Abstract Introduction: Estimates of the magnitude of consanguinity related profound bilateral sensory neural non syndromic hearing loss (PSNHL) children in Pakistan are limited. Methods: A descriptive study was conducted among deaf children age 0-18 year in three rehabilitation schools in Karachi in January 2020 that fulfilled the following case criteria: (1) Deaf child with profound sensory neural hearing loss (SNHL), pure tone audiometry (PTA), Brainstem Evoked Response Audiometry (BERA) and registered in a deaf school (2) presence or absence of self-reported kinship between the husband and his wife, (3) presence or absence of self-reported husband-and-wife part of a WATA SATA arrangement. Results: A total of 714 children aged ���18 years identified for confirmed PSNHL. Audiology findings from 260 (36.4 %) children met the criteria for confirmed PSNHL and consanguinity was detected in 235 of them (90.3%). The minimum detected prevalence of consanguinity related PSNHL in Karachi area was 7.1 per 1000 in children < 18 years of age. The average student age was 9.81 years with male (62%) predominance. The study population included males (62%) and females (38%) and ratio was 1.7:1. Most of PSNHL student (85%) belonged to 05���14-year age group. The majority of the respondents (69.6%) reported to have one deaf child in family. Nearly half students (43%) were of secondary class education formed the majority. Conclusion: Mandatory pre-pregnancy & genetic counselling, non-consanguineous marital relation awareness, tele health, pro-bono medical and surgical services and consanguinity related PSNHL new born birth record registration is justified for inclusion in expanded programme of immunization card for early referral to best treatment modalities like cochlear implant surgery in Pakistan.