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Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype–phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype–phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a “typical” phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85–90% of the patients showed a hearing level of 20–39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed “true” auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype–phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype–phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

There is no report on acute sensorineural hearing loss with congenital cytomegalovirus (cCMV) infection in basic experiments.The aim of this study was to evaluate the effect of dexamethasone, an anti-inflammatory steroid, on acute sensorineural hearing loss in the mouse cytomegalovirus (MCMV) infection model mice.Sensorineural hearing loss model mice were divided into two groups, one with and one without intratympanic dexamethasone. Dexamethasone was injected into the tympanic cavity of only the right ear, and hearing ability was assessed at the ages of three, six, and eight weeks by auditory brainstem response measurement.Among the 23 mice intratympanically injected with dexamethasone (15 μg/mouse) at the age of three weeks, five (21.7%) had a hearing improvement of at least 10 dB and 18 (78.3%) had no improvement at the age of six weeks. Among the 19 mice that did not receive a dexamethasone injection, one (5.3%) showed improvement and 18 (94.7%) showed no improvement (In this study, transtympanic infusion of dexamethasone into the tympanic cavity was effective in some mice with sensorineural hearing loss, suggesting that, in addition to angiogenesis, anti-inflammatory activity might be a mechanism of treatment for hearing loss.

In layered van der Waals materials, the stacking of layers plays a crucial role in their electronic states. To investigate the effect of stacking of layers on the electronic states, we utilized the stacking of two kinds of $\sqrt{13}\ifmmode\times\else\texttimes\fi{}\sqrt{13}$ domains, which are rotated $27.8{}^{\ensuremath{\circ}}$ relative to each other, in Nb-substituted $1T\text{\ensuremath{-}}\mathrm{Ta}{\mathrm{Se}}_{2}$. We found the appearance of a two-dimensional superstructure of the Star of David (SoD) lattice. The SoDs in the superstructure show three different electronic states due to the different stackings of the SoDs between the top surface and the layer below. This stacking of $\sqrt{13}\ifmmode\times\else\texttimes\fi{}\sqrt{13}$ domains provides insights into the effects of stacking on electronic structure and has the potential to produce unique electronic states.

In this paper, we report scanning tunneling microscopy/scanning tunneling spectroscopy measurements of the evolution of the electronic state from the Mott phase to the nearly commensurate phase in Nb-substituted $1T\text{\ensuremath{-}}\mathrm{Ta}{\mathrm{S}}_{2}$. Although substitution of 1% Nb for Ta reduced the transition temperature, the sample was still in the Mott phase at the measurement temperature of 4.2 K. In this sample, domain walls were generated, and the electronic state changed from the Mott state at and around the domain walls. At substitution of 5% Nb, the sample did not undergo the phase transition to the Mott phase down to 4.2 K. In this sample, the number of the walls increased and the size of the domains largely reduced. The increase in the number of walls caused the overlap of the changed electronic states near the walls. This overlap induced the overall change in the electronic state of the sample.

Objectives Valganciclovir (VGCV) has been shown to improve sensorineural hearing loss (SNHL) and neurological outcomes in patients with neonatal symptomatic congenital cytomegalovirus (cCMV) infection. However, reports on the pharmacokinetics, efficacy and safety of oral VGCV are limited. The aim of this study is to evaluate the pharmacokinetics of VGCV for use in the treatment of cCMV. Methods This was a single-center, retrospective observational study conducted at Saitama Children's Medical Center in Japan between 2012 and 2017. CMV DNA copy number, maximum plasma VGCV concentration (Cmax), and adverse events (ADEs) during treatment were evaluated. Results A total of 26 patients with cCMV who received VGCV were included in this study. The median age at VGCV initiation was 9.5 months (range 0–46). Twenty-one patients (81%) had SNHL at baseline. Of these, five patients (19%) presented with improved SNHL, and none experienced worsened SNHL during treatment. The mean VGCV Cmax was 3.5 μg/mL (range 2–5.3), with no significant variation among individual values, and the values were maintained during treatment. Furthermore, there were no correlations between the Cmax values and age, sex, SNHL improvement or ADEs. Neutropenia ( Conclusions VGCV prevented the progression of SNHL without serious ADEs due to its stable pharmacokinetics. This study provides safety and tolerability of VGCV for the treatment of cCMV patients.

The development of an unconventional synthesis method has a large potential to drastically advance materials science. In this research, a new synthesis method based on a solid-state electrochemical reaction was demonstrated, which can be made available for intercalation and ion substitution. It was referred to as proton-driven ion introduction (PDII). The protons generated by the electrolytic dissociation of hydrogen drive other monovalent cations along a high electric field in the solid state. Utilizing this mechanism, Li+, Na+, K+, Cu+, and Ag+ were intercalated into a layered TaS2 single crystal while maintaining high crystallinity. This liquid-free process of ion introduction allows the application of high voltage around several kilovolts to the sample. Such a high electric field strongly accelerates ion substitution. Actually, compared to conventional solid-state reaction, PDII introduced 15 times the amount of K into Na super ionic conductor (NASICON)-structured Na3–xKxV2(PO4)3. The obtained materia...

We report scanning tunneling microscopy study to clarify the modulation structure in silver intercalated stage 2 2H-NbSe2 at low temperature. In addition to the surface structure expected from previous electron diffraction measurements, we observed unidirectional superstructure with period of 2 3 a 0 . Appearance of this new superstructure indicates that the instability of original charge density wave in pristine 2H-NbSe2 against strain induced by intercalants.

Single crystal of 2H-TaS2 was synthesized with flux method using the flux in which alkali metal ions were included. Analysis of the composition and the structure by EDX measurements and X-Ray diffraction showed that the hydrates of potassium ion were intercalated in 2H-TaS2 synthesized with NaCl/KCl flux. The local intercalation was confirmed by the observation of the superstructures with the period of 2a0 × 2a0 or √3a0 × √3a0 by scanning tunnelling microscopy measurements.

We report on the scanning tunneling microscopy (STM) and spectroscopy (STS) study on a newly discovered superconductor, Ir 1 − x Pt x Te 2 . We previously found that the sample which shows superconductivity forms so called “patchwork structure (PS)”. To obtain the spatial evolution of electronic structure on the PS, we performed STM/STS measurements on the PS. STS measurements revealed that an averaged spectrum shows the same energy asymmetry as that in the low temperature phase in the parent material, IrTe 2 . Furthermore, the spectrum at the trough on the PS is more asymmetric than that at the crest. This tendency is similar to that observed on the supermodulation in the low temperature phase in IrTe 2 .

The magnetoelectric coupling, i.e., cross-correlation between electric and magnetic orders, is a very desirable property to combine functionalities of materials for next-generation switchable devices. Multiferroics with spin-driven ferroelectricity presents such a mutual interaction concomitant with magneto-active and electro-active excitations called electromagnons. TbMnO3 is a paradigmatic material in which two electromagnons have been observed in the cycloidal magnetic phase. However, their observation in TbMnO3 is restricted to the cycloidal spin phase and magnetic ground states that can support the electromagnon excitation are still under debate. Here, we show by performing Raman spectroscopy measurements under pressure that the lower-energy electromagnon (4 meV) disappears when the ground state enters from a cycloidal phase to an antiferromagnetic phase (E-type). On the contrary, the magneto-electric activity of the higher-energy electromagnon (8 meV) increases in intensity by one order of magnitude. Using microscopic model calculations, we demonstrate that the lower-energy electromagnon, observed in the cycloidal phase, originates from a higher harmonic of the magnetic cycloid, and we determine that the symmetric exchange-striction mechanism is at the origin of the higher-energy electromagnon which survives even in the E-type phase. The colossal enhancement of the electromagnon activity in TbMnO3 paves the way to use multiferroics more efficiently for generation, conversion and control of spin waves in magnonic devices. Two electromagnons are found to evolve differently when the ground state enters from a cycloidal phase to an antiferromagnetic phase in TbMnO3. An international team led by Maximilien Cazayous from Universite Paris Diderot-Paris 7 performed Raman spectroscopy measurements and microscopic model calculations to understand how magneto-electric excitations evolve in TbMnO3 when hydrostatic pressure is applied. They found that two electromagnons, which are magneto-active and electro-active excitations, evolve differently when the ground state enters from a cycloidal phase to an antiferromagnetic phase. The lower-energy electromagnon only exists in the cycloidal phase, whereas the higher-energy one survives the phase transition and is enhanced by one order of magnitude in the antiferromagnetic phase. They attribute this to a symmetric exchange-striction mechanism, which may lead to a better use of multiferroics in magnonic devices.

We present scanning tunneling microscopy and spectroscopy measurements on a cleaved surface of the LaO0.9F0.1BiSe2 single crystals. Tunneling spectra show a finite local density of states at E F, which is consistent with metallic conductivity in bulk. In addition, the existence of the supermodulation running along the diagonal directions of Bi square lattice was revealed. The period of the supermodulation was about 3 to 5 times the length of the lattice constant. This period is close to that observed in LaO0.5F0.5BiSe2.

Antimony (Sb) Substitution less than 10 % is examined on a single crystal of a layered superconductor NdO0.7F0.3BiS2. Superconducting transition temperature of the substituted samples decreases with increasing Sb concentration. A lattice constant along the c axis showed a large decrease compared with that along the a axis. Since in-plane chemical pressure monotonically decreases with increasing Sb concentration, the suppression of the superconductivity is well described in terms of the decrease in in-plane chemical pressure.

Antimony (Sb) substitution of less than 8% was examined on a single crystal of a layered superconductor NdO0.7F0.3BiS2. The superconducting transition temperature of the substituted samples decreased as Sb concentration increased. A lattice constant along the c-axis showed a large decrease compared with that along the a-axis. Since in-plane chemical pressure monotonically decreased as Sb concentration increased, the suppression of the superconductivity is attributed to the decrease in the in-plane chemical pressure.

We examined Lead (Pb) Substitution effect on a single crystal of a layered superconductor LaO0.5F0.5BiS2. Pb concentration dependence of the lattice constant showed slight anomaly at about 8% and 9% substitution of Pb for Bi. These samples showed the enhancement of the superconducting transition temperature and the superconducting volume fraction. Furthermore, these samples showed the anomaly in the temperature dependence of the resistivity at about 150K. These results were not observed in Pb substituted NdO0.7F0.3BiS2. Therefore, the enhancement of the superconducting properties by Pb substitution is related to the structural instability for the pale perturbation in LaO0.5F0.5BiS2., 8 pages, 4 figures, to be published in Solid State Communications

Transition metal dichalcogenides 2H-TaS2 and 2H-TaSe2 show charge density wave (CDW) state and superconductivity. It has been reported that elemental substitution of chalcogenide suppresses the CDW and enhances superconductivity. However, how the CDW order is suppressed by the substitution has not been clarified yet. To clarify this, we performed real space observation of the CDW state in the commensurate phase of 2H-TaS1.8Se0.2 by scanning tunneling microscopy. The existence of domains separated by walls at which the phase of the CDW shifts was observed. The change in the electronic state on the domain boundaries in 2H-TaS1.8Se0.2 was not as drastic as that in 1T-Fe x Ta1-x S2.

Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recovering hearing, necessitating cochlear implants. We report a case of a 6-year-old boy with congenital deafness and bilateral inner ear malformations (right side, incomplete partition type I [IP-I]; left side, common cavity deformity). Hearing aids had a remarkable effect in this patient, enabling sufficient and favorable hearing recovery such as to allow the patient to engage in daily conversations. Per-rotatory nystagmus was recorded on an electronystagmogram for both right and left rotations in a damped rotational chair test. It is rare for deaf children with severe bilateral inner ear malformation to demonstrate favorable development in hearing and good equilibrium function. Our findings suggest that auditory-vestibular hair cells in this patient may have been partially preserved despite IP-I in the right ear and common cavity deformity of the left ear.

Objective Approximately 8–10% of newborns with asymptomatic congenital cytomegalovirus (cCMV) infection develop sensorineural hearing loss (SNHL). However, the relationship between CMV load, SNHL and central nervous system (CNS) damage in cCMV infection remains unclear. This study aimed to examine the relationship between urinary CMV load, SNHL and CNS damage in newborns with cCMV infection. Study design The study included 23 368 newborns from two maternity hospitals in Saitama Prefecture, Japan. Urine screening for cCMV infection (quantitative real-time PCR) and newborn hearing screening (automated auditory brainstem response (AABR) testing) were conducted within 5 days of birth to examine the incidence of cCMV infection and SNHL, respectively. CNS damage was assessed by MRI of cCMV-infected newborns. Results The incidence of cCMV infection was 60/23 368 (0.257%; 95% CI 0.192% to 0.322%). The geometric mean urinary CMV DNA copy number in newborns with cCMV was 1.79×106 copies/mL (95% CI 7.97×105 to 4.02×106). AABR testing revealed abnormalities in 171 of the 22 229 (0.769%) newborns whose parents approved hearing screening. Of these 171 newborns, 22 had SNHL (12.9%), and 5 of these 22 were infected with cCMV (22.7%). Newborns with both cCMV and SNHL had a higher urinary CMV DNA copy number than newborns with cCMV without SNHL (p=0.036). MRI revealed CNS damage, including white matter abnormalities, in 83.0% of newborns with cCMV. Moreover, newborns with CNS damage had a significantly greater urinary CMV load than newborns without CNS damage (p=0.013). Conclusions We determined the incidence of cCMV infection and urinary CMV DNA copy number in seemingly healthy newborns from two hospitals in Saitama Prefecture. SNHL and CNS damage were associated with urinary CMV DNA copy number. Quantification of urinary CMV load may effectively predict the incidence of late-onset SNHL and neurodevelopmental disorders.

In this paper, we report on scanning tunneling microscopy and spectroscopy (STM/STS) measurements on iron doped 1$T$-Ta$_{1-x}$Fe$_x$S$_2$. A novel domain structure composed of the domains with localized nature divided by the walls is observed in Ta$_{0.99}$Fe$_{0.01}$S$_2$, where the Mott transition is completely suppressed. This indicates that the melting of the Mott state accompanies the appearance of the domain structure. Since the number of walls increase in superconducting Ta$_{0.98}$Fe$_{0.02}$S$_2$, the domain walls seem to be responsible for superconductivity in iron doped 1$T$-TaS$_2$.

In general, no consensus has been reached regarding the diagnostic criteria for obstructive sleep apnoea syndrome (OSAS) in children and the criteria for selecting treatment are inconsistent. Therefore, the craniofacial and pharyngeal airway morphology of OSAS in children who had undergone drug therapy (non-op group) and OSAS in children who had undergone both drug therapy and surgical therapy (adenotonsillectomy) (op group) were compared. The purpose of this study was to examine the effectiveness of craniofacial morphology and pharyngeal airway morphology analysis in the treatment of children with OSAS.The craniofacial and pharyngeal airway morphology of the control group, the non-op group and the op group were compared to examine the differences of each group. The comparison used Mann-Whitney's U test.A comparison between the non-op and the op groups showed significant differences in the facial axis, mandibular plane angle, ramus plane to the SN (porion and orbit) point, point Pog (pogonion) to the McNamara line, anteroposterior dysplasia indicator (APDI), D-AD1 [the distance between the posterior nasal spine (PNS) point and the nearest adenoid tissue, measured along the PNS-Ba (basion) point plane], D-AD2 (the distance between the PNS point and the nearest adenoid tissue, measured along a line from the PNS point perpendicular to the S (sella turcica)-Ba point plane), upper pharynx and soft palatal length. The op group showed significantly lower values of APDI than the non-op group, indicating that the op group showed a significant occlusion of class II, and that the mandibular bone was positioned posteriorly relative to the maxillary bone.The op group showed a significant posterior position and backward rotation of the mandibular bone, stenosis of the nasopharyngeal airway and an elongated soft palate compared with the non-op group, and it was speculated that there was a high probability of the necessity of surgical therapy (adenotonsillectomy) when a morphological factor played a major role as a cause of obstructive sleep apnoea. We recommend craniofacial morphology analysis and pharyngeal airway morphology analysis in the diagnosis and treatment planning of OSAS children.

We have performed scanning tunneling microscopy on Fe1.07Te that is the parent compound of the iron-chalcogenide superconductors (SCs). The results indicate the existence of the unidirectional electronic order with a period of a 0 (a 0 interchalcogen distance) along one Te–Te direction. From the one-dimensionality of the electronic order, it is believed that the structural or antiferromagnetic transition is closely related to the formation of this electronic order. The results in this study will be crucial key in understanding the parent state of iron-chalcogenide SCs.

We have performed scanning tunneling spectroscopy on a single crystal of Bi2Sr2Ca(Cu1−x Zn x )2O8+δ (x=0.005) and found several regions where at least two Zinc (Zn) impurities aggregate within a few nm. In these regions, there are two-dimensional local density-of-states (LDOS) modulations with the period of about 1.41a 0 (a 0 is Cu–O–Cu distance) along the direction tilted 45° from Cu–O–Cu direction within the energy of below 10 mV. Since these modulations are suppressed in a map of the ratio between LDOS at a positive energy and at its negative, it is presumable that these modulations do not stem from the simple quasiparticle interference whose pattern should be enhanced in the ratio map. We suggest the possible explanations with respect to the origin of the observed modulations.

前庭水管拡大症 (EVA: enlargement of the vestibular aqueduct) は最も頻度の高い内耳奇形である。EVAが認められる疾患としては, 先天性で発症し進行する難聴やめまいを呈し, ヨード有機化障害による甲状腺腫を伴うPendred症候群が知られている。またPendred症候群以外の症候群性難聴や非症候性難聴で認められる場合もある。Pendred症候群の原因としてはSLC26A4 遺伝子変異が報告されている。これまで画像検査による前庭水管拡大の程度とPendred症候群に関連する臨床的特徴についての関係はまだ確立されていない。今回我々は両側性難聴を, 画像検査による前庭水管拡大の程度から確実例とボーダーライン例とに分類し, SLC26A4 遺伝子変異を含むPendred症候群に関連する検査所見を比較検討した。拡大確実例群において遺伝子変異が89%で認められ, ボーダーライン例群では33%であった。Mondini奇形は確実例群で67%に認めたが, ボーダーライン例群では33%であった。めまい症状は確実例群で33%に認め, ボーダーライン例の17%であった。甲状腺腫は確実例群で22%に認め, ボーダーライン群で17%であった。甲状腺機能低下は確実例群の成人例で1例を認めるのみであった。サイログロブリン (TG) の上昇は, 両群ともに検査症例中約50%に認め, その全例にSLC26A4 遺伝子変異が認められた。以上の結果は前庭水管拡大の確実例群とボーダーライン群では, 確実例群に遺伝子変異と強い相関を認め症例の病態の構成が異なることが予想されたが, 臨床症状には明らかな有意な差は認められなかった。

We reported two cases of Pelizaeus-Merzbacher disease. Both cases visited our hospital manifesting horizontal nystagmoid movements present from birth, and delayed motor development. Magnetic resonance imaging of the brain showed diffuse dysmyelination of the cerebral white matter, and auditory brainstem response showed waves I and II but absence of all subsequent components. Conditioned orientation reflex (COR) audiometry showed poor reactions in an infantile case whose development was severely retarded, and who spoke no meaningful words. Auditory steady-state response (ASSR) was a helpful tool for identifying her auditory ability; thereafter, her communication skills improved naturally. The other case was mildly developmentally retarded, and the results of COR audiometry and ASSR were considered the same level. The discrepancy between results of these hearing tests may arise under the influence of developmental level of the case.

当科で人工内耳埋込術を施行した小児内耳奇形14例の内耳奇形の形態分類, 術前聴力と補聴器装用下聴力レベル, 手術内容, また術後の聴取能力と言語獲得について検討した。内耳奇形の内訳は1例が外側半規管低形成, 4例が両側前庭水管拡大症 (EVA), 2例が common cavity (CC), 7例が incomplete partition (IP) であり, IP7例中2例に両側内耳道狭窄, 1例に両側前庭水管拡大症が認められた。人工内耳術後の顔面神経麻痺, 髄膜炎, 電極脱落例は無かった。両側内耳道狭窄例は2例とも人工内耳装用下の語音聴取能改善が難しく, 言語発達のために術後に視覚言語を併用していた。CCの2症例のうち1例は術後語音聴取能, 言語表出が良好となったが, 1例は言語獲得不良であった。IP, 外側半規管低形成, 両側前庭水管拡大症例は, 全例術後の音声言語コミュニケーションが良好となった。

The spatial evolution of the background conductance in the tunneling spectra was investigated with low-temperature scanning tunneling spectroscopy on a slightly overdoped Bi 2 Sr 1.74 La 0.26 CuO 6+δ single crystal at 4.2 K. The asymmetry in the background conductance between positive and negative biases strongly correlates with the local energy gap, which shows the inhomogeneous spatial variation: the tunneling spectra become more asymmetric in the regions where the spectra exhibit larger gap value.

We measured the dielectric dispersion in multi-ferroic TbMnO 3. We observed two kinds of the dielectric dispersions. One dispersion showed the monotonous temperature dependence of the relaxation frequency across the ferroelectric transition temperature, Tc. This dispersion is thought to be originated from the localized charge. The other dispersion existed only near the Tc, attributed to the ferroelectric transition. We found the former dispersion enhanced its strength near Tc. This indicates that the localized charge couples with the electric moment which orders in the ferroelectric phase.

Here, we report the case of a male child with achondroplasia who was diagnosed with obstructive sleep apnea and underwent adenoidectomy and tonsillectomy. By analyzing lateral cephalograms, we evaluated the craniofacial and pharyngeal airway morphology immediately before surgery (age, 5 years 6 months) and 1 year 2 months after surgery (age, 6 years 8 months). Adenoidectomy and tonsillectomy dilated the pharynx and improved the craniofacial and pharyngeal morphologies, apparently thus improving the sleep apnea. (J. Oral Sci. 49, 173-177, 2007)

Objective To analyze the difficulty of discriminating Japanese nonsense monosyllables in each of several grades of high-frequency hearing loss and to evaluate the limitations of amplification. Methods We collected retrospective data on the discrimination of Japanese nonsense monosyllables by patients with three grades of high-frequency hearing loss who fulfilled or nearly fulfilled the Japanese criteria for EAS. Discrimination of the twenty monosyllables included in the 67-S speech audiometric test, which is approved by the Japan Audiological Society, was evaluated under quiet conditions. Results One hundred and five ears of ninety-one adults with high-frequency hearing loss were tested. We classified the ears according to hearing threshold at 1000 Hz; Group 1

We have investigated correlation between structural and electronic anisotropies in a parent compound of Fe-chalcogenide superconductor ${\mathrm{Fe}}_{1+\ensuremath{\delta}}\mathrm{Te}$ with $\ensuremath{\delta}=0.09$ by means of synchrotron x-ray diffraction and in situ in-plane resistivity anisotropy measurements with uniaxial stress applied along a tetragonal $a$ axis. This system is known to exhibit a tetragonal-to-monoclinic structural transition at ${T}_{\mathrm{S}}\ensuremath{\sim}60$ K. We have confirmed that the in-plane resistivity anisotropy in the low-temperature monoclinic phase is attributed to the asymmetry in volume fractions of the monoclinic domains, as was suggested in a previous study [Jiang et al., Phys. Rev. B 88 115130 (2013)]. On the other hand, we found another in-plane resistivity anisotropy above ${T}_{\mathrm{S}}$. The present x-ray diffraction and resistivity anisotropy measurements have revealed that this anisotropy is not due to an onset of the low-temperature monoclinic phase but to the lattice softening enhanced toward ${T}_{\mathrm{S}}$. As one of the possibilities, we suggest that the orbital fluctuation contributes to the lattice softening and the resistivity anisotropy above ${T}_{\mathrm{S}}$.

We evaluated the craniofacial and airway morphology in children with achondroplasia complicated by snoring and apnea during sleep (AP group) in comparison with children with snoring and apnea during sleep without chromosomal aberrations (adenoid group) and healthy children without sleep disordered breathing or malalignment (healthy group). Lateral cephalograms in 10 children each (four males and six females) in the three groups were analyzed. When the AP and healthy groups were compared, the AP group showed significantly lower values for facial depth, nasal floor length, point A, point pog, and saddle angle (p