Introduction
Bilateral profound hearing loss, whether congenital or acquired, is one of the most disabling anomalies a child can have. As the child grows up, there is associated delay in development of speech leading to social and functional stigmata for the child as well as the caregivers. Here comes the importance of early diagnosis and intervention. Out of the well-studied causes, Inner Ear Malformations (IEMs) comprise 20% of causes of congenital hearing loss (1,2). IEMs have been classified exhaustively by various authors depending on the site of involvement, each category displaying a variable degree and type of hearing loss. A normal cochlea has 2 ½ or 2 ¾ turns with modiolus as the central axis and interscalar septum as a partition between cochlear inner wall and modiolus (2,3). The cochlea is said to be hypoplastic when there are less than 2.5 turns. There are four types of cochlear hypoplasia (I to IV) with hearing status ranging from normal to profound loss; it being very rare to have normal hearing (2). When the cochlea has 1.5 turns then it is termed as Mondini dysplasia. Before proceeding to any intervention in case of IEMs, it is imperative to look for the presence of cochlear nerve and various audiologic parameters (4). Here we are reporting two cases, one with cochlear hypoplasia type IV and one patient with Mondini dysplasia. The point of interest in these cases was a preserved audiologic status inspite of severe malformations of the inner ear. The clinico-radiological discrepancies piqued our interest and thus, we would like to share these cases owing to their rarity.