Background: Muckle-Wells syndrome (MWS) is a rare auto-inflammatory disease characterized by the presence of recurrent urticaria, deafness and amyloidosis. Progressive sensorineural hearing loss (SNHL) is reported to occur in up to 85% of patients occurring in the second and third decades and as early as the first decade in patients with a more severe phenotype, thus potentially having a significant impact on a child's development. IL-1 inhibitors, such as Anakinra, have been described to improve systemic inflammation, and stabilize or improve hearing status as well. However, complete resolution of hearing loss has been rarely reported. The objective of this article is to highlight the clinical presentation of a pediatric patient with a severe form of MWS and report on the complete resolution of SNHL with the use of Anakinra. Case presentation: A 3-year-old boy was referred to our hospital to assess for the possibility of MWS given a history of hives and recurrent episodes of fever with a family history of MWS in his mother. Of note, the patient's history was significant for conductive hearing loss, speech delay, as well as recurrent acute otitis media episodes. Genetic analysis was performed and diagnosis of MWS was confirmed due to the presence of a NLRP3 gene mutation. Further work-up demonstrated the presence of papilledema and elevation of systemic inflammatory markers for which Canakinumab was initiated. Despite initiation of this treatment, audiogram evaluation demonstrated a new right-sided SNHL. Lumbar puncture also revealed aseptic meningitis. Canakinumab was eventually discontinued and Anakinra initiated. Within 7 months of treatment with Anakinra at 5 mg/kg sc daily, resolution of the SNHL was observed. With further escalation of the Anakinra dose, there was also complete resolution of the aseptic meningitis. Conclusions: Progressive hearing loss is a significant finding in patients with MWS. Early screening as well as initiation of Anakinra can lead to complete resolution of SNHL even in a patient with a severe spectrum of MWS. However, as this case demonstrates, longer treatment duration and higher doses of Anakinra may be required to achieve this.

Background: Intraoperative parathyroid hormone level (IOPTH) is withdrawn during parathyroidectomy to confirm the success of the procedure. Recently, the importance of IOPTH has been put to question. The purpose of this study is to determine whether IOPTH is necessary for all patients undergoing parathyroidectomy in the presence of frozen section. Materials and methods: A cohort study of parathyroidectomies was performed in three university affiliated hospitals during 2007-2012. The patients were divided into two groups. Group 1: Patients with two preoperative concordant imaging localizing a hyperactive gland. Group 2: Patients without two concordant imaging. A comparison of benefit of IOPTH was carried out. Frozen section results were also analyzed to determine sensitivity and predictability of a parathyroid adenoma. Results: The study considered 221 patients having parathyroidectomies for primary hyperparathyroidism (PHPT). Of them, 10 were excluded due to incomplete data. Among the remaining, 186 had 2 concordant imaging preoperatively localizing an adenoma. 93.5% of whom were found intraoperatively in that location. IOPTH was not found to be of importance in 98.92% of the preoperative localized adenomas in the presence of frozen section. IOPTH added an estimate of 30.9 minutes on average to the surgery time. Conclusion: This study demonstrates that the added operating time associated with IOPTH may not be justified for patients undergoing parathyroidectomy who have 2 concordant imaging preoperatively in the presence of frozen section. This study suggests a simple algorithm, The McGill Parathyroid Protocol (MPP), to help in approaching PHPT patients undergoing parathyroidectomy.

Background: Ultrasound guided fine-needle aspiration (USFNA) biopsy of thyroid nodules often gives a result of indeterminate pathology, placing thyroid specialists in difficult management situations. The aim of this study is to evaluate the incidence of malignancy in patients undergoing surgery and to correlate these results with the McGill Thyroid Nodule Score (MTNS). Methods: We performed a retrospective study comparing USFNA results, MTNS and histopathology of patients undergoing thyroid surgery between 2010 and 2012. Pre-operative USFNA results were divided into three subgroups: benign, indeterminate and suspicious for/malignant. The indeterminate USFNA subgroup comprised of Bethesda type III (atypia of undetermined significance) and Bethesda type IV (follicular neoplasms, including Hurthle cell neoplasms) lesions. Post-operative histopathology was divided into benign or malignant groups. Results: Of the 437 patient charts reviewed, 57.0% had an indeterminate USFNA biopsy. Within the indeterminate group, the malignancy rate was 39.8%. For indeterminate USFNA, the median MTNS was 7 (32% risk of malignancy) for benign nodules and 9 (63% risk of malignancy) for malignant nodules on post-operative histopathology ( p < 0.05). Conclusion: The rate of malignancy in operated patients with an indeterminate USFNA result was 39.8%. The MTNS can be of value to thyroid specialists in pre-operative decision-making when dealing with an indeterminate result of a thyroid nodule on USFNA.

Objectives: The reported prevalence of vestibular dysfunction after cochlear implantation (CI) is varies between different scientific papers. The aim of this study is to determine the reported post-implantation outcome in terms of dizziness, and to measure its impact on quality of life using the Dizziness handicap inventory (DHI). Methods: This was a prospective questionnaire based study of postoperative cochlear implant patients. The questionnaire assessed the type and onset of dizziness in addition to the DHI. Results: 122 patients were recruited in this study, which is the largest sample size in the literature reported so far. Dizziness was evident in 45.9% of the population post-CI and in 27% pre-CI. The commonest subtype of the dizziness was unsteadiness followed by lightheadedness. The dizziness was mild in most of the patients. Conclusion: Although mild, dizziness is a common complaint post-cochlear implantation. An understanding of symptoms helps counsel patients preoperatively.