RFP Journal of ENT and Allied Sciences

Introduction: Heterotopic Brain and Encephaloceles of middle ear usually present with symptoms of ear discharge and hearing loss. Some patients have additional symptoms of headache, abnormal ringing sensation and vertigo. Radiology may or may not show a communication with central nervous system in encephaloceles and the bony defect may be unnoticeable or attributed to the thinning of bone due to chronic otitis media. On computerised tomography, no distinction may be made between fluid, granulations, cholesteatoma and other space occupying lesions. Morphologically, it may be identified grossly if it presents with its characteristic pink colour and typical convoluted cerebriform pattern (CCP).  Methods: A 26 years female presented to us with unilateral ear discharge since birth and unilateral hearing loss on ipsilateral side for last 18 months. Clinically, radiologically and morphologically it mimicked chronic otitis media with cholesteatoma with no pre-operative and intra-operative distinction from the later. However, the coronal section at the level of anterior tegmen showed a small defect of the tegmen. T2 weighted MRI confirmed presence of brain tissue in middle ear and mastoid cavity. Results: Combined Middle Cranial Fossa and mastoid approach were employed, unviable brain tissue was excised and sent for histopathology. Viable brain tissue was reduced and tegmen defect was closed with temporalis muscle, conchal cartilage and temporalis fascia. Histopathology revealed glial tissue, ependyma and choroid plexus without any cholesteatoma. Conclusion: Tympanic encephalocelesare very rare and may closely mimic chronic otitis media. It may become very difficult to differentiate between the two which may lead to unexpected complications andloss

Van der Hoeve’s syndrome is an all-round variant of Lobstein’s disease (late osteogenesis imperfecta) and it is characterized by the presence of osteoporosis, deafness and blue sclera. Earlier it was known as Adair Dighton Syndrome (Dighton, 1912). Vrolik in 1849 described this condition for the first time a in new-born infant who was having poorly ossified calvarium and called it osteogenesis imperfecta.The temporal bone may also be affected by this generalized abnormality of the skeletal system, which may result from a specific dominant gene abnormality. Here we discuss a case of 23yr old female with blue sclera presented to ENT OPD with chief complaint of bilateral decreased hearing from last 6yrs and unilateral ringing sensation in ear from 2yrs. Patient was also having generalised body weakness. In this article we discuss how we managed the case conservatively after proper blood and radiological investigations

Unilateral Palsy of Palate which is acquired in nature is a rare entity, mostly affecting children. Patient usually presents with sudden onset of nasal regurgitation of fluids, rhinolalia and asymmetric movement of palate. The diagnosis is established only after exclusion of major causes such as infection, trauma, tumor, brain stem lesion etc. it is termed idiopathic when all potential causes are ruled out. The disease is usually self limiting and recovery is excellent. We report a case of twelve year old boy who presented in opd with complaints of regurgitation of fluids from unilateral nostril and nasal speech. Clinical examination was done followed by MRI brain which was unremarkable. Final diagnosis, unilateral isolated temporary idiopathic palsy of palate was established. Patient had complete recovery within ten days of tapering methyl prednisolone therapy with no residual paralysis on follow up.