Isolated primary unilateral stenosis of the internal auditory canal

Congenital primary stenosis of the internal auditory canal (TAC) may exist in isolation or along with a number of other osseous anomalies of the tempo ral bone. Most of the literature on IAC stenosis is concerned with its effe ct on the outcome of cochlear implantation (i.e. patients with profound bil ateral hearing loss). In addition, some degree of canal, asymmetry has been noted in patients with normal hearing, questioning the causal relationship of this finding to deafness. We describe two children with computed tomogr aphy (CT) documented severe primary unilateral narrowing of the IAC and an associated ipsilateral sensorineural hearing loss. Typical radiographic fin dings are described, and the relevant developmental pathology is discussed. The ipsilateral association of stenosis and hearing loss strengthens the l ink between narrowing of the IAC and deafness. (C) 1999 Elsevier Science Ir eland Ltd. All rights reserved.