Dr. RAJESH PATTEBAHADUR

Dr. PUJA HINGORANI – BANG, DR. PURVASHA SINGH NARANG, DR. KANISHK SINGH

Abstract

A 13 year old male child was referred from Pediatrics department with complaints of blurring of vision, outward deviation of right eye, nystagmus and hearing loss. On presentation, best corrected visual acuity OD was finger counting at 1 meter and OS 6/36 (Snellen’s) with accurate projection of rays. On gross examination, bluish hazy corneas with manifest latent nystagmus and right eye exodeviation were seen.On slit lamp examination, corneal posterior stromal and endothelial oedema was observed. Corneal diameter was approx. normal. Rest anterior & posterior segment examination was within physiological limits.

The patient was also referred to ENT department ,comprehensive work-up was done & brain stem evoked potential was performed showing right ear moderate- severe sensorineural hearing loss and left ear moderate hearing loss. The trial for hearing aid was advised . on the basis of an endothelial abnormality along with hearing loss, thus, the diagnosis of Harboyan Syndrome was made.

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