Granulomatosis with Polyangiitis as a Cause of Sudden-Onset Bilateral Sensorineural Hearing Loss: Case Report and Recommendations for Initial Assessment.

Granulomatosis with polyangiitis (GPA) is a severe systemic vasculitis that commonly affects the paranasal sinuses, upper and lower respiratory tracts, and kidneys. GPA has also been associated with sensorineural hearing loss (SNHL), through inflammation of the cochlear apparatus. Early recognition, diagnostic laboratory evaluation, and appropriate treatment are essential to improve outcomes and achieve remission for patients with GPA. Here, we present a case of bilateral sudden sensorineural hearing loss (SSNHL) and distal symmetric polyneuropathy as the first presenting signs of GPA. A specific diagnostic work-up to rule out autoimmune inner-ear disease in patients with bilateral SSNHL is not clearly stated in the clinical practice guidelines from the American Academy of Otolaryngology-Head and Neck Surgery. The aim of this paper is to delineate an appropriate diagnostic work-up for patients with bilateral SSNHL when there is concern for autoimmune disease.

Pediatric Calvarial Bone Thickness in Patients With and Without Aural Atresia.

OBJECTIVE: To compare temporal bone thickness along a three-dimensional arc of potential osseointegrated implant sites for bone-anchored hearing aids in children with and without aural atresia using computed tomographic imaging (CT). STUDY DESIGN: Retrospective case review. SETTING: Tertiary children's hospital. PATIENTS: Children with or without aural atresia aged less than 11 years who had a temporal bone CT. INTERVENTION (S): Calvarial bone volume on CT was rendered in three-dimensional and thickness was reconstructed and measured at up to 12 defined sites along an arc of recommended implant sites. MAIN OUTCOME MEASURE (S): Determining whether a majority of observed potential implant sites have 2, 3, or 4 mm of bone thickness while controlling for age differences and atresia status. RESULTS: A total of 40 atretic (from 34 patients) and 34 control (from 34 patients) temporal bones were compared using CT. Likelihood ratio tests indicated that diagnosis did not have a statistically significant effect on whether patients reached thresholds of 2, 3, or 4 mm at most observed sites (p = 0.781, 0.773, and 0.529, respectively) when adjusting for age. For all children measured, 93% had >50% of measured points greater than or equal to 2 mm thick. CONCLUSION: Most children had greater than 2 mm of temporal bone thickness at >50% of the sites measured regardless of age or atresia diagnosis. The likelihood of reaching 4 mm of thickness at most sites improves with age. In unilateral patients, there was not a significant difference in thickness between affected and unaffected sides. There was also no significant difference in thickness when comparing patients with atresia to those without.

Acute Management of Paradoxical Vocal Fold Motion (Vocal Cord Dysfunction).

Paradoxical vocal fold motion disorder, also commonly termed vocal cord dysfunction, is a poorly understood cause of acute upper airway obstruction. Patients with paradoxical vocal fold motion frequently present to the emergency department (ED) with acute respiratory distress and stridor. Lack of familiarity with this disorder may lead to delayed diagnosis or misdiagnosis and unnecessary intubations or surgical airway procedures. Although long-term management of paradoxical vocal fold motion is well described, there is a paucity of information about acute evaluation and management. This article aims to summarize the ED presentation and management of paradoxical vocal fold motion.

Toxic shock syndrome and rhinosinusitis in children.

OBJECTIVE: To determine the association between toxic shock syndrome (TSS) and rhinosinusitis in children. DESIGN: Eighteen-year retrospective review of medical records. SETTING: Tertiary children's hospital. PATIENTS: A total of 76 patients were identified as having TSS. Twenty-three of them were also diagnosed as having either acute or chronic rhinosinusitis, with no other source of infection in 17 cases. INTERVENTIONS: Of the 23 patients with TSS and rhinosinusitis, 10 were admitted to the intensive care unit, 4 required pressors, and 6 received surgical intervention. Surgical intervention for sinus disease included bilateral antral lavage in 5 patients and bilateral maxillary antrostomy and ethmoidectomy in 1 patient. MAIN OUTCOME MEASURES: Patients with TSS and rhinosinusitis were identified using a rigorous set of definitions and detailed data pertaining to history, imaging studies, microbiologic studies, and hospital course. RESULTS: Correlation of the data revealed 4 patients who met the criteria for proven TSS and proven rhinosinusitis, 2 patients who met the criteria for probable TSS and proven rhinosinusitis, 7 patients who met the criteria for proven TSS and possible rhinosinusitis, and 3 patients who met the criteria for probable TSS and possible rhinosinusitis. CONCLUSIONS: Rhinosinusitis was found to be the primary cause of TSS 21% of the time in this series. Rhinosinusitis should be considered the primary cause of TSS when another site of infection has not been identified. Once the link is made, prompt otolaryngology consultation and sinus lavage should be considered.