PMC3040286_01
PMC3040286_02
PMC3040286_03
a and b: Current appearance of the proband.
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F1
The diagnosis of OSMED was established based on midfacial hypoplasia with a flat nose and short upper extremities (Fig. 1a and b).
a and b: Left upper and lower extremity radiographs show short and dumbbell shape humerus and tibia. Metaphyses of long bones are enlarged (megametaphyses) and flared.. c: Lateral spine radiography demonstrates mild platyspondyly.
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F2
The hights of anterior part of lower thoracal vertebral bodies were decreased (Fig. 2a, 2b and 2c).
Hearing thresholds of the proband with Visual Reinforcement Audiometry while the case was at 17 months.
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F3
HL (with visual reinforcement audiometry), which are shown in Fig. 3. Hearing mesurement was performed within soundproof rooms, using an AC-40 audiometer (Interacoustics, Assens, Denmark).
Hearing thresholds of the proband at 4 years old.
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F4
Hearing thresholds of both ears are shown in Fig. 4. Speech Awereness Threshold (SAT) was 40 dB HL in the right ear and 50 dB HL in the left ear. He used hearing aids on regular basis after 20 months of age and his verbal communication was the same level as that in peers.
Clinical ABR outcomes for click stimulation.
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F5
Clinical ABR outcomes were obtained for click stimulation and are shown in Fig. 5.