[Analysis of hearing effects of three ossicular reconstruction methods in 123 cases of atticotomy surgery].

Retrospective analysis of clinical data of 123 patients with atticotomy, exploring the clinical characteristics of patients undergoing atticotomy and the efficacy of hearing reconstruction methods. 123 patients with atticotomy were divided into three groups according to the ossicular chain treatment method: preservation of the ossicular chain group（37 cases）, cartilage elevation of stapes group（49 cases）, and PORP group（37 cases）. The clinical characteristics of patients with atticotomy, preoperative and postoperative hearing levels of the three groups of patients, and postoperative complications were analyzed.

[Selection of inner ear fenestration strategy and surgical effect of patients with oval window atresia accompanied by facial nerve aberration].

To summarize the clinical features and postoperative efficacy of patients with oval window atresia accompanied by facial nerve aberration. The clinical data of patients with congenital middle ear malformation with facial nerve aberration admitted to our hospital from January 2015 to March 2023 were retrospectively analyzed. There were 97 cases (133 ears) in total.

Two novel compound heterozygous HOXB1 variants in congenital facial palsy: A case report and a brief review of the literature.

Hereditary congenital facial palsy (HCFP) is a medical condition caused by dysfunction of the seventh cranial nerve. HCFP is characterized by feeding difficulties and dysmorphic features in the orofacial region. In some cases hearing loss, strabismus, limb malformations, and musculoskeletal defects may be associated.

Overview of Unilateral and Bilateral Pediatric Facial Paralysis: Workup, Treatment, and Frontiers.

Pediatric facial nerve paralysis can present significant challenges based on its various etiologies, unique approach to treatment options, and overall outcomes. It can impact both the child and parent when regarding function, appearance, and psychosocial implications. The etiology of facial nerve palsy can include congenital, traumatic, iatrogenic, and idiopathic causes.

Exploring the Impact of Genetics in a Large Cohort of Moebius Patients by Trio Whole Exome Sequencing.

Moebius syndrome (MBS) is a rare congenital disorder characterized by non-progressive facial palsy and ocular abduction paralysis. Most cases are sporadic, but also rare familial cases with autosomal dominant transmission and incomplete penetrance/variable expressivity have been described. The genetic etiology of MBS is still unclear: de novo pathogenic variants in and are reported in only a minority of cases, suggesting the involvement of additional causative genes.

Postoperative multimodal rehabilitation for spontaneous smile restoration in severe Moebius syndrome: A case report and a short literature review.

Moebius syndrome (MS) is a rare congenital disorder characterized by bilateral facial paralysis, significantly impacting an individual's ability to convey emotions. The lengthening temporalis myoplasty (LTM) surgery is a crucial palliative intervention that can restore the smile in patients with MS by transferring the functional temporalis muscle to the paralyzed facial commissure. However, successful outcomes are closely tied to postoperative functional rehabilitation.

Clinical features and management strategies concerning auditory canal duplication anomalies in children with congenital first branchial cleft anomalies.

Objective: This study aimed to summarise the clinical features and management strategies concerning auditory canal duplication anomalies in children with congenital first branchial cleft anomalies (CFBCAs), and to provide guidance for precise treatment.

Methods: We retrospectively analysed 84 children with CFBCAs who had complete data, diagnosed between December 2018 and February 2024.

Results: All the lesions identified were located around the external auditory canal or near the mandibular angle, manifested as pinhead-sized perforations in 10 cases, painless masses in 18 cases, recurrent swelling and pain with purulent discharge in 52 cases, and otorrhea in 4 cases.

Diagnosis and Management of Unexplained Conductive Hearing Loss With Intact Tympanic Membrane: A Systematic Review.

To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 642 articles were retrieved from databases such as PubMed, Embase, Web of Science, and Cochrane.

Management of Petrous Bone Cholesteatoma: The Gruppo Otologico Experience.

Background: The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare incidence accounting for only 4-9% of petrous bone lesions. Lesions of petrous bone represent a real surgical challenge due to its complex relationship with critical neurovascular structures.

Uncommon Bilateral Idiopathic Facial Nerve Palsy (Bell's Palsy) in an Infant: An Atypical Age and Clinical Challenges Explored: A Case Report.

Our case study explores Bell's palsy, a rare lower motor neuron facial nerve paralysis, particularly rare in newborns. While often idiopathic, it can stem from congenital or developmental factors. Unilateral facial paralysis is more prevalent, with Bell's palsy accounting for more than 50%, while bilateral facial paralysis is exceptionally rare.

Facial Nerve Pathology in Children.

Facial nerve pathology in children has devastating functional and psychosocial consequences. Facial palsy occurs less commonly in children than adults with a greater proportion caused by congenital causes. Most pediatric patients have normal life expectancy and few comorbidities and dynamic restoration of facial expression is prioritized.

[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.

Adult-onset congenital cholesteatoma in the hypotympanum initially presenting as Bell's palsy: A case report.

Introduction: Cholesteatoma is a rare disease characterized by the accumulation of keratinized squamous epithelial cells in the middle ear or mastoid cavity. Vertigo and facial palsy, which are rare complications, may indicate erosion into the semicircular canals or the fallopian canal.

Patient Concerns: A 40-year-old woman presented to our clinic with progressive right-sided hearing loss over 5 years (primary concern).

A Rare Case of Isolated Congenital Facial Nerve Aplasia in an Infant.

Facial nerve aplasia is an exceptionally rare condition, with only a few reported cases in the existing medical literature. Congenital facial palsy (CFP) is characterized by the clinical manifestation of facial paralysis involving the seventh cranial nerve, either evident at birth or shortly thereafter. This condition is categorized based on various parameters, including the presence of trauma or developmental origins, unilateral or bilateral involvement, and whether the paralysis is complete or incomplete.

Dangers and therapeutic difficulties of intracranial hemangioma in infants: A CARE case report.

Introduction: Description of neurological complications induced by intracranial hemangioma in infants and by the initiation of beta-blocker treatment (propranolol).

Observation: A 2-month-old infant was referred for grade 5 non-congenital unilateral peripheral facial palsy. Work-up revealed ipsilateral profound hearing loss and two intracranial hemangiomas: one in the ipsilateral internal auditory canal (IAC), the other in the cerebellum opposite the nodule of vermis.

Case report: Identification of a novel variant p.Gly215Arg in the gene causing Moebius syndrome.

Moebius Syndrome (MBS) is a rare congenital neurological disorder characterized by paralysis of facial nerves, impairment of ocular abduction and other variable abnormalities. MBS has been attributed to both environmental and genetic factors as potential causes. Until now only two genes, and have been identified to cause MBS.

Crocodile tear syndrome treated with lacrimal gland incobotulinum toxin A injection: a report of two cases.

Crocodile tear syndrome (CTS) is a late complication of facial nerve palsy characterized by unilateral lacrimation in response to gustatory stimulation. We present 2 cases of patients diagnosed with CTS after recovering from unilateral idiopathic facial nerve palsy. Both patients underwent transconjunctival lacrimal gland incobotulinumtoxinA injection, with doses of 5-16 units.

Physiopathologic Bases of Moebius Syndrome: Combining Genetic, Vascular, and Teratogenic Theories.

Moebius syndrome (MBS) is a congenital cranial dysinnervation disorder (CCDD) characterized by a bilateral palsy of abducens and facial cranial nerves, which may coexist with other cranial nerves palsies, mostly those found in the dorsal pons and medulla oblongata. MBS is considered a "rare" disease, occurring in only 1:50,000 to 1:500,000 live births, with no gender predominance. Three independent theories have been described to define its etiology: the vascular theory, which talks about a transient blood flow disruption; the genetic theory, which takes place due to mutations related to the facial motor nucleus neurodevelopment; and last, the teratogenic theory, associated with the consumption of agents such as misoprostol during the first trimester of pregnancy.

Expanding the Phenotype of Hereditary Congenital Facial Paresis Type 3.

The gene encodes a homeobox transcription factor pivotal in the development of rhombomere 4. Biallelic pathogenic variants in this gene are associated with congenital facial paresis type 3 (HCFP3). Only seven single nucleotide variants have been reported in the literature to date.