Autosomal-recessive genes account for over 80% of non-syndromic deafness patients, with a large proportion of those resulting in cochlear disease. In our case, on the left, there was a total lack of all inner ear structures, including the cochlea, semicircular canals, vestibular and cochlear aqueducts, resulting in Michael deformity/full labyrinthine aplasia. However, cochlea abnormally has 1.5 turns: a basal turn and a cystic apex with an expanded vestibule, resulting in the mondini deformity on the right. Pakistan has a high frequency of inherited pre-lingual deafness due to the strong cultural practice of consanguineous marriages and a lack of information regarding screening procedures. [ABSTRACT FROM AUTHOR]