Craniofacial, Temporal Bone, and Audiologic Abnormalities in the Spectrum of Hemifacial Microsomia

Rahbar, Reza; Robson, Caroline D.; Mulliken, John B.; Schwartz, Lynn; DiCanzio, James; Kenna, Margaret A.; McGill, Trevor J.; Healy, Gerald B.

doi:10.1001/archotol.127.3.265

Cited by 56 publications

(44 citation statements)

References 17 publications

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“…All temporal CT scans were evaluated for specific criteria on both sides. 10 These criteria for CT evaluation were based on the methodology described by Rahbar and associates. 10 Individual ordinal scores were tallied for each area and were summed to a total radiographic score.…”

Section: Methodsmentioning

confidence: 99%

“…10 These criteria for CT evaluation were based on the methodology described by Rahbar and associates. 10 Individual ordinal scores were tallied for each area and were summed to a total radiographic score. Areas evaluated were the external auditory canal (stenosis, N ϭ 1; atresia, N ϭ 2), middle ear space (hypoplasia, N ϭ 1; atresia, N ϭ 2), ossicular formation (fused, N ϭ 1; unidentified, N ϭ 2), mastoid air space (poorly pneumatized, N ϭ 1; no pneumatization, N ϭ 2), facial nerve (displaced, N ϭ 1; not identified, N ϭ 2), condyle (displaced, N ϭ 1; hypoplastic, N ϭ 2), and inner ear (abnormal, N ϭ 1).…”

Section: Methodsmentioning

confidence: 99%

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Clinical, Radiological, and Audiological Relationships in Hemifacial Microsomia

Wan

Meara

Kovanlıkaya

et al. 2003

Annals of Plastic Surgery

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Hemifacial microsomia is a craniofacial disorder involving structures derived from the first and second brachial arches. Bony structures, soft tissue, and the facial nerve can all be hypoplastic or absent. In this retrospective study of 70 patients at the Children's Hospital Los Angeles, craniofacial dysmorphology, temporal bone computed tomography, and audiological evaluations were examined. The purpose of this study was to identify further relationships between clinical findings, temporal bone anatomy, and audiological findings in these patients. Significant relationships were identified between total radiographic score and overall clinical findings (p<0.001). Clinical measurements of mandibular hypoplasia were also predictive of temporal computed tomographic findings (p<0.001), whereas clinical ear findings were related specifically to specific temporal bone abnormalities such as hypoplasia of the middle ear (p=0.008) and fusion or absence of ossicles (p<0.001). Interestingly, neither clinical findings nor temporal bone computed tomographic findings were related to either hearing loss type or degree. Audiology should not be deferred on the basis of relatively mild clinical findings.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Clinical, Radiological, and Audiological Relationships in Hemifacial Microsomia

Wan

Meara

Kovanlıkaya

et al. 2003

Annals of Plastic Surgery

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“…The subject in the present case presented with hypodontia in the mandibular incisors, premolars and molars on the affected side, which is consistent with the literature. The incidence of delayed tooth development with hemifacial microsomia is proportional to the extent of mandibular deformity (7,8). …”

Section: Discussionmentioning

confidence: 99%

Hemifacial microsomia: clinicoradiological insight and report of a case

Chhabra

2017

Ethiop J Health Sci

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BACKGROUND: Hemifacial microsomia is a congenital malformation characterized by deficiency in the amount of hard and soft tissue on one side of the face. It is primarily a syndrome of the first branchial arch, involving underdevelopment of the temporomandibular joint, masticatory muscles, mandibular ramus, ear and, occasionally, defects in facial nerve and muscles. CASE DETAILS: The clinical and radiological manifestations of a 14-year-old male patient having hemifacial microsomia is highlighted in this article to enhance our knowledge and diagnostic skill of this rare entity. CONCLUSION: This case illustrates that early diagnosis and intervention in a patient with hemifacial microsomia is quintessential for proper functioning and esthetics of the orofacial structures, which will lead to a better prognosis.

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“…71 In the setting of hemifacial microsomia, 86% of patients have conductive hearing loss associated with stenosis or atresia of the external auditory canal, ossicular malformation and atresia and hypoplasia of the oval window. 72 Although typically unilateral, up to 30% of patients with hemifacial microsomia have bilateral otologic findings. 73 The Pierre Robin sequence (PRS) is characterized by a triad of glossoptosis, micrognathia and cleft palate.…”

Section: Syndromic Conductive Hearing Lossmentioning

confidence: 99%