Neck extension because of contraction of cervical extensor muscles often brings about a lower-positioned tongue secondary to jaw opening in patients with congenital myopathy (CM). We hypothesized that neck extension in control subjects would reproduce the lower position of the tongue similar to that found in a CM patient. A simple method was formulated to evaluate the tongue position in terms of tongue pressure on the maxillary molar. A pair of pressure sensors was attached to the buccal and lingual surfaces of the upper molar for both the CM patient and four control subjects. Changes in the buccal and tongue pressures were recorded at the neck extension position for the CM patient and during both the natural head position and neck extension for the control subjects. There was a remarkable difference between buccal and tongue pressures in the neck extension position in the CM patient: tongue pressure was not detected at all, indicating there was no contact between tongue and upper molar. The buccal and tongue pressures were approximately equal in the natural head position in the control subjects. However, both buccal and tongue pressures were reduced during neck extension in the control subjects, with a greater decrease in the tongue pressure than the buccal pressure. These findings suggest that neck extension in a control subject reproduces the lower position of the tongue observed in CM patients. We propose that the pressure sensor enables evaluation of the tongue position, but further investigation is required.
Stickler syndrome (MIM 108300, 604841, 184840) is an autosomal dominant disease characterized by midfacial flattening and variable disorders of vision, hearing and articulation. There are three types of the syndrome caused by mutations in different genes (type 1, COL2A1; type 2, COL11A1; and type 3, COL11A2). About 20% of type 1 patients have cleft palate or bifid uvula, but there have been no case reports of orthodontic treatment of this syndrome so far. The Japanese female patient presented here with Stickler syndrome was characterized by a flat midface and had high myopia, sensorineural hearing loss, enlarged joints, and cleft of the soft palate. She had fairly small SNA and SNB angles and a steep mandibular plane with an enlarged gonial angle. The incisors of both arches were retroclined, and a large overjet and overbite were noted. Orthodontic treatment was initiated at 11 years of age using a lingual arch appliance followed by an edgewise multibracket appliance. Stable functional occlusion was obtained after the treatment. Most of the other seven Stickler syndrome patients exhibited pretreatment characteristics of small SNA and SNB angles, steep mandibular planes, enlarged gonial angles, and retroclined incisors of both arches, demonstrating the characteristic skeletal and occlusal features of this syndrome.
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