ABSTRACT
The lacrimo-auriculo-dento-digital (LADD) syndrome is a rare inherited disorder affecting the lacrimal, auricular, salivary, and osseous system. Only very limited data on LADD associated petrous bone lesions are available up to now. We describe a young girl suffering from the LADD syndrome with sensorineural hearing loss and inner ear dysplasia. This case suggests, that inner ear dysplasia might be a more common feature of the LADD syndrome than has been assumed so far.
Subject(s)
Abnormalities, Multiple/diagnosis , Ear, Inner/abnormalities , Adolescent , Audiometry , Bone and Bones/abnormalities , Ear, Inner/diagnostic imaging , Epiglottis/abnormalities , Female , Hearing Loss, Sensorineural/diagnosis , Humans , Imaging, Three-Dimensional , Lacrimal Apparatus/abnormalities , Syndrome , Tomography, X-Ray Computed , Tooth AbnormalitiesABSTRACT
OBJECTIVES: To evaluate the impact of multislice computed tomographic (MSCT) imaging, a recently developed computed tomographic technique, on imaging of temporal bone malformations. STUDY DESIGN: Retrospective case review. SETTING: The study was performed at a tertiary referral center. PATIENTS: High-resolution temporal bone studies of 168 consecutive patients were reviewed for various temporal bone malformations. MAIN OUTCOME MEASURES: Visualization of various dysplastic conditions of the temporal bone without the need for supplementary scan procedures or additional imaging techniques. INTERVENTION: MSCT imaging was performed on a scanner with four detector rows by using the following parameters: 120 kV, 50 mA/s, 0.5-mm slice thickness, 0.2-mm reconstruction increment, pitch factor of 0.75, and a field of view of 160 mm. Two-dimensional and three-dimensional image reconstructions were performed subsequent to data transfer to a workstation. In one patient suspected of having a vascular dysplasia, high-resolution MSCT imaging was supplemented by multislice computed tomographic angiography. RESULTS Temporal bone dysplasias were encountered in 28 patients (16.7%), affecting the external auditory canal (n = 15), the middle ear (n = 18), the inner ear (n = 5), and the vascular system (n = 3). All temporal bone dysplasias were visualized by two-dimensional and three-dimensional images of excellent detail resolution. In none of the patients were supplementary computed tomographic scans necessary, except for one patient with evidence of an aberrant course of the internal carotid artery. Multislice computed tomographic angiography obviated the need for invasive diagnostic procedures. CONCLUSION: MSCT imaging of the temporal bone allows for comprehensively assessing various dysplasias through high-quality two-dimensional and three-dimensional image reconstructions.
Subject(s)
Bone Diseases, Developmental/diagnostic imaging , Temporal Bone/abnormalities , Temporal Bone/diagnostic imaging , Tomography, X-Ray Computed/instrumentation , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Retrospective StudiesABSTRACT
OBJECTIVE: To evaluate 3-dimensional inner ear visualization by volume rendering of high-resolution magnetic resonance data in patients with clinically suspected inner ear abnormality. DESIGN: Prospective comparative study of different postprocessing techniques, based on blinded film readings. SETTING: Tertiary referral hospital. SUBJECTS: Fifty patients (17 females and 33 males) aged 1 to 77 years (average age, 42 years) with sensorineural hearing loss, vertigo, and/or tinnitus. INTERVENTION: Postprocessing of magnetic resonance data to inner ear reconstructions by the use of volume rendering as well as maximum-intensity projection; caloric testing by electronystagmography. MAIN OUTCOME MEASURES: Film was read blindly by 4 radiologists using a 5-point parameter scale for image quality and diagnostic value. The assessibility of inner ear subsegments was evaluated. The specificity of volume-rendered reconstructions for detecting semicircular canal obliterations was assessed in a subgroup of 9 patients by caloric testing. The time required for data postprocessing as well as film reading was recorded by means of a stopwatch. RESULTS: Volume-rendered inner ear reconstructions were superior in image quality (P<.001), diagnostic value (P<.001), subsegment inner ear assessment (P<.01 to P<.001), and film reading time (P<.001) compared with maximum-intensity projections. The data postprocessing time was comparable for both techniques. Caloric weakness was noted in all patients assessed by electronystagmography. CONCLUSION: Volume rendering is the postprocessing technique of choice for 3-dimensional inner ear visualization, performing better than maximum-intensity projections with respect to various parameters.
