Rationale:Hypertrophic pachymeningitis (HP) is an uncommon, life-threatening disease that is seen in elderly patients with antineutrophil cytoplasmic antibody (ANCA) positivity. Proteinase-3 (PR3)-ANCA-positive HP has not been reported in adolescents. Here, we report the first case of adolescent PR3-ANCA-positive HP successfully treated with immunosuppressive therapy.Patient concerns:A 14-year-old female presented with fullness and pain in her right ear unresponsive to antibiotics. Laboratory tests showed an elevated C-reactive protein and PR3-ANCA positivity. Computed tomography and magnetic resonance imaging revealed mastoiditis in the right temporal bone. Surgical biopsy revealed severe fibrosis and prominent inflammatory-cell infiltration. She received prednisolone and methotrexate therapy, and then underwent a right mastoidectomy. Five months later, she developed headache, dysarthria, and multiple cranial nerve palsies. Further imaging revealed enhancement and thickening of the right hemispheric dura.Diagnosis:PR3-ANCA-positive HP.Interventions:She was successfully treated with steroid pulse therapy for 3 days, followed by high doses of prednisolone and intravenous cyclophosphamide.Outcome:The treatment resulted in significant improvement of her symptoms, laboratory data, and radiologic findings.Lessons:PR3-ANCA-positive HP can present not only in the elderly, but also in adolescence, and prompt diagnosis and treatment with immunosuppressive therapy is vital.

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Objectives: In the management of vestibular schwannoma (VS), patient-perceived outcomes should be given as much attention as physician-reported objective outcomes. Concomitant symptoms can be annoying for sufferers, lowering quality of life (QoL). However, it is difficult to determine the degree to which these symptoms lower QoL using only objective measures. The Penn Acoustic Neuroma Quality of Life Scale (PANQOL) is a validated disease-specific questionnaire commonly used to assess QoL in VS patients. It was developed and published in English. In this study, we translated the PANQOL questionnaire into Japanese and validated its reliability and utility. We also evaluated patient factors that could influence PANQOL scores. Study Design: Cross-sectional study. Methods: Seventy-two VS patients monitored conservatively were included in this study. They were assessed using the Japanese versions of PANQOL (PANQOL-J) and questionnaires about specific symptoms related to VS. The PANQOL questionnaire was translated into Japanese according to the accepted standards. The reliability and utility of the PANQOL-J questionnaire, as well as the relationship with VS-specific symptoms, were statistically analyzed. Results: The mean total score was 78.7. Almost all of its domains had good internal consistency. Most PANQOL-J domains were related to those of the Short Form-36, with the exception of facial dysfunction domain. Scores of all PANQOL-J domains were comparable to scores of other questionnaires related to the VS-specific symptoms. Our analyses indicated tinnitus or facial palsy might affect the PANQOL-J total score. Conclusions: We confirmed the validity of the Japanese version of PANQOL questionnaire and its reliability and utility in the management of VS symptoms concerning facial function.

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Background Inflammatory pseudotumors (IPTs) are rare, idiopathic, and inflammatory lesions that are histopathologically benign. Here, we present three cases of labyrinthine destruction caused by an IPT. Methods The first patient was a 74‐year‐old male with a mass lesion extending from the inner ear to the external ear canal. The second patient was a 62‐year‐old female with a foliated polycystic lesion in the petrous bone on the dorsal side of the left internal auditory canal. The third patient was a 68‐year‐old female with a mass extending from the inner ear to the middle ear, destroying the semicircular canal and cochlea. Results In two cases, we performed surgical resection successfully with no recurrence. In the other case, the lesion showed shrinkage after chemotherapy for colorectal cancer incidentally found during the examination process. Conclusion Surgical technique and indication for IPT should be based on the location and function of the lesion. In addition, there is room to consider pharmacotherapy as a treatment option for IPT of the temporal bone. Level of Evidence 4.