Objective: To investigate the clinical classification and surgical outcomes of congenital middle ear malformations accompanied by facial nerve anomalies. Methods: A retrospective cohort study was conducted to analyze clinical data of patients with congenital middle ear malformations and concomitant facial nerve anomalies who received treatment in the Department of Otolaryngology, Zhujiang Hospital, Southern Medical University, between January 2021 and December 2024. Facial nerve anomalies were categorized into four distinct types: partial obstruction of the oval window, complete obstruction of the oval window, aberrant course over the promontory, and branching anomalies. Ossicular chain malformations were classified in accordance with the Teunissen classification system. Individualized hearing reconstruction strategies were implemented based on the specific anatomical characteristics of the anomalies. All patients were followed up for 3 to 24 months. Postoperative therapeutic efficacy was evaluated by comparing preoperative and postoperative mean air conduction thresholds and air-bone gaps (ABG), while monitoring surgical complications. Results: Thirteen patients (17 ears) aged [M (Q1, Q3)] 15.0 (11.5, 21.0) years were included, with eight males and five females. Facial nerve anomalies were categorized as follows: partial obstruction of the oval window in six ears (three cases of Type Ⅰ, three cases of Type Ⅲ), complete obstruction of the oval window in three ears (one case of Type Ⅰ, two cases of Type Ⅲ), aberrant course over the promontory in six ears (all Type Ⅳ), and branching anomalies in two ears (both Type Ⅳ). Hearing reconstruction modalities employed included: incus-stapedial piston placement following vestibular fenestration in nine ears, malleus-stapedial piston placement following vestibular fenestration in two ears, partial ossicular replacement prosthesis (PORP) implantation in three ears, total ossicular replacement prosthesis (TORP) implantation in one ear, stapes mobilization in one ear, and laser-assisted ossicular chain release in one ear.Statistical analysis revealed that postoperative mean air conduction thresholds [(33.8±15.2) dB HL vs (60.1±13.8) dB HL, P<0.001] and mean ABG [(14.6±6.9) dB vs (37.2±9.2) dB, P<0.001] were significantly reduced compared with preoperative values. Postoperative ABG≤20 dB was achieved in 15 ears (88.2%). Postoperative complications were limited to transient vertigo in five cases and taste disturbance in two cases. No cases of facial paralysis, sensorineural hearing loss, tympanic membrane perforation, or tinnitus were observed. The median follow-up duration was 6.0 (5.5, 20.0) months. Conclusion: Individualized hearing reconstruction strategies based on the classification of facial nerve and ossicular chain anomalies can effectively protect the facial nerve and achieve significant hearing improvement, demonstrating favorable clinical outcomes.

Cushing disease (CD) secondary to adrenocorticotropin (ACTH)-secreting pituitary macroadenomas is uncommon, accounting for 10% to 15% of cases. We describe 3 patients from South Africa who presented with overt clinical features of hypercortisolism and considerable systemic complications, including hypertension, diabetes mellitus, and osteoporosis. Biochemical testing confirmed ACTH-dependent hypercortisolism with hypopituitarism and mild hyperprolactinemia consistent with stalk effect. Magnetic resonance imaging demonstrated pituitary macroadenomas, measuring 10 mm or greater, with parasellar extension. Mass effect was observed in 1 patient resulting in cranial nerve III palsy, while the others had visual field deficits secondary to optic chiasm compression. All patients underwent endoscopic transsphenoidal surgery, with 1 achieving remission and 2 developing recurrence. These cases highlight that ACTH-secreting macroadenomas can present with severe systemic disease and significant mass effect, and emphasize the importance of lifelong follow-up.

BACKGROUND Oculomotor neuropathy represents a very small proportion of cranial nerve palsies in children. It may result from both congenital and acquired causes. Acquired causes include vasculopathies, inflammation, infections, and neoplasms. However, only a limited number of studies in adults and children have identified post-infectious isolated oculomotor neuropathy, and even fewer have investigated the therapeutic role of corticosteroids in its treatment. CASE REPORT A previously healthy 4-year-old Saudi boy presented to the emergency department with acute onset of substantial drooping of the left upper eyelid, diplopia, and subjective low-grade fever. There were no symptoms of increased intracranial pressure and no history of head trauma. Physical examination revealed complete left-sided oculomotor neuropathy and anisocoria, without involvement of other cranial nerves or additional focal findings. Neuroimaging (computed tomography, contrast-enhanced magnetic resonance imaging, magnetic resonance angiography, and magnetic resonance venography), cerebrospinal fluid analysis, and opening pressure assessment showed normal results. Other serological findings were unremarkable. No underlying etiology was identified, and a post-infectious immune-mediated process was suspected. Oral prednisolone was initiated at a dose of 2 mg/kg/day for 5 days, followed by a 5-day taper. Follow-up at 3 months demonstrated complete resolution. CONCLUSIONS This case of isolated unilateral oculomotor neuropathy after presumed infection demonstrated complete clinical recovery after a short course of oral corticosteroids. Although a causal relationship cannot be established, such treatment may be cautiously considered in children who present with acute oculomotor neuropathy and a possible post-infectious process after an extensive, inconclusive workup. Further studies are needed to confirm this observation.

B-cell maturation antigen-targeting chimeric antigen receptor (CAR) T-cell therapies have demonstrated remarkable efficacy in relapsed/refractory multiple myeloma (RRMM). However, emerging non-immune effector cell-associated neurotoxicity syndrome (non-ICANS) neurologic events-including cranial nerve palsy (CNP) and immune effector cell (IEC)-parkinsonism (also called movement and neurocognitive toxicity)-warrant vigilance. This review summarizes clinical findings from the CARTITUDE trials, which evaluated ciltacabtagene autoleucel in > 300 patients with RRMM. CNP occurred in 6.3% of patients, with a lower incidence in more heavily pretreated patients (≥ 3 prior lines of treatment [pLOT]: 3% vs. 1-3 pLOT: 9%). Median CNP onset was day 22 postinfusion; most cases were low grade, and 90% recovered completely. IEC-parkinsonism involves motor, cognitive, and personality changes; lower incidence was observed in less heavily pretreated patients (1% vs. 6%). Median time to onset was 56 days postinfusion; however, with greater awareness, IEC-parkinsonism may be recognized earlier. Emerging data suggest that early, aggressive intervention may result in better outcomes. High CAR-T cell expansion is associated with increased risk of CNP and IEC-parkinsonism. In the early postinfusion period (days 10-28), absolute lymphocyte count (ALC) strongly correlates with circulating CAR-T cell levels and could help identify patients at increased risk of neurologic events. Prophylactic interventions, including a short course of steroids, triggered by elevated ALC before symptom onset, are under investigation. Education of patients, caregivers (including family), local oncologists, and neurologists seeing patients outside CAR-T infusion centers is essential to facilitate timely recognition, referral to the CAR-T center, and management.

Extranodal natural killer/T-cell lymphoma (ENKTL) is a rare and aggressive form of non-Hodgkin lymphoma. It most commonly affects the nasal cavity and sinuses. While only few of cases of third cranial nerve palsy have been reported in association with diffuse large B-cell lymphoma and Burkitt lymphoma, its occurrence as a presenting feature of ENKTL is exceptionally rare. Here, we present a patient with isolated third cranial nerve palsy as the initial manifestation of relapsed ENKTL. A 67-year-old woman with a history of ENKTL in remission was admitted with bilateral periorbital swelling, nasal purulent discharge, and congestion following nasal reconstruction. She was diagnosed with preseptal cellulitis and sinusitis and subsequently treated with IV antibiotics. She later developed a right third nerve palsy with pupillary involvement. A CT angiogram ruled out an aneurysm, however brain MRI showed perineural spread affecting the cavernous sinus. Further cranial nerve involvement prompted a biopsy of the sinus, confirming ENKTL. A PET scan was done. No uptake in the brain, sinuses, or the rest of the body was observed. A bone marrow biopsy was subsequently performed revealing marrow involvement with ENKTL, suggesting metastatic spread beyond the sinuses. Unfortunately, the patient developed sepsis and subsequently passed away. This case emphasizes the importance of early consideration of malignancy who present with atypical neurological symptoms. Given the risk of false-negative imaging results, a multimodal approach is essential. This should include high-resolution MRI coupled with neuroradiological review, biopsy with histopathological analysis when feasible, FDG PET scan and lumbar puncture with CSF analysis.

Recurrent painful ophthalmoplegic neuropathy (RPON) is characterized by recurrent unilateral headache associated with ipsilateral ophthalmoplegia due to palsy of one or more ocular cranial nerves, including the oculomotor, trochlear, and abducens nerves. Herein, we report a case of RPON with frequent recurrence over a short period, wherein further recurrence was prevented using high-dose riboflavin. The patient was a 6-year-old boy who presented with a 3-day history of headache, right eye pain, vomiting, and right oculomotor nerve palsy. He had a similar episode 3 years earlier, which resolved spontaneously after 1 month. Initial treatment with intravenous methylprednisolone alleviated eye pain, ocular motility disorder, and ptosis, but not the dilated pupil. Despite steroid treatment, the patient experienced frequent relapses triggered by infectious diseases, resulting in side effects such as moon face and weight gain. High-dose riboflavin (200 mg/day) was initiated as prophylaxis after other treatments, including lower doses of riboflavin and sodium valproate, failed to prevent relapses. The patient remained relapse-free for 9 months after increasing the riboflavin dose. Although it remains unclear whether high-dose riboflavin alone or in combination with valproic acid was effective, this case suggests that high-dose riboflavin might be an effective prophylactic treatment for RPON.