Inner Ear Malformation Masquerading as CSF Rhinorrhea - New Perspectives of Management and Literature Review

INTRODUCTION: Spontaneous cerebrospinal fluid (CSF) rhinorrhea is rare and may develop secondary to inner ear malformation. A possible diagnosis of CSF leak should be considered in any Pediatric patient who presents with hearing impairment, rhinorrhea, or otorrhea. CASE REPORT: We describe a case of 13 months male infant presenting with rhinorrhoea which on evaluation found to be CSF oto-rhinorrhoea due congenital inner ear malformation. Imaging showed malformed inner ear on both sides with CSF leak on left side with bilateral profound sensory neural hearing loss. A multidisciplinary management was considered. Child underwent CSF leak repair on left side followed by Cochlear implantation on right side in another setting. CONCLUSION: This case is a perfect example to describe the cumbersome management of CSF leak with inner ear anomaly addressing the auditory habilitation on the grounds of recent innovations. As per available literature inner ear anomaly is an important subgroup of population of cochlear implant candidates with promising auditory outcomes.