Isolated microtia as a marker for unsuspected hemifacial microsomia.
OBJECTIVE
To determine the proportion of hemifacial microsomia (HFM) in patients with unilateral or bilateral "isolated" microtia.


METHODS
Prospective cohort clinical study.


METHODS
University-affiliated, tertiary referral clinic for patients with microtia.


METHODS
One hundred consecutive patients with isolated microtia.


METHODS
All the patients underwent a clinical examination and audiologic evaluation. The OMENS classification system was used to grade the severity of craniofacial features: orbital deformity, mandibular hypoplasia, ear deformity, nerve (cranial nerve VII) involvement, and soft-tissue deficiency. Each anatomical abnormality was graded from 0 (normal) to 3 (most severe) (score range, 0-15).


METHODS
The OMENS scores, percentage of patients with isolated microtia and undiagnosed HFM, and isolated microtia as an early clinical marker for HFM.


RESULTS
Forty patients (40%) with microtia were determined to have HFM (31 unilateral and 9 bilateral). Mean patient age was 9.2 years (range, 6 weeks to 41 years), with male predominance (27 males and 13 females). The OMENS scores were less than 5 in 24 patients and 6 to 10 in 16 patients. Thirty patients had cranial nerve deficits, and 37 had mandibular asymmetry. Thirty-seven patients demonstrated conductive hearing loss, and 1 had sensorineural hearing loss.


CONCLUSIONS
Isolated microtia served as an early clinical marker for asymmetrical facial growth in 40% of the patients. Isolated microtia and HFM could represent a spectrum of expression of the same developmental phenomenon.
Keogh
Ivan J
IJ
Department of Otolaryngology, Pediatric Otolaryngology Service, Massachusetts Eye and Ear Infirmary, and Department of Genetics, Harvard Medical School, Boston, MA, USA. ivankeogh@oceanfree.net
Troulis
Maria J
MJ
Monroy
Angelo A
AA
Eavey
Roland D
RD
Kaban
L B
LB
D000015:Abnormalities, Multiple / Q000145:classification / Q000175:diagnosis; D000293:Adolescent; D000328:Adult; D001300:Audiometry, Evoked Response; D001301:Audiometry, Pure-Tone; D002648:Child; D002675:Child, Preschool; D002971:Cleft Lip / Q000145:classification / Q000175:diagnosis; D002972:Cleft Palate / Q000145:classification / Q000175:diagnosis; D004431:Ear, External / Q000002:abnormalities*; D005146:Facial Asymmetry / Q000145:classification / Q000175:diagnosis* / Q000503:physiopathology; D005154:Facial Nerve / Q000002:abnormalities; D005260:Female; D005500:Follow-Up Studies; D006309:Hearing / Q000502:physiology; D006801:Humans; D007223:Infant; D008297:Male; D008334:Mandible / Q000002:abnormalities; D009915:Orbit / Q000002:abnormalities; D011379:Prognosis; D011446:Prospective Studies; D011862:Radiography, Panoramic; D012720:Severity of Illness Index