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Ivan J. Keogh, MD, FRCS; Maria J. Troulis, DDS, MSc; Angelo A. Monroy, MD; Roland D. Eavey, MD, SM; L. B. Kaban, MD, DMD

Arch Otolaryngol Head Neck Surg. 2007;133(10):997-1001.

Objective  To determine the proportion of hemifacial microsomia (HFM) in patients with unilateral or bilateral "isolated" microtia.

Design  Prospective cohort clinical study.

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

Patients  One hundred consecutive patients with isolated microtia.

Interventions  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).

Main Outcome Measures  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.

Author Affiliations: Department of Otolaryngology, Pediatric Otolaryngology Service, and Department of Otology and Laryngology, Massachusetts Eye and Ear Infirmary (Drs Keogh, Monroy, and Eavey); Department of Oral and Maxillofacial Surgery, Massachusetts General Hospital (Drs Troulis and Kaban); and Department of Genetics, Harvard Medical School (Drs Keogh, Monroy, and Eavey), Boston, Massachusetts; and Royal College of Surgeons in Ireland, Dublin (Dr Keogh). Dr Keogh is currently at the Academic Department of Otolaryngology, University College Hospital, Galway, Ireland.