Case 2
A 15 years old male who had apparently normal hearing till the age of 14 years with normal speech and language development presented to us with bilateral fluctuating progressive hearing loss in both ears (left>right). There was no history suggestive of any vestibular symptoms apart from intermittent tinnitus. On clinical examination bilateral tympanic membrane was intact. Otoacoustic Emissions test showed refer in both ears suggestive of poor functioning of outer hair cells. Pure tone audiometry showed moderately severe sensorineural hearing loss in right ear and moderately severe mixed hearing loss in left ear (Figure. 3). Multiple pure tone audiograms were performed revealing progressive hearing loss in left ear. HRCT Temporal bone and MRI brain and IAC showed an abnormal cochlea with one and a half turns suggestive of Mondini malformation on the left side (Figure. 4 A-C).