Three Years of Follow-up of Otodental Syndrome in 3-year-old Chinese Boy: a Rare Case Report

Overview

Journal BMC Oral Health

Publisher Biomed Central

Specialty Dentistry

Date 2019 Jul 27

PMID 31345197

Citations 1

Authors

Ji-Mei Su

Su-Juan Zeng

Xiao-Wei Ye

Zhi-Fang Wu

Xin-Wen Huang

Janak L Pathak

Affiliations

Soon will be listed here.

Abstract

Background: Otodental syndrome is an exceptionally rare autosomal dominant condition characterized by a delayed eruption of posterior teeth, globodontia, lisping, and sensorineural hearing loss. In this case report, we reported a 3-year-old Chinese boy with the otodental syndrome.

Case Presentation: A 3-year-old Chinese boy was referred to our hospital with complaint of no eruption of primary canines and molars. Three years follow-up showed lately erupted bulbous primary canines with hypoplastic enamel spot, globe-shaped primary molars and sensorineural hearing loss at 4 and a half-year-old age. We diagnosed otodental syndrome in the patient's mother with hearing loss at 16-year-old age. Gene sequencing and analysis of deafness-related genes GJB2, GJB3, SLC26A4, and mtDNA did not reveal any mutation or SNPs in the patient and his mother.

Conclusions: This case report highlights the importance of detailed medical, dental, and family history examination, as well as multi-disciplinary teamwork for diagnosis and treatment of otodental syndrome.

Citing Articles

Clinicopathological and molecular insights into odontogenic tumors associated with syndromes: A comprehensive review.

Schuch L, Silveira F, Pereira-Prado V, Sicco E, Pandiar D, Villarroel-Dorrego M World J Exp Med. 2024; 14(4):98005.

PMID: 39713074 PMC: 11551705. DOI: 10.5493/wjem.v14.i4.98005.

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