastating subsequences. In ear surgeries, certain landmarks and relations to each other are key for safe drilling. In this report, we describe a case with computed tomography findings during routine preoperative assessment for cochlear implant with anomalous FN course being displaced posterolaterally on the tympanic and mastoid segments. This highlights the importance of imagining before such surgeries.

A Case of Cochlear Implantation in a Child with Congenital Aural Atresia and Cochlear and Cochlear Nerve Malformation

Congenital aural atresia (CAA) is usually accompanied by an anomalous course of facial nerves, which may run over the round window niche toward the promontory in some cases of CAA. Very rarely, cochlear malformation might coexist at the same ear which has CAA, in which case cochlear implantation can be difficult due to anomalous facial nerve. Herein we present a case of cochlear implantation performed in a child who had CAA along with cochlear hypoplasia and cochlear nerve aplasia. The mastoid segment of facial nerve was displaced anteriorly, running over the round window niche. The surgery was done successfully using the round window approach without facial nerve injury.

A Case of Congenital Bifurcation of the Labyrinthine Segment of Facial Nerve

The most common anomaly of facial nerve is bony dehiscence of the fallopian canal, being present in approximately 55% of the population. Abnormal courses of facial nerve are reported commonly. Of the malformation of the facial nerve, bifurcation of its intratemporal portion has been rarely reported, in which one or more segments of the facial nerve splits. Nine years-old girl (right-handed) came to our outpatient clinic for left hearing loss, which was incidentally found by herself. Audiologic and radiologic work-up demonstrated unilateral sensorineural hearing loss, which was resulted from the narrow bony cochlear nerve canal, and incidentally found the bifurcation of the labyrinthine segment of ipsilateral facial nerve. We described characteristic findings on the computed tomogram of this anomaly and discussed it. It is important to identify the anomalous facial nerve in the patients undergoing evaluation for congenital hearing loss. (J Clinical Otolaryngol 2019;30:83-87)

CT Imaging Categorization and Biomarker Study of Anomalous Tympanic Segment of the Facial Nerves in Patients With Hearing Loss in the Absence of Microtia.

The purpose of this study is to categorize anomalous tympanic facial nerve (FN) on high-resolution computed tomography (HRCT) and to determinate the significance of associated temporal bone anomalies and congenital syndromes without microtia in patients with hearing loss. A retrospective analysis of HRCT findings in 30 temporal bones in 18 patients with anomalous FN was performed. Abnormalities of the tympanic FN were categorized as follows: category 1: FN medially positioned, but above the oval window; category 2: FN in the oval window niche; and category 3: FN below the oval window. Potential associated findings that were assessed included stapes abnormalities, oval window atresia, and inner ear anomalies, as well as the presence of a known congenital syndrome with hearing loss. The most common type of anomalous tympanic FN was category 1 (67%, n = 20), following by group 2 (20%, n = 6) and group 3 (13%, n = 4). Stapes anomalies were detected in 77% of temporal bones (n = 23), oval window atresia was detected in 43% of temporal bones (n = 13), and inner ear anomalies were detected in 70% of temporal bones (n = 21). Anomalous tympanic facial nerves in temporal bone with conductive hearing loss were often (60%) not associated with oval window atresia. The combination of aberrant tympanic FN and inner ear anomalies was significantly (P = .038) associated with a known congenital syndrome (6 patients), including CHARGE syndrome, oculo-auriculo-vertebral spectrum, Pierre-Robin sequences, and Down syndrome. Therefore, an anomalous tympanic FN in conjunction with inner ear anomalies appears to be a biomarker for certain congenital syndromes with hearing loss in the absence of microtia.

Anomalies of the facial nerve: a surgeon’s nightmare

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Most of the anomalies of the facial nerve have been encountered during otological surgery or dissection of the temporal bones. ENT surgeons are taught from a nascent stage to always be wary of an anomalous facial nerve during otological surgery. Today’s surgeon is assisted with high definition imaging and nerve monitoring; yet iatrogenic facial palsy still is encountered even today.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; This study was conducted in a select population of patients who reported with aural symptoms with an aim to see the number of facial nerve anomalies one encounters during aural surgery. The filter applied was no patient with congenital anomaly was considered and patients with squamous COM were also excluded. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; Almost 4.5% of the patients subjected to surgery had varying kinds of facial nerve anomaly, the most common being dehiscence of the fallopian canal.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Facial nerve anomalies are not so uncommon as one expects it to be. So it is mandatory that every ENT surgeon should be well versed with facial nerve anatomy and be wary of any structural anatomical abnormality, and irrespective of the experience it pays to be extra cautious when operating on the ear because in the event of damage to the facial nerve the patient has to carry the stigma of a facial deformity for his/her life.&lt;/p&gt;

Anomalous Extratemporal Facial Nerve in Oculoauriculovertebral Spectrum

Facial nerve dysfunction is common in oculoauriculovertebral spectrum (OAVS). However, the course of the nerve has rarely been described. A 23-year-old woman with OAVS underwent excision of microtic ear remnants in preparation for an osseointegrated prosthesis and suffered iatrogenic transection of the facial nerve-the pes anserinus was within the subcutaneous tissue 15 mm posterior and 15 mm cephalad to the external acoustic meatus. The patient underwent primary nerve repair and regained nearly complete preoperative function. When considering reconstruction for OAVS patients, clinicians should have a high index of suspicion for anomalous facial nerve anatomy.

Vestibulotomy in Congenital Stapes Fixation with Anomalous Facial Nerve

Vestibulotomy in Congenital Stapes Fixation with Anomalous Facial Nerve

Surgical findings &amp; audiological outcomes of Stapedotomy in patients with Otosclerosis

Objective: This study was focused on the assessment of patient’s improvement in hearing, as measured by pure tone audiometry after stapedotomy for primary otosclerosis. Materials and Methods: This prospective clinical study was performed in a total of 50 patients diagnosed with Primary Otosclerosis, who underwent Stapedotomy at the Department of ENT &amp; Head-Neck Surgery, Combined Military Hospital, Dhaka between June 2012 to January 2014. All patients were evaluated as per the candidacy criteria for stapedotomy and selected patients underwent surgery during the study period and were followed up for a period of 12 months in the Otology clinic. Pre-operative and Post-operative audiometric evaluation was done using conventional pure tone audiometry with standard calibrations. Post-operative audiometry was performed at 03 month, 06 months and 12 months. The bone-conduction &amp; air conduction thresholds and the Air- Bone gap (ABG), were documented and analysed at 0.5 KHz, 1 KHz, 2 KHz and 4 KHz frequencies respectively. The subjective outcomes in hearing were also recorded with a patient satisfaction questionnaire to assess improvement in quality of hearing after stapedotomy. Results: Overall, the frequency specific pre-operative mean averaged Air Conduction thresholds were 63.3dB at 500Hz, 57.5dB at 1 KHz, 55.1dB at 2 KHz and 45.7dB at 4 KHz. The frequency specific post-operative mean averaged Air conduction thresolds were 34.6 dB at 500Hz, 28.9 dB at 1Kz, 30.5dB at 2Kz and 33.3dB at 4Kz.The frequency specific mean average post operative Air Bone Gap (ABG) closure was achieved by 28.7dB at 500Hz, 27.6 dB at 1KHz, 24.6 dB at 2KHz and 12.4 dB at 4KHz by the time of completion of the study at 01 year. A successful closure of AB gap to less than 10dB was achieved in the speech frequencies of 2 KHz and 4 KHz in 84% of cases. Overall, the frequency specific bone-conduction thresholds were unchanged postoperatively in all cases except one, showing that no significant sensori-neural impairment had occured due to the stapedotomy procedure. 1 patient developed post-op severe vertigo which was self-limiting after six weeks. A few interesting cases with anomalous intra-operative findings were also documented and reported herewith. These included monopodal stapes (n=1), anomolous facial nerve (n=1), laterally placed chorda tympani nerve (n=1), high jugular bulb, Malleus ankylosis (n=1) and persistent stapedial artery (n=1). All patients included in our study had significant subjective audiological improvement and responded satisfactorily to the questionnaire formulated to assess their hearing quality after stapedotomy. Conclusions: Our case study confirms that stapedotomy is a safe and successful procedure providing long-term hearing improvement in primary otosclerosis. Obliterative otosclerosis, biscuit or floating footplate, monopodal stapes, anomalous facial nerve and persistent stapedial artery may be special scenarios encountered during stapedotomy and they need judicious management by an experienced surgeon. Our study shows that meticulous selection of cases for stapedotomy will result in highly successful audiological outcomes. DOI: http://dx.doi.org/10.3329/bjo.v20i2.22024 Bangladesh J Otorhinolaryngol; October 2014; 20(2): 87-92

Anomalous Facial Nerve in Congenital Stapes Fixation

The aim of this article was to assess the prevalence and influence of anomalous facial nerves in patients undergoing surgery for congenital stapes anomalies. The medical records of all patients who underwent surgery for congenital stapes fixation between January 1999 and December 2012 were retrospectively reviewed. An abnormal facial nerve was found in 7 ears in 5 patients (from a total of 62 ears). The patients' temporal bone computed tomography results, operation records, video clips, and preoperative and postoperative hearing tests were reviewed. Oval window atresia was found in all 7 ears, and the facial nerves in all 7 ears ran inferior medially over the atretic oval window niche. The atretic plate was fenestrated into the vestibule in the superior margin of the facial nerve. A piston wire was anchored to the long process of the incus in 3 ears, to the handle of the malleus in 3 ears, and attached to the tympanic membrane in 1 ear. The postoperative ABG at long-term follow-up was 13.1 dB (SD, 4.6 dB; range, 8.8-18.3 dB). No patient had postoperative facial weakness. An anomalous facial nerve was found in 11.2% of ears in patients undergoing surgery for congenital stapes fixation. Despite the anomalous course of facial nerves in these patients, vestibular fenestration was successful without facial nerve damage. The audiometric outcome at long-term follow-up was serviceable hearing for all cases.

Cochlear Implantation in Children with Anomalous Cochleovestibular Anatomy

To determine the influence of inner ear dysplasia on both surgical and audiologic outcomes following pediatric cochlear implant (CI) surgery. MEDLINE (1982-2009) and data from Massachusetts Eye and Ear Infirmary. A systematic review of the literature was performed. Variables assessed included age at implantation, duration of CI use, radiologic and operative findings, and speech perception outcome data. The initial search yielded 1326 articles. Including data from our own study, twenty-two fulfilled criteria for inclusion, representing 311 patients. Data for bilateral implants were recorded only for the first implant. Data on simultaneous bilateral implants were not recorded. The most common anomaly seen was large vestibular aqueduct (89/311 or 29%). When comparing patients with mild-moderate or severe dysplasia, rates of cerebrospinal fluid gusher were 31% versus 35% (odds ratio [OR] = 0.50), anomalous facial nerve anatomy was seen in 11% versus 51% (OR = 0.15), and postoperative speech perception abilities were found in 84% versus 54% (OR = 1.93), respectively. A large heterogeneity was found among studies regarding all outcome measures. Although we found that severe inner ear dysplasia was associated with increased surgical difficulty and lower speech perception, the lack of uniformity in published clinical data limited the strength of these results. Standardization of surgical and radiologic reporting as well as more consistent speech perception testing is needed to better determine the association between anomalous cochleovestibular anatomy and clinical outcomes.

Retrofacial approach of cochlear implantation in inner ear malformation with aberrant facial nerve: A case report

Cochlear implantation is regarded as a safe surgery for young children with minimal complications. However, inner ear malformations and aberrant course of facial nerves may impede electrode insertion via the round window approach and increase the risk of iatrogenic facial nerve injury. We report a case of cochlear incomplete partition in a patient with anomalous facial nerve anatomy. The anterior and inferior displacement of the facial nerve obscured the round window. A retrofacial approach was used to expose the round window and the electrode was inserted successfully. No surgical complications were found postoperatively, and the child showed significant improvement in speech perception. As the course of the aberrant facial nerve is difficult to track preoperatively, surgeons should proceed with caution to reduce the risk of facial nerve injury during the operation.

Surgical Anatomy of the Anterior and Retrofacial Approach to the Sinus Tympani (ST)

Cochlear implantation is regarded as a safe surgery for young children with minimal complications. However, inner ear malformations and aberrant course of facial nerves may impede electrode insertion via the round window approach and increase the risk of iatrogenic facial nerve injury. We report a case of cochlear incomplete partition in a patient with anomalous facial nerve anatomy. The anterior and inferior displacement of the facial nerve obscured the round window. A retrofacial approach was used to expose the round window and the electrode was inserted successfully. No surgical complications were found postoperatively, and the child showed significant improvement in speech perception. As the course of the aberrant facial nerve is difficult to track preoperatively, surgeons should proceed with caution to reduce the risk of facial nerve injury during the operation.

Facial nerve anomalies in association with congenital hearing loss

Objective: Facial nerve (FN) anomalies are exceedingly rare. The association between a hearing loss and an anomalous facial nerve has never been touched deeply in the literature. The aim of this study is to report facial nerve anomalies in patients presenting with congenital hearing loss (COHL) and/or facial nerve palsy. Methods: A retrospective chart review over 10 years from two academic tertiary referral centers. Eight patients were diagnosed with facial nerve anomalies in association with congenital hearing loss. The age ranged from 7 months to 13 years. Results: Five patients had conductive congenital hearing loss (CCHL). Two had congenital sensorineural hearing loss (CSNHL). The eighth patient had a mixed hearing loss (MHL). Surgical findings for patients with conductive congenital hearing loss included facial nerve passing inferior to oval window (OW) (two patients), facial nerve covering head of stapes (three patients), deformed stapes (two patient), deformed incus (two patient), and absent incus (one patient). Radiological findings for patients with congenital sensorineural hearing loss and mixed hearing loss include, single cavity anomaly (one patient), dilated vestibule and superior semicircular canal (one patient), and absent internal auditory canal (IAC) (two patients). For patients with conductive congenital hearing loss, the radiological findings consistency demonstrates a soft tissue opacification in the middle ear. Conclusions: Congenital hearing loss may be associated with facial nerve anomalies. Pediatric otolaryngologists should be cautious when exploring patients with conductive congenital hearing loss.

Cochlear implantation in patients with cochlear malformations.

To report operative findings, postoperative course, and postimplantation performance in patients with cochlear malformations who underwent cochlear implantation. Case study and intervention study (before-after trial). Minimum follow-up of 12 months; average follow-up of 24 months. Academic tertiary referral center. Six patients, including five children who underwent implantation at ages 3.5 to 13 years and one adult who underwent implantation at age 27 years. malformations included common cavity deformity (n = 1), cochlear hypoplasia (n = 2), and incomplete partition (n = 3). All patients with cochlear malformations who underwent implantation at the University of Michigan, Ann Arbor, are included, selected from a group of 196 patients so treated since 1986. Implantation with a standard multichannel cochlear implant. Operative findings described include round window and facial nerve anatomy and cerebrospinal fluid leak. Postoperative roentgenographic findings, electrode activation, and reason for non-use of electrodes were investigated. Standard tests of speech perception were used to compare preoperative and postoperative performance for each subject. Operative findings included round window abnormalities (three patients), anomalous facial nerve (one patient), and cerebrospinal fluid leak (three patients). No surgical complications occurred. A minimum of 10 electrodes were activated for all patients. Electrode thresholds and discomfort levels were variable for several months after implantation. All patients demonstrated improved performance after implantation. Four subjects demonstrated open-set speech perception. Two other subjects, whose poor language skills precluded administration of standard tests, showed increased awareness of environmental sounds and increased vocalization after implantation. Cochlear implantation can be a successful method of rehabilitation in patients with congenital deafness who have cochlear malformations.