Department of Dentistry, Saudi Board, Saudi Arabia
Case Report
Dental Management for Patient with Mondini Syndrome
Author(s): Badria Al Matrafi*, Mohammad Al Sughyer, Saud Al Saif, Majeed Al Jadeed, Sahar Mansour, Saleh Al Garzai, Abdullah Al Eiss and 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 audit.. View More»