Audiologic Outcomes After Vestibulotomy in Patients With Congenital Absence of the Oval Window.

OBJECTIVE: To examine the clinical features and surgical outcomes in patients with congenital absence of the oval window (CAOW), and to investigate the potential factors that affect audiologic results. STUDY DESIGN: A retrospective chart review. SETTING: A tertiary academic center. PATIENTS AND INTERVENTION: A total of 17 ears among 16 patients were confirmed to have CAOW. Among them, 13 ears underwent vestibulotomy for hearing reconstruction. Clinical parameters associated with the hearing outcomes were analyzed. MAIN OUTCOME MEASURES: A mean air-bone gap (ABG) after 6-month and long-term follow-up was compared with preoperative measurements. RESULTS: Intraoperative findings showed that anomalies of the malleus or incus were observed in 11 ears (64.7%), stapes anomalies were present in all ears (100%), and facial nerve anomalies were present in 10 ears (58.8%). Because of unfavorable facial nerve anomalies, hearing reconstruction was aborted in four cases (23.5%). In the hearing reconstruction group, the mean ABG at 6 months postoperation was significantly reduced after compared with the preoperative value (44.0 ± 8.4 dB versus 58.8 ± 9.1 dB, p = 0.006). After dividing ears into a success subgroup (ABG ≤ 30 dB, seven ears) and non-success subgroup (ABG > 30 dB, six ears), the use of a drill during vestibulotomy was significantly related to a poor hearing outcome (100% versus 16.7%, p = 0.015). The long-term follow-up result (mean, 60 mo) revealed no deterioration compared with the 6-month postoperative result. Five ears (29.4%) underwent revision surgery, and three of them showed ABG improvements. No serious complications were reported. CONCLUSION: Vestibulotomy is an effective and safe option for hearing restoration in patients with CAOW, particularly when the use of a drill is not required. The long-term audiologic outcome is also reliable.

[Cochlear implantation through retro-facial approach with congenital microtia malformation with facial nerve deformity: a case report].

The difficulty of cochlear implantation in patients with congenital microtia is usually increased due to the vague anatomical marks and facial nerve malformation. The common types of facial nerve malformation include facial nerve bony cover loss, aberrant position, and bifurcation malformation. Bifurcation malformation may obscure the oval window, press against stapes, and bifurcate in the vestibular window while obscuring the round window. It is important to correctly identify the facial nerve and choose a reasonable surgical approach to avoid postoperative complications. This article describes a case of profound sensorineural hearing loss due to facial nerve malformation in our institution. The patient underwent cochlear implantation through the retro-facial approach. There was no facial nerve injury or dysfunction symptoms such as facial paralysis and hemifacial spasm 2 years after the operation, and the cochlear implant works well. The score of the categories of the auditory performance（CAP） questionnaire was 7, and the score of the speech intelligibility rating（SIR） questionnaire was 4. When the round window cannot be exposed through the facial recess approach during surgery, the retro-facial approach is a feasible method. To avoid facial nerve injury, a thin-section CT of the temporal bone should be performed before the middle and inner ear surgery for patients with facial nerve malformation, and the intraoperative facial nerve monitor should be used to clarify the course of the facial nerve to avoid injury.

Síndrome de Moebius y ortotropía en posición primaria: ¿es infrecuente esta asociación? (Colombia) / Moebius syndrome and orthotropia in primary position: is this association uncommon? (Colombia) / Síndrome de Moebius e ortotropia em posição primária: é uma associação incomum? (Colômbia)

El síndrome de Moebius es una enfermedad congénita poco común que se caracteriza por el compromiso unilateral o bilateral del VI y VII par craneal, lo que compromete los músculos que controlan la oculomotricidad, produciendo una parálisis en la abducción del globo ocular y los músculos involucrados en la expresión facial. Su presentación clínica y grados de severidad son variables, puede presentar compromiso simétrico o asimétrico. Adicionalmente, gran parte de los casos se relacionan con trastornos del lenguaje, anomalías musculoesqueléticas y orofaciales. En el presente artículo se presenta el caso de una paciente femenina de 3 años producto de un embarazo trigemelar con diagnóstico clínico de síndrome de Moebius al nacer, confirmado por neuroimagen en la que se evidencia la ausencia bilateral del nervio facial en ángulos pontocerebelosos, adicionalmente con un defecto completo en los movimientos oculares de abducción y aducción lo que impide el estrabismo convergente común en estos pacientes.

Moebius syndrome is a rare congenital disease characterized by unilateral or bilateral involvement of the VI and VII cranial nerves, which compromises the muscles that control ocular motricity with paralysis in the abduction of the eyeball and the muscles involved in the facial expression. Its clinical presentation and degrees of severity are variable, and it can be symmetric or asymmetric. Additionally, most of the cases are related to language disorders, musculoskeletal and orofacial anomalies. This paper presents the case of a 3-year-old female patient, product of a trigemellar pregnancy with a clinical diagnosis of Moebius syndrome at birth, confirmed by neuroimaging, which shows the bilateral absence of the facial nerve in point-lateral angles. Additionally she has a complete defect in abduction and adduction eye movements, which prevents the common convergent strabismus in these patients.

A síndrome de Moebius é uma doença congênita rara caracterizada pelo envolvimento unilateral ou bilateral dos nervos cranianos VI e VII, que compromete os músculos que controlam a oculomotricidade com paralisia na abdução do globo ocular e dos músculos envolvidos na expressão facial. Sua apresentação clínica e graus de gravidade são variáveis, podendo ser um comprometimento simétrico ou assimétrico. Além disso, a maioria dos casos está relacionada a distúrbios de linguagem, anomalias musculoesqueléticas e orofaciais. Este paper apresenta o caso de uma paciente de 3 anos de idade, fruto de uma gravidez trigêmea com diagnóstico clínico de Síndrome de Moebius ao nascimento, confirmado por neuroimagem em que é evidente a ausência bilateral do nervo facial nos ângulos ponto-cerebelares. Além disso, ela tem um defeito completo nos movimentos oculares de abdução e adução, o que impede o estrabismo convergente comum nesses pacientes.

A bibliometric analysis of research on the treatment of facial nerve palsy.

BACKGROUND: There are various treatments for facial nerve palsy, and research into this topic is ongoing. In the present study, we carried out bibliometric and visualized analyses to identify the trends of research into facial nerve palsy treatment. METHODS: To identify articles, the SCOPUS database was searched for articles published from its inception to December 27, 2020. The search was conducted twice, with Search 1 investigating general treatment trends and Search 2 narrowing the scope to complementary and alternative treatment. The extracted keywords were analyzed using the Visualization Of Similarities (VOS) viewer. Through analysis of keywords, research hotspots in the treatment of facial nerve palsy were identified. RESULTS: A total of 1609 and 223 articles were identified in Searches 1 and 2, respectively. The number of articles published each year showed a tendency to increase, and most of the studies were only conducted in a few countries. In terms of subject area, "medicine" was overwhelmingly the most common(77.6%). Based on the analysis of 316 keywords in Search1, "medication treatment," and "complementary and alternative treatment" were the hotspots of research. CONCLUSION: This study provides the overall trends of facial nerve palsy treatment. To date, research on medication treatment has been main focus, and antiviral use among medication treatment and complementary and alternative treatment has emerged in recent years.

Salivary Gland Choristoma of the Middle Ear in a Child: A Case Report.

Salivary gland choristoma is an extremely rare middle ear pathology. We present the case of a 10-year-old girl with unilateral conductive hearing loss. Tympanotomy showed a nonspecific middle ear mass, absence of stapes, anomaly of incus, and displaced facial nerve. It was not possible to remove the mass completely. Histology confirmed salivary gland choristoma. The hearing in this case can be improved with a bone-anchored hearing aid.

Cochlear implant surgery through oval window: A novel approach in a child with facial nerve aberration.

Cochlear implantation is safe for simple case presentations. However, facial nerve (FN) aberrations may impede insertion of the electrode through a round window and increase the risk of iatrogenic FN injury. We developed a novel method of cochlear implantation, a present the case of a child with FN aberrations. Inferior displacement of a horizontal segment of FN lay on the surface of th e promontory, obscuring the round window. The oval window was used and the electrode successfully inserted. No postoperative complications occurred, and significant improvement in speech perception was achieved. This novel approach is suitable for patients with FN aberrations.

A Novel Classification: Anomalous Routes of the Facial Nerve in Relation to Inner Ear Malformations.

OBJECTIVES/HYPOTHESIS: The objective of this study was to classify anomalous facial nerve (FN) routes and to determine their association with inner ear malformations (IEMs). STUDY DESIGN: Retrospective cross sectional study. METHODS: The computed tomography images of 519 patients (796 ears) with IEMs were retrospectively evaluated, and the abnormal routes of the FN were classified as: Meatal segment: type 1, normal internal auditory canal (IAC); type 2, narrow IAC; type 3, facial canal (FC) only; type 4: separate FC/duplicated IAC. Labyrinthine segment (LS): type 1, normal; type 2a/b/c, mild/moderate/severe anterior displacement; type 3, superior displacement; type 4: straight LS. Tympanic segment (TS): type 1, normal; type 2, superiorly displaced TS; type 3, TS at the oval window; type 4: TS inferior to the oval window; type 5: unclassified. Mastoid segment: type 1, normal facial recess (FR)/normal mastoid segment; type 2: narrow FR; type 3, unclassified. RESULTS: In meatal segment classification, a narrow IAC was common in ears with cochlear hypoplasia (CH) (76.1%), and only FC was common in ears with severe IEMs (62.7%) such as Michel deformity, common cavity, and cochlear aplasia. Incomplete partition-III has its unique superiorly displaced LS (100%). CH-IV also has its unique mild anterosuperior displacement. Ears with a superiorly displaced TS usually (93.1%) had aplastic or hypoplastic semicircular canals. The FR is likely to be narrow in CH and severe IEMs. CONCLUSIONS: The FN route is affected in IEMs, which must be kept in mind when operating on ears with IEMs. Especially in CH cases, all segments of the FN can be abnormal. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:E696-E703, 2020.

Cochlear-facial dehiscence in a pediatric patient.

Temporal bone dehiscence is an infrequent but clinically important phenomenon. Cochlear-facial dehiscence, the rarest type, has been described only in adults. We report cochlear-facial dehiscence in a child. Knowledge of this entity is imperative for radiologists searching for anatomical markers of inner ear pathology and for cochlear implant planning.

Is CT necessary for imaging paediatric congenital sensorineural hearing loss?

Objectives: To determine if MRI alone is adequate for pre-operative assessment of paediatric congenital sensorineural hearing loss (SNHL). While aberrant intratemporal facial nerve anatomy is usually occult on MRI, we postulate that the majority of cases have no adverse bearing on surgical outcomes.Methods: MRI and CT of the temporal bones were analysed in 240 children who underwent both on the same day for SNHL. Only children under the age of 5 years with no reported clinical syndrome or dysmorphic external ear anatomy were included.Results and discussion: 169 patients satisfied the inclusion criteria. MRI detected 32/54 cases of cochleovestibular dysplasia, with the majority of the additional CT findings comprising subtle incomplete partition type 2 (IP2) anomalies. Of the 22 cases not evident on MRI, 13 patients also had large vestibular aqueduct syndromoe (LVAS), which would have prompted evaluation with CT due to the near universal co-existence of these entities. Only one patient exhibited aberrant intratemporal facial nerve anatomy that may have conferred surgical risk and was occult on MRI.Conclusion: In a defined paediatric SNHL cohort, the addition of routine temporal bone CT to MRI offers limited additional yield but confers a significant radiation burden on a young population.

Bifid Facial Nerve with Dual Origin Identified During Resection of Vestibular Schwannoma.

Facial nerve identification and preservation is a critical step in the resection of vestibular schwannoma. The use of intraoperative neurostimulation to positively identify the facial nerve along its entire course is essential to prevent injury. To our knowledge, this is the first reported case of a bifid facial nerve with a dual origin at the brainstem, which we observed during resection of vestibular schwannoma via the translabyrinthine approach. Both roots were visualized to join as one facial nerve trunk outside the brainstem in the cisternal segment of the facial nerve, and both trunks demonstrated positive signal with neurostimulation. This case highlights an important anatomic variation and also the importance of correct identification of facial nerve anatomy during resection of vestibular schwannoma and other pathologies within the cerebellopontine angle.

Systematic review of cochlear implantation in CHARGE syndrome.

Objective: CHARGE syndrome presents with a collection of congenital anomalies affecting multiple organs. Ear and temporal bone anomalies, including hearing loss are highly prevalent. We present an aid to management of this challenging condition and report the strategies and outcomes of cochlear implantation. Methods: Systematic review of Medline, EMBASE, Web of Science, CENTRAL and clinicaltrials.gov was performed up to 21/10/2018 The review and meta-analysis of studies were performed according to the PRISMA statement. Patient demographics, comorbidity, anatomical factors, details of cochlear implantation and audiological outcome were extracted and summarized. Anatomical and surgical factors were evaluated by meta-analysis. Audiological outcomes reported were too heterogeneous for meta-analysis. All statistics were calculated with SPSS v23.0 (IBM, New York, USA). Results: Thirty-one studies reported 165 cochlear implants in 156 patients with CHARGE syndrome. Temporal bone and facial nerve anomalies were common. Discussion: The assessment and management of patients with CHARGE syndrome undergoing cochlear implantation is challenging. Outcomes may be affected by cochlear nerve deficiency, inner ear anomalies, and developmental delay. Surgery is almost invariably complicated by abnormal anatomy, and increased incidence of complications. Conclusion: A careful, case-by-case assessment of an individual's requirements within a multi-disciplinary setup is essential for achieving the best possible outcome.

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.

Isolated Congenital Absence of Cranial Nerves: Report of Two Cases.

Isolated cranial nerve absence is a rare condition that can be diagnosed using high-resolution cranial nerve magnetic resonance (MR) imaging. Thorough clinical examination with proper knowledge of the course of cranial nerves may help diagnose this rare condition. We describe two cases, one each of, isolated congenital absence of the third and seventh cranial nerve with their clinical presentation. High-resolution T2-weighted MR imaging was done in both patients which revealed absence of cisternal segment of the right-sided third nerve and cisternal with canalicular segment of the right-sided facial nerve.

Atypical Presentation of Incomplete Kawasaki Disease: A Peripheral Facial Nerve Palsy.

BACKGROUND: Kawasaki disease (KD) is a multisystem vasculitic disease. Coronary artery aneurysms (CAAs) are the most important and life-threatening complication of KD. Various neurologic complications have been described to occur in 1-30% of patients with KD, but peripheral facial nerve palsy (FNP) is rare (0.9%). CASE REPORT: We describe a 5-month-old male infant who presented to us with unilateral left infranuclear FNP in the convalescent phase (day 18 of illness) of incomplete KD. The initial diagnosis was not made during the first 10 days of illness (therapeutic window for immunoglobulin treatment) as he was suspected to have hand-mouth-foot disease. We believe that both the delay in diagnosis and treatment of an atypical presentation of KD, combined with the more severe vasculitis and inflammatory burden reported in these cases, contributed to the development of CAA in our patient. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case highlights the importance of considering KD diagnosis in children with prolonged unexplained fever, even with incomplete diagnostic features, as well as the need to be aware of unusual manifestations, such as FNP. Atypical cases like this may be at increased risk of CAA because of delayed diagnosis and a higher inflammatory burden; therefore, a more aggressive treatment approach may be necessary.

Facial Paralysis in Patients With Hemifacial Microsomia: Frequency, Distribution, and Association With Other OMENS Abnormalities.

Although facial paralysis is a fundamental feature of hemifacial microsomia, the frequency and distribution of nerve abnormalities in patients with hemifacial microsomia remain unclear. In this study, the authors classified 1125 cases with microtia (including 339 patients with hemifacial microsomia and 786 with isolated microtia) according to Orbital Distortion Mandibular Hypoplasia Ear Anomaly Nerve Involvement Soft Tissue Dependency (OMENS) scheme. Then, the authors performed an independent analysis to describe the distribution feature of nerve abnormalities and reveal the possible relationships between facial paralysis and the other 4 fundamental features in the OMENS system. Results revealed that facial paralysis is present 23.9% of patients with hemifacial microsomia. The frontal-temporal branch is the most vulnerable branch in the total 1125 cases with microtia. The occurrence of facial paralysis is positively correlated with mandibular hypoplasia and soft tissue deficiency both in the total 1125 cases and the hemifacial microsomia patients. Orbital asymmetry is related to facial paralysis only in the total microtia cases, and ear deformity is related to facial paralysis only in hemifacial microsomia patients. No significant association was found between the severity of facial paralysis and any of the other 4 OMENS anomalies. These data suggest that the occurrence of facial paralysis may be associated with other OMENS abnormalities. The presence of serious mandibular hypoplasia or soft tissue deficiency should alert the clinician to a high possibility but not a high severity of facial paralysis.

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.

Usefulness of reconstructed 3D images for cochlear implantation in a case with a facial nerve anomaly.

Facial nerve anomalies are a potential problem in patients with cochleovestibular malformations. A case of cochlear implant (CI) surgery in the presence of intra-temporalbone facial nerve bifurcation is presented. During the first surgery, the facial nerve bifurcation obscured the promontory and round window. It was difficult to perform cochleostomy because of the lack of landmarks of the basal turn of the cochlea, and the first surgical attempt at cochleostomy was abandoned. A repeat CT scan was performed after the first surgery with reconstructed 3D images of the temporal bone and the cochlea, and then the cochlea was successfully opened at revision surgery. Reconstructed 3D CT images were very useful to identify the site of cochleostomy in this case with such difficult temporal bone anatomy.