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Craniofacial, Temporal Bone, and Audiologic Abnormalities in the Spectrum of Hemifacial Microsomia
Reza Rahbar, DMD, MD;
Caroline D. Robson, MD;
John B. Mulliken, MD;
Lynn Schwartz, MS, CCC-S;
James Dicanzio, MS;
Margaret A. Kenna, MD;
Trevor J. McGill, MD;
Gerald B. Healy, MD
Arch Otolaryngol Head Neck Surg. 2001;127:265-271.
Objectives To evaluate the clinical, audiologic, and temporal bone computed tomograpic
findings in patients with hemifacial microsomia and to use the OMENS (each
letter of the acronym indicates 1 of the following 5 dysmorphic manifestations:
O, orbital asymmetry; M, mandibular hypoplasia; E, auricular deformity; N,
nerve involvement; and S, soft tissue deficiency) grading system to assess
possible correlations between the severity of dysmorphic features with the
type of abnormalities in the temporal bone and with degree of hearing deficit.
Design Retrospective study.
Setting Tertiary care children's hospital.
Patient Forty patients with hemifacial microsomia.
Result Mandibular hypoplasia and auricular abnormalities were the most common
clinical manifestations, present in 39 patients (97%) and 38 patients (95%),
respectively. Conductive hearing loss was noted in 35 patients (86%) and sensorineural
hearing loss in 4 patients (10%). Facial nerve weakness was present in 20
patients (50%). Twenty patients had unilateral aural atresia, 12 patients
had unilateral aural stenosis, and 7 patients had bilateral anomalies. Moderate
hypoplasia or atresia of the middle ear was noted in 36 patients (90%) and
ossicles were malformed in 30 patients (75%). Hypoplasia of the oval window
was the most common inner ear abnormality.
Conclusions Severity of craniofacial features (total OMENS score) significantly
correlated with the degree of temporal bone abnormality, but no correlation
was noted with the degree or type of hearing loss. We recommend the following:
(1) use of the OMENS classification system for documentation and analysis
of dysmorphic finding in hemifacial microsomia; (2) complete audiologic evaluation
in all patients with hemifacial microsomia regardless of the type of craniofacial
abnormalities; and (3) temporal bone computed tomography for further evaluation
of hearing deficit.
From the Department of Otolaryngology and Communication Disorders (Drs
Rahbar, Kenna, McGill, and Healy), Department of Radiology (Dr Robson), Division
of Plastic Surgery (Dr Mulliken), Division of Audiology (Ms Schwartz), and
the Department of Clinical Research (Mr Dicanzio), The Children's Hospital;
and Harvard Medical School, (Drs Rahbar, Kenna, McGill, Healy, Robson, and
Mulliken), Boston, Mass.
Corresponding author: Reza Rahbar, DMD, MD, Department of Otolaryngology
and Communication Disorders, The Children's Hospital, 300 Longwood Ave, Boston,
MA 02115 (e-mail: rahbar{at}a1.tch.harvard.edu).
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