scholarly journals Facial nerve paresis in the course of masked mastoiditis as a revelator of GPA

Author(s):  
Joanna Marszał ◽  
Anna Bartochowska ◽  
Randy Yu ◽  
Małgorzata Wierzbicka

Abstract Purpose The aim of this study was to present a series of 6 patients with facial nerve palsy and masked mastoiditis which constituted as revelators of localized granulomatosis with polyangiitis (GPA) and to evaluate the utility of the ACR/EULAR 2017 provisional classification criteria for GPA in such cases. Methods Study group included 58 patients with GPA. Cases with facial nerve palsy and masked mastoiditis were thoroughly analyzed. Results The mean age of patients was 37 years. All manifested unilateral facial nerve palsy and hearing loss, while only 2 reported aural complaints suggesting inflammatory cause of the disease. All cases were qualified for surgical intervention. Intraoperative findings were similar: granulation tissue in tympanic cavity and/or pneumatic spaces of the mastoid process. Only 50% of histopathological results suggested vasculitis. In all cases, elevated levels of antineutrophil cytoplasmic antibodies (ANCA) against peroxidase 3 (PR3-ANCA) were determined. Two patients presented rapid progression of the disease and died within 1 week and 2 months, respectively. Four other patients manifested gradual improvement of hearing and facial nerve function after treatment. Conclusion GPA should be included into differential diagnosis in all cases of persistent facial nerve palsy especially when otological symptoms coexist. Even localized GPA could be very aggressive, revelating generalized form of the disease. Rapid systemic treatment of GPA can protect hearing and facial nerve from permanent severe dysfunction. The ACR/EULAR 2017 provisional classification criteria for GPA seem to be valuable tool in diagnosing ENT patients with localized otological form of the disease.

2014 ◽  
Vol 29 (1) ◽  
pp. 20-22
Author(s):  
Mee Ling Tang ◽  
Govindaraju Revadi ◽  
Raman Rajagopalan ◽  
Sushil Brito-Mutunayagam

Objective: To report a case of vertebrobasilar dolichoectasia presenting with ipsilateral facial nerve paresis and concomitant severe sensorineural hearing loss.   Methods:                Design: Case Report                Setting: Secondary Government Hospital                Patient: One   Results:  We report a case of vertebrobasilar dolichoectasia with concomitant ipsilateral facial nerve paresis and severe sensorineural hearing loss in an elderly female.  She presented to us with left facial nerve palsy House-Brackmann Grade III with prior history of ipsilateral sensorineural hearing loss.  MRI of the brain showed normal inner ear structures, but revealed a dilated and tortuous basilar artery with compression on the left medulla and possible branches of anterior inferior cerebellar artery as it coursed superiorly, and possible partial thrombosis of proximal basilar artery.    Conclusion:  Concommitant facial nerve paresis and sensorineural hearing loss can be the clinical presentations of this rare but important condition.  MRI is vital in diagnosing vertebrobasilar dolichoectasia.   Keywords: Vertebrobasilar dolichoectasia, facial nerve palsy, sensorineural hearing loss, basilar artery


2021 ◽  
Vol 10 (18) ◽  
pp. 1353-1355
Author(s):  
Shruti Chaudhary ◽  
Gyanavelu Injeti ◽  
Amar Taksande ◽  
Revat Meshram ◽  
Amol Lohkare

Congenital facial palsy (CFP) is facial palsy of the seventh cranial nerve present at birth. It is commonly believed to be either developmental or originally acquired. Facial palsy of developmental origin is associated with other anomalies including ear, eye or cardiac anomalies. But it is rarely associated with polydactyly. We report a 10- month-old female infant who had right CFP with bilateral microtia and polydactyly. Congenital facial nerve palsy unilaterally manifesting as weakness of entire face on one side due to infranuclear cause is a rare occurrence in paediatric population.1 The facial palsy of lower motor neuron type involves weakness of same side of muscles of face of one side. Facial nerve as it emerges out of cranium traverses through facial canal medial to mastoid process and divides into multiple branches. It supplies ear, autonomic fibres for lacrimal gland, salivary glands, and motor supply to the face. Any pathology, congenital or acquired causing compression of facial nerve in this pathway can lead to facial palsy. Microtia is a congenital condition that varies from minor structural defects to full ear absence in severity and may occur as an individual defect or as part of a syndrome. Polydactyly is the most common hereditary limb anomaly characterized by extra fingers or toes. Here, we report a case presented with bilateral microtia and right-side facial nerve palsy.


2017 ◽  
Vol 06 (02) ◽  
pp. 141-143 ◽  
Author(s):  
Mukesh Bhaskar ◽  
Rakesh Kumar ◽  
Sunil Singh ◽  
M. Meel

AbstractVestibular schwannomas are the most common cerebellopontine angle tumors. These tumors commonly present with ipsilateral dysfunction of acoustic, vestibular, trigeminal, and facial nerves. Vestibular schwannoma with involvement of contralateral facial nerve is very unusual, and whenever present, it is considered as a false localizing sign. It seems that displacement and distortion of the brainstem by the large mass lesion may lead to this atypical presentation. We report a case of vestibular schwannoma with contralateral abducens and facial nerve paresis.


2018 ◽  
Vol 4 (5) ◽  
pp. 369-371
Author(s):  
Rajashree U Gandhe . ◽  
Chinmaya P Bhave . ◽  
Avinash S Kakde . ◽  
Neha T Gedam .

2020 ◽  
Vol 6 (1) ◽  
pp. 1-5
Author(s):  
Daichi Fujii ◽  
Hikari Shimoda ◽  
Natsumi Uehara ◽  
Takeshi Fujita ◽  
Masanori Teshima ◽  
...  

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii352-iii352
Author(s):  
Dennis Tak-Loi Ku ◽  
Matthew Ming-Kong Shing ◽  
Godfrey Chi-Fung Chan ◽  
Eric Fu ◽  
Ping-Wa Yau ◽  
...  

Abstract INTRODUCTION Infantile glioblastoma is rare with poor prognosis. Recent molecular study for infantile hemispheric high grade glioma found its association with ALK/ROS1/NTRK/MET pathway. This suggested the potential use of targeted therapy for refractory / relapse patients. CASE: A newborn presented with apnea, CT brain showed intracranial haemorrhage. MRI then showed a left parietal tumour with bleeding and mass effect. Craniotomy achieved subtotal resection. Chemotherapy VCR/CPM alternating with CDDP/VP-16 was given for one year. Patient was stable with static residual tumour during chemotherapy. However patient developed status epilepticus two weeks after off treatment. MRI showed significant tumour progression which required 2nd & 3rd debulking surgery. Molecular assay by nanostring panel showed BRAF-KIAA1549 fusion. MEK inhibitor Trametinib was tried for 3 months and stopped as disease progression. Further molecular assay by RNASeq showed presence of ROS1 fusion (ZCCHC8-ROS1) while absent of BRAF fusion. Patient underwent 4th debulking surgery as impending herniation while waiting for the targeted therapy. It was complicated with right hemiplegia and facial nerve palsy postoperatively. Finally, ROS1 inhibitor Entrectinib was started 2 weeks later. It was well tolerated without significant adverse reaction. Patient made dramatic neurological recovery including improved facial nerve palsy, able to walk unaided and self feed. MRI brain 1 and 3 months after Entrectinib showed interval reduction in residual tumour. Patient is currently progression-free for 6 months. CONCLUSION Early molecular study for infantile glioblastoma is useful to guide novel therapy. Molecular result may varies between different panels or change over time, to be interpreted with caution.


2021 ◽  
Vol 14 (5) ◽  
pp. e242540
Author(s):  
Rahul Kumar Bafna ◽  
Suman Lata ◽  
Anusha Sachan ◽  
Mohamed Ibrahime Asif

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