Abstract

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

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