Cochlear implant in a patient with mondini's deformity of the cochlea: pilot patient in Pakistan.

Autosomal-recessive genes account for about 80% of the patients of non-syndromic deafness, and a major portion of those lead to cochlear pathology. Given the strong cultural practice of consanguineous marriages and the lack of awareness regarding screening modalities, a high prevalence of hereditary pre-lingual deafness is seen in Pakistan. Considering the situation, cochlear implant surgery was introduced by Aga Khan University Hospital (AKUH), Karachi, Pakistan, in 2003. Recently we decided to expand the profile and services available and conducted the first ever cochlear implant on an anatomically-challenged cochlea. The case report relates to the experience of our pilot patient who was suffering from Mondini's deformity.