Granulomatosis with polyangiitis presenting as facial nerve palsy in a teenager.

Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e.g. fever, malaise), or when other organs are involved (kidney, lungs, etc.). We present an interesting case of a 14-year-old female with eight-weeks of bilateral otalgia, unilateral facial nerve palsy, decreased appetite, and fatigue refractory to steroid, anti-viral, and antibiotic treatment ultimately diagnosed with GPA.

Steroids for treatment of sudden sensorineural hearing loss: a meta-analysis of randomized controlled trials.

OBJECTIVE: To systematically review the available evidence regarding steroid treatment for sudden sensorineural hearing loss (SSHL) and to update prior analyses when possible. DATA SOURCES: OVID Medline. REVIEW METHODS: An electronic database search (OVID Medline) was performed with the objective of identifying all randomized controlled trials examining the use of steroids for the treatment of SSHL. The search was limited to English-language publications between January 1980 and June 2013. Reference lists were examined for additional articles. RESULTS: A total of 15 articles including 1,166 subjects were included in three separate analyses. Three articles (181 subjects) were included in the steroid versus placebo analysis, which resulted in an odds ratio of 1.52 (95% confidence interval [CI]: 0.83-2.77). Six articles (702 subjects) were included in the systemic versus intratympanic steroids analysis, which resulted in an odds ratio of 1.14 (95% CI: 0.82-1.59). Six articles (283 subjects) were included in the salvage treatment analysis, which resulted in an odds ratio of 6.04 (95% CI: 3.26-11.2). CONCLUSIONS: A meta-analysis of randomized controlled trials does not support the use of steroids over placebo in this condition, a finding consistent with previous analyses. Although systemic or intratympanic steroid administration does not have a significant treatment effect, steroids for salvage treatment of patients failing traditional therapy appear to have an effect. However, this result should be interpreted with caution given the poor quality of component trials. Implications for clinical practice and future trial design are discussed.

Pleomorphic rhabdomyosarcoma presenting as a hypopharyngeal mass.

BACKGROUND: Rhabdomyosarcoma (RMS) is a rare malignancy derived from skeletal muscle with approximately 40% of cases involving the head and neck. The pleomorphic variant, however, most commonly occurs in the extremities and has never, to our knowledge, been described in the pharynx. METHODS: A 46-year-old man with no significant medical history presented to the emergency department complaining of hemoptysis. A CT scan of the head and neck revealed a hypopharyngeal mass originally favored to be a benign process. RESULTS: Operative endoscopy revealed a previously unseen mucosal ulceration, and subsequent biopsy resulted in the final diagnosis of pleomorphic RMS. CONCLUSION: Although quite rare, RMS should be considered in the differential diagnosis of a hypopharyngeal mass.