Diagnosis and management of facial nerve palsy secondary to granulomatosis with polyangiitis - A systematic review.

OBJECTIVE: Granulomatosis with polyangiitis is associated with otolaryngologic complaints in 70-95 % of cases, with the most common being serous otitis media. In rare cases, patients may experience facial nerve palsy in conjunction with otologic or nasal symptoms; and, often, initially present to an otolaryngologist. It is important for healthcare professionals to be able to recognize the nuisances of facial nerve palsy as a potential presentation of granulomatosis with polyangiitis. STUDY DESIGN: Systematic review. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Protocol, PubMed and MED-LINE Databases were queried for articles published from January 2007 to December 2022 describing facial nerve palsy in the context of Granulomatosis with polyangiitis, formerly known as Wegener's Granulomatosis. The keywords included "facial nerve palsy", "facial palsy", "granulomatosis with polyangiitis", "Wegener's granulomatosis", "ANCA positive" in the title/abstract. All full-text articles available in English were screened, including single case presentations. Abstracts, commentaries, and publications deemed outside the scope of our study aims were excluded from review. After removal of duplicate articles, a total of 85 articles were screened. After applying inclusion and exclusion criteria, 14 articles were included in the review. RESULTS: There were a total of 28 reports of facial nerve palsy in the literature in patients who were eventually diagnosed with granulomatosis with polyangiitis. The patients' ages ranged from 14 to 68 years old. None of the patients had been previously diagnosed with GPA, and a majority of them presented initially with other otologic symptoms. Hearing loss was reported in 24 patients (86 %), otalgia was present in 11 patients (39 %), and otorrhea was present in 6 patients (21 %). Bilateral facial paralysis was reported in 10 patients in the literature (36 %). In total, 16 patients underwent surgery for facial paralysis: 6 tympanomastoidectomies, 4 mastoidectomies, 2 explorative tympanotomies. Surgery was generally considered ineffective in resolving facial weakness. All patients ended up receiving some combination of steroids and immunosuppressant, most commonly prednisolone and cyclophosphamide or rituximab, which was eventually transitioned to azathioprine for maintenance. Unlike auditory thresholds, which remained decreased in two patients, all patients recovered facial function following appropriate medical treatment of their vasculitis. CONCLUSIONS: Facial nerve paralysis in patients with granulomatosis with polyangiitis is a rare but treatable phenomenon. In patients with intractable otitis media, unresolving facial palsy, or a combination of otologic issues, it is important to consider GPA as a possible source. The prognosis for facial function appears to be excellent in patients who undergo appropriate treatment for vasculitis, but further studies are needed for confirmation.

Assessing Patient Barriers to Cochlear Implantation.

OBJECTIVE: Evaluate barriers that deter adult patients from following through with cochlear implantation. STUDY DESIGN: Retrospective chart review and phone survey. SETTING: Single tertiary referral center. PATIENTS: Between January 2019 and August 2021, 113 patients, without a previous cochlear implant (CI), were determined to be candidates for cochlear implantation. Thirty-eight (33.6%) patients deferred cochlear implantation. Survey response rate was 61.1% (22/36). INTERVENTION: None. MAIN OUTCOME MEASURES: Demographic, socioeconomic, otologic history, and comorbidity factors associated with deferment of cochlear implantation. Patient survey assessment of factors that had the greatest impact, rated on a scale of 1 to 10 (10 being the most impactful), on their decision to defer a CI. RESULTS: Out of the 113 patients who met inclusion criteria, 75 (66.3%) underwent cochlear implantation and 38 (33.6%) patients deferred. Comparing implanted versus deferred groups, there was no statistical difference in age (67.1 y versus 68.5 y; p = 0.690) or male sex (53.3% versus 57.9%; p = 0.692). The deferred group had higher mean neighborhood disadvantage state decile (5.3 versus 4.3; p = 0.064) and national percentile (73.0 versus 66.2; p = 0.106) scores, although neither were statistically significant. A greater proportion of the deferred group were not living independently at time of CI evaluation (13.2 versus 2.7%; p = 0.017). Fear of losing residual hearing was rated the highest among the survey respondents (mean rating of 5.1), followed by general medical health (4.9) and cost and financial concerns (3.6). CONCLUSION: The greatest concern among the patients was the fear of losing residual hearing. Spending greater time educating patients about the success and failure rates of cochlear implantation may reduce patient hesitancy with implantation.