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).