Journal of Dental Science and Medicine
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Mondini syndrome is a rare congenital sensorineural deafness. Malformation of cochlea in inner ear unilateral or bilateral may cause hear loss, recurrent meningitis and otorrhea. Mondini syndrome can be isolated or combined with another ear malformation or other syndrome. The condition occurs at seventh week of embryonic development. This case reports 5 years old female child patient came to the dental clinic complaining from pain in lower right second molar. First case in officer clinic diagnosed with profound hearing loss bilateral with sever Mondini deformity in the inner ear. Patient 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 deformity in cochlea. Otoacoustic emissions and ABR was consist with the diagnosis of severe to profound sensorineural hearing loss. Failed trial of cochlear implant, period of hearing aid trial failed to show any response status post brainstem implant done in Germany. Communication with hearing impaired patient has some difficulties and needs a good level of skills and awareness. In this case, it was the father helped with sign language and communicating with the child patient easier. Dentists should learn simple techniques for dealing with such impairment like removing mask to allow for lip reading, writing or drawing what they want to say or using sign language.
Faisal Al Rumaihi has completed his BDS in 1988 from King Saud University in Riyadh KSA and Residency program at RKH Hospital in 1989. He has completed Restorative and Cosmetic Dentistry in 1995 at Boston University, USA and PhD degree at Boston University Goldman School of Graduate Dentistry USA. He is the Consultant Restorative in Dentistry in Prince Sultan Military Medical City in Riyadh. He is Director of Restorative section in dental clinic and have a year of teaching and clinical supervision experience.
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