A Rare Association of Deaf Mutism, Syndactyly and Squamous cell carcinoma of the Temporal bone

摘要

Malignant tumours of the temporal bone are rare, the majority being squamous cell carcinoma. We here report a rare syndromic complex of squamous cell carcinoma of the temporal bone, deaf-mutism & syndactyle in a 12 year old male child, presented in Department of Otorhinolaryngology, Hamidia Hospital, Bhopal, with one month history of mass in left external auditory canal & lower motor neuron facial nerve palsy on a background of left chronic suppurative otitis media since childhood. Introduction Malignant tumours of mastoid and middle ear are rare, accounting for the 5-26% of all ear neoplasms1,2. Of these neoplasms, squamous cell carcinoma is most common with an incidence of one case in one million and peak age of 60 years1,2. We here reported a rare presentation of a squamous cell carcinoma of the temporal bone in a 12-year-old child in association with syndactyly and deafness mutism. Case Report A 12-year-old deaf & dumb child presented in department of ENT with mass in left ear and left facial nerve palsy since one month. He had a history of intermittent recurrent left ear discharge since childhood. Examination showed a fleshy granular mass filling the left external auditory canal with swelling in lower part of pre and postauricular region (fig 1). Tympanic membrane was not seen. Patient also had a complete infranuclear facial palsy. Bony syndactyly of right hand was also present. (fig 2).