Otodental Syndrome: A Case Report

Resident: Adam J. Bottrill
Date: 11JUN10
Region: Providence
Article title: Otodental Syndrome
Author(s): Colter, J.D. et al
Journal: Pediatric Dentistry
Page #s: pp. 482-485
Year: 2005, 27:6
Major topic: Otodental Syndrome
Minor topic(s): NA
Type of Article: Case Report
Main Purpose: Present a case report of the rare Otodental Syndrome

Key points in the article discussion:

I. Otodental Syndrome

A. Abnormalities in dental crown morphology

1. Max/Man primary and permanent incisors normal
2. Canines and molars large and bulbous in both dentitions
3. Canines of both dentitions present with yellow hypoplastic areas on labial surf.
4. Cusps of affected molars separated by deep vertical enamel fissures.
5. Abnormal molars MAY be result of fusion.
6. Pulp chambers of molars often duplicated. (difficult endo)
7. Absent or micro premolars.
8. Conical supernumeraries and odontomas.

B. Hearing

1. Loss of hearing typically above 1,000Hz.
2. Can start as early as 2-3 yo or as late as puberty.
3. Not all patients present with hearing loss.

C. Etiology

1. Inherited... Autosomal dominant.
2. Variable penetrance and expressivity.
3. Genes: BMP4, MSX1, FGF8, BARX1, DLX1/2

D. Case

1. 9 yo male, NSMH (hearing was not effected),
2. DH: ext of Max left prim 2nd molar due to ectopic eruption of perm molar.
3. Exam: Multiple carious lesions, canines and molars large, spherical and bulbous in perm and prim dentition, deep vert enamel fissures, 6-8 cusps on each tooth, hypoplastic areas on labial surfaces of canines, duplicated pulp chambers, supernumeraries or odontomas, micro premolars, 4. Hearing test not performed.

E. Dental Concerns

1. Dx from dentist (semper vigilans!!!)
2. Future endo concerns
3. OH and preventative care EXTREMELY IMPORTANT

Assessment of article: If I were to do a case presentation on otodental syndrome, I probably would have waited for the hearing tests to be done before I published... shenanigans.