Badria Al Matrafi*, Mohammad Al Sughyer, Saud Al Saif, Majeed Al Jadeed, Sahar Mansour, Saleh Al Garzai, Abdullah Al Eiss, Reem Al Moslem
Mondini syndrome is a rare congenital sensorineural deafness. Malformation of cochlea in inner ear is unilateral or bilateral. May cause hear loss, recurrent meningitis and otorrhea. Mondini syndrome can be isolated or combined with other ear malformation or other syndrome. The condition occurs at seventh week of embryonic development. We report 4 years old female child come to our dental clinic diagnosed with profound hearing loss bilateral with sever mondini deformity in the inner ear, status post brainstem implant done in Germany. Patient is not responding to any sounds. There is no history of any other congenital problems. There is positive history of consanguinity and there is no family history of congenital hearing loss. No history of trauma or meningitis, no significant perinatal history. The patient looks well, no dysmorphic features, normal external auditory canal and bilateral dull tympanic membrane. Examination was otherwise normal. CT revealed mild deformity in cochlea. Otoacoustic emissions and ABR consist of the diagnosis of severe to profound sensorineural hearing loss. Failed trial of cochlear implant, period of hearing aid trial failed to show any response. The communication with hearing impairment child has some difficulties and need some skills and awareness.
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