Ipsilateral Hypertrophy of the Mastoid Process in Surgical Cases of Congenital Muscular Torticollis.

OBJECTIVE: This study was to investigate ipsilateral hypertrophy of the mastoid process in the patients with congenital muscular torticollis (CMT). DESIGN: Retrospective cross-sectional study. PATIENTS: Children with CMT. METHODS: A total of 212 surgical cases of patients with CMT (age: 50.9 ± 44.3 months) and 212 age- and gender-matched controls (age: 50.4 ± 44.2 months) were included. The mastoid process volume was calculated and compared for both groups on the computed tomography axial images. A linear regression analysis was performed between the age and the intrasubject volume difference in the mastoid process. RESULTS: The volume of the mastoid process in the CMT side was significantly larger than that of the non-CMT side in the CMT group (32.2 ± 30.3 cm3 vs 21.9 ± 22.8 cm3; P < .001). In the control group, there was no significant difference between the volume of the right and left mastoid process (21.6 ± 24.6 cm3 vs 21.2 ± 23.8 cm3; P = .472). The intrasubject volume difference in the mastoid process in the CMT group showed a linear increase with the age (adjusted R2 = 0.286; P < .001), and the volumetric asymmetry of the mastoid process became more severe over age compared to the control group. CONCLUSION: We showed ipsilateral hypertrophy of the mastoid process in patients with CMT and demonstrated that the volumetric asymmetry increased with age.

Association of hyperuricemia and pulmonary hypertension: A systematic review and meta-analysis.

Objectives: To investigate the association of hyperuricemia with pulmonary hypertension (PH) in term of subsequent development, severity, and prognosis of PH.Methods: The authors systematically reviewed articles from databases and conducted meta-analyses as follows: (1) association of serum uric acid (UA) levels with the presence of PH; (2) association between serum UA levels and subsequent development of PH, in terms of odds ratio of the development of PH; and (3) association of serum UA levels with severity and prognosis of PH, in terms of pulmonary arterial pressure and hazard ratio of death.Results: Twenty-six studies published between 1999 and 2017 were included. The level of serum UA was higher in subjects with PH than subjects without PH and this finding was observed regardless of status of diuretics use or renal function. The odds ratio of the development of PH was 2.32 (95% CI, 1.05-5.15) in subjects with hyperuricemia. Pulmonary arterial pressure among subjects with PH was also higher in subjects with hyperuricemia than subjects with normouricemia. There was a 19% increased hazard ratio of death (95% CI, 1.06-1.33) among patients with PH who had hyperuricemia.Conclusion: Hyperuricemia is associated with the subsequent development, worse severity and poor prognosis of PH.

Identification of a rare homozygous c.790C>T variation in the TFB2M gene in Korean patients with autism spectrum disorder.

Mitochondrial dysfunction and subsequent enhanced oxidative stress is implicated in the pathogenesis of autism spectrum disorder (ASD). Mitochondrial transcription factor B2 (TFB2M) is an essential protein in mitochondrial gene expression. No reports have described TFB2M mutations and variations involved in any human diseases. We identified a rare homozygous c.790C>T (His264Tyr) variation in TFB2M gene in two Korean siblings with ASD by whole-exome sequencing. The roles of the TFB2M variation in the pathogenesis of ASD were investigated. Patient fibroblasts revealed increased transcription of mitochondrial genes and mitochondrial function in terms of ATP, membrane potential, oxygen consumption, and reactive oxygen species (ROS). Overexpression of the TFB2M variant in primary-cultured fibroblasts demonstrated significantly increased transcription of mitochondrial genes and mitochondrial function compared with overexpression of wild-type TFB2M. Molecular dynamics simulation of the TFB2M variant protein suggested an increase in the rigidity of the hinge region, which may cause alterations in loading and/or unloading of TFB2M on target DNA. Our results suggest that augmentation of mitochondrial gene expression and subsequent enhancement of mitochondrial function may be associated with the pathogenesis of ASD in Korean patients.

Torticollis Caused by Nontraumatic Craniovertebral Junction Abnormalities.

Torticollis could be the only symptom and sign of craniovertebral junction (CVJ) abnormality. It could be difficult to identify CVJ abnormality as a cause of torticollis due to their rarity, especially for the subjects with torticollis caused by nontraumatic CVJ abnormalities. There has been no report to focus on nontraumatic CVJ abnormalities as a cause of torticollis. The objective of this study was to report 27 patients of torticollis caused by nontraumatic CVJ abnormalities, with the aim of helping clinicians to identify nontraumatic CVJ abnormalities as a cause of torticollis. This is a retrospective cohort study including 27 subjects who had torticollis caused by nontraumatic CVJ abnormalities. The CVJ was examined in terms of atlanto-occipital angle, atlanto-axial angle, and lateral and anterior atlanto-dens intervals for the evaluation of occipital condylar hypoplasia, rotation of atlanto-axial joint, and lateral and anterior shift of the dens, respectively. Abnormalities of the lower cervical or thoracic spine were also evaluated. Occipital condylar hypoplasia, rotation of atlanto-axial joint, and lateral shift of the dens were the most common CVJ abnormalities. The 18.5% of the subjects had concurrent anomalies of lower cervical or thoracic vertebrae along with CVJ abnormalities. Each subject had 2.22 ±â€Š1.10 types of CVJ abnormalities on average. In conclusion, comprehensive evaluation of CVJ abnormalities is recommended for differential diagnosis of subjects with unexplained torticollis. Once CVJ abnormalities are identified, concurrent abnormalities of other vertebrae need to be evaluated.

Craniovertebral Junction Abnormalities in Surgical Patients With Congenital Muscular Torticollis.

Our clinical experience led us to realize that craniovertebral junction (CVJ) abnormalities were common in surgical patients with congenital muscular torticollis (CMT). This study aimed to report the concurrence rate of CVJ abnormalities in surgical patients with CMT, along with comprehensive evaluation of type of concurrent CVJ abnormalities. This was a retrospective cohort study in a tertiary hospital, including 41 subjects who underwent surgical release for CMT at the mean age of 8.38 years. The presence of CVJ abnormalities was analyzed, using craniofacial 3-dimensional computed tomography images. The concurrence rate of CVJ abnormalities was 70% in surgical patients with CMT. Subjects with CVJ abnormalities had, on average, 1.48 abnormalities. The CVJ abnormalities were rotation and lateral shift of the atlanto-axial joint along with rotation of atlanto-occipital joint, where rotation of the atlanto-axial joint was most common (82.76%). There is no patient with anterior shift of the atlanto-axial joint. Chronic mechanical tension by the contracted unilateral sternocleidomastoid muscle of CMT could be responsible for concurrent CVJ abnormalities. The CVJ abnormalities are more common in the atlanto-axial joint than in the atlanto-occipital joint. In conclusions, CVJ abnormality seems to be a common concurrent skeletal complication of CMT, at least, in surgical patients. The CVJ abnormality might be included in the list of skeletal complications of CMT. If CVJ abnormalities are significantly more common in surgical patients with CMT, CVJ abnormalities might be one of predictors of surgical patients with CMT.

The first Korean patient with Potocki-Shaffer syndrome: a rare cause of multiple exostoses.

Potocki-Shaffer syndrome (PSS, OMIM #601224) is a rare contiguous gene deletion syndrome caused by haploinsufficiency of genes located on the 11p11.2p12. Affected individuals have a number of characteristic features including multiple exostoses, biparietal foramina, abnormalities of genitourinary system, hypotonia, developmental delay, and intellectual disability. We report here on the first Korean case of an 8-yr-old boy with PSS diagnosed by high resolution microarray. Initial evaluation was done at age 6 months because of a history of developmental delay, hypotonia, and dysmorphic face. Coronal craniosynostosis and enlarged parietal foramina were found on skull radiographs. At age 6 yr, he had severe global developmental delay. Multiple exostoses of long bones were detected during a radiological check-up. Based on the clinical and radiological features, PSS was highly suspected. Subsequently, chromosomal microarray analysis identified an 8.6 Mb deletion at 11p11.2 [arr 11p12p11.2 (Chr11:39,204,770-47,791,278)×1]. The patient continued rehabilitation therapy for profound developmental delay. The progression of multiple exostosis has being monitored. This case confirms and extends data on the genetic basis of PSS. In clinical and radiologic aspect, a patient with multiple exostoses accompanying with syndromic features, including craniofacial abnormalities and mental retardation, the diagnosis of PSS should be considered.

Identification of the rare compound heterozygous variants in the NEB gene in a Korean family with intellectual disability, epilepsy and early-childhood-onset generalized muscle weakness.

We examined a Korean family with complex phenotypes characterized by intellectual disability, epilepsy and early-childhood-onset generalized muscle weakness. Since we did not find any abnormality using several conventional genetic tests for detection of chromosomal aberrations, gene copy number variations and mitochondrial gene mutations, we aimed to identify disease-causing genetic alteration(s) in this family. We conducted whole-exome sequencing (WES) in this family. After filtering the WES data, we compared five exome sequences of two affected siblings, one unaffected sibling and the unaffected parents, and we determined the allele frequency of the identified variants in an Asian population. Finally, we selected one candidate variant pair which is unique in the patients and corresponds to an autosomal recessive genetic model. The two affected siblings had the same compound heterozygous variation in the NEB gene encoding nebulin, which was composed of two different missense variants: c.2603T>C (p.L868P) in exon 27 and c.21340C>T (p.R7114W) in exon 143. We confirmed these variations by Sanger sequencing. On the basis of the fundamental role of nebulin in the brain and skeletal muscles, we concluded that this compound heterozygous NEB variation may be a sound candidate for the disease-causing mutation in this family. Since the patients are characterized by generalized muscle weakness together with neurodevelopmental phenotypes, it is suggested that NEB mutations could manifest more diverse phenotypes than those previously described.

Reciprocal deletion and duplication of 17p11.2-11.2: Korean patients with Smith-Magenis syndrome and Potocki-Lupski syndrome.

Deletion and duplication of the -3.7-Mb region in 17p11.2 result in two reciprocal syndrome, Smith-Magenis syndrome and Potocki-Lupski syndrome. Smith-Magenis syndrome is a well-known developmental disorder. Potocki-Lupski syndrome has recently been recognized as a microduplication syndrome that is a reciprocal disease of Smith-Magenis syndrome. In this paper, we report on the clinical and cytogenetic features of two Korean patients with Smith-Magenis syndrome and Potocki-Lupski syndrome. Patient 1 (Smith-Magenis syndrome) was a 2.9-yr-old boy who showed mild dysmorphic features, aggressive behavioral problems, and developmental delay. Patient 2 (Potocki-Lupski syndrome), a 17-yr-old boy, had only intellectual disabilities and language developmental delay. We used array comparative genomic hybridization (array CGH) and found a 2.6 Mb-sized deletion and a reciprocal 2.1 Mb-sized duplication involving the 17p11.2. These regions overlapped in a 2.1 Mb size containing 11 common genes, including RAI1 and SREBF.

The usefulness, reliability, and quality of YouTube video clips on congenital muscular torticollis: A STROBE compliant study.

This study aimed to evaluate the usefulness, reliability, quality, and related characteristics of YouTube video clips on congenital muscular torticollis (CMT). This cross-sectional study analyzed 47 YouTube video clips on CMT. They were classified as either useful or misleading by 2 rehabilitation doctors. The modified DISCERN tool and the Global Quality Scale (GQS) were used to evaluate their reliability and quality. An analysis was conducted using the characteristics, such as presenters, ownership of YouTube channel accounts, countries, contents, and the video popularity. Of the 47 YouTube video clips, 8 (17%) were evaluated as misleading, which indicated that they included at least one scientifically unproven piece of information on CMT or more. They were less reliable and of lower quality than the useful video clips. The video clips presented by healthcare professionals were more useful compared to those presented by others (P = .015). However, the video popularity was not related to its usefulness. The reliability and quality (3.70 ± 0.82 vs 0.75 ± 0.50 and 2.95 ± 1.21 vs 1.50 ± 1.00) assessed by the modified DISCERN tool and GQS, respectively, were significantly higher in the video clips presented by healthcare professionals compared to those presented by others. There were misleading YouTube video clips on CMT. Video clips presented by healthcare professionals could be more useful, reliable, and of better quality. The popularity of the video clips does not indicate more usefulness, reliability, and better quality. YouTube viewers should be aware of these findings. We recommend that the viewers preferentially choose video clips on CMT presented by healthcare professionals, not by the video popularity.

Effects of abdominal drawing-in maneuver with or without prior iliopsoas stretching on gluteus maximus activity during prone hip extension.

BACKGROUND: Prone hip extension (PHE) has been investigated to strengthen the hip joint and back extensor muscles. However, it has not been compared with various PHE exercises in individuals with iliopsoas shortness. OBJECTIVE: This study compared pelvic compensation and hip and back extensor muscle activities in individuals with iliopsoas shortness during prone hip extension (PHE) using the abdominal drawing-in maneuver alone (PHEA) and after iliopsoas stretching (PHEAS). METHODS: Twenty-five individuals with iliopsoas shortness were included in the study. Electromyography was used to investigate bilateral erector spinae (ES) and ipsilateral gluteus maximus (GM), biceps femoris (BF), and semitendinosus (ST) muscles during PHE, PHEA, and PHEAS. Pelvic anterior tilting and rotation angles were measured during each PHE exercise via electromagnetic motion tracking. A modified Thomas test was used to examine the hip extension angle before and after iliopsoas stretching. One-way repeated-measures analysis of variance was used to investigate differences in pelvic anterior tilting and rotation angle and in hip and back extensor muscle activities among PHE, PHEA, and PHEAS. The level of statistical significance was set at α= 0.01. RESULTS: GM muscle activity was significantly greater with PHEAS, compared to PHE and PHEA (p< 0.01). Bilateral ES and ipsilateral BF and ST muscle activities were significantly reduced with PHEAS, compared to PHE and PHEA (p< 0.01). Anterior pelvic tilting and rotation angles were significantly reduced with PHEAS, compared to PHE and PHEA (p< 0.01). CONCLUSIONS: PHEAS is recommended to selectively strengthen GM muscles with minimal BF and ST muscle activities and pelvic compensation in individuals with iliopsoas shortness. The abdominal drawing-in maneuver (ADIM) after iliopsoas stretching is more efficient than ADIM alone during PHE, especially in individuals with iliopsoas shortness.

Sternocleidomastoid muscle asymmetry in unilateral congenital superior oblique palsy.

BACKGROUND: To determine whether there is an asymmetry in bilateral sternocleidomastoid muscle (SCM) thickness in patients with unilateral congenital superior oblique palsy (SOP) and its association with surgical results. METHODS: The medical records of 186 patients with head tilt secondary to unilateral SOP, who were evaluated for the status of the SCM with neck ultrasound or magnetic resonance imaging, were reviewed. The SCM asymmetry index was calculated as a bilateral difference in the maximal muscle thickness divided by each tilted-side SCM thickness. The presence of SCM asymmetry, defined as an index of >10%, and its relationship to residual torticollis ≥5° after SOP surgery were assessed. RESULTS: Of 186 patients with a median age of 1.2 years, SCM asymmetry was present in 102 (54.8%) patients (6.8 ± 1.9 mm for the SOP side vs. 6.6 ± 2.1 mm for the tilted side). The SCM asymmetry did not differ according to age, amount of head tilt or hypertropia. In the patients with SCM asymmetry, more patients (87.3%) underwent physiotherapy than those without asymmetry (61.9%) (P = 0.021). In 99 patients who underwent surgery for SOP, the resolution of torticollis was not significantly different between patients with and without SCM asymmetry (87.2% vs. 76.9%, P = 0.184). CONCLUSIONS: Nearly half of the patients with congenital SOP had SCM thickness asymmetry that was already determined at a young age. However, the surgical results did not differ significantly with respect to SCM asymmetry when physiotherapy was combined. Thus, SOP surgery can be considered despite preoperative SCM asymmetry.

Effect of combined pulmonary fibrosis and emphysema on patients with connective tissue diseases and systemic sclerosis: a systematic review and meta-analysis.

BACKGROUND: This study aimed to analyze the literature systematically to determine the clinical characteristics and prognosis of patients with connective tissue disease (CTD) with combined pulmonary fibrosis and emphysema (CPFE) compared to those of patients with CTD-interstitial lung disease (CTD-ILD) without emphysema. METHODS: We searched MEDLINE, EMBASE, Cochrane Library, and KoreaMed for relevant articles published before July 2019. Studies meeting all the following criteria were included: (1) original research studies evaluating the effect of CPFE on CTD, (2) studies that compared patients with CTD-CPFE to those with CTD-ILD without emphysema, and (3) studies providing data on physical capacity, pulmonary function, or death in patients with CTD. Clinical characteristics of patients with CTD-CPFE were compared with those of patients with CTD-ILD without emphysema, and the influence of CPFE on physical capacity, pulmonary function, and death was analyzed. RESULTS: Six studies between 2013 and 2019 were included. Two hundred ninety-nine (29.5%) and 715 (70.5%) patients had CTD-CPFE and CTD-ILD without emphysema, respectively. Regarding the type of CTD, 711 (68.3%) patients had systemic sclerosis, 263 (25.3%) rheumatoid arthritis, and 67 (6.4%) other CTDs. Patients with CTD-CPFE had a higher frequency of pulmonary hypertension and pulmonary fibrosis > 20% of the total lung volume, higher ratio of the forced vital capacity to the diffusion capacity of the lung for carbon monoxide (DLCO), lower arterial oxygen pressure at rest, and lower DLCO compared to those in patients with CTD-ILD without emphysema. In addition, more deaths occurred among those with CTD-CPFE (odds ratio, 2.95; 95% confidence interval, 1.75-4.96). CONCLUSION: CTD-CPFE is associated with worse physical and pulmonary function and more deaths compared to those in CTD-ILD without emphysema. These findings indicate the need for increased awareness and close monitoring of patients with CTD-CPFE.

Korean Academy of Medical Sciences Pediatric Impairment Guideline for brain lesion.

The permanent impairment evaluation for children in developmental stage is very difficult and it is even impossible in some cases. The impairment evaluation for developing children has not yet been included in the guideline of the American Medical Association. Due to frequent medical and social demands in Korea, we developed an impairment evaluation guideline for motor impairment, intellectual disability/mental retardation, developmental speech-language disorder and epilepsy caused by pediatric cerebral injuries, or cerebral lesions other than the developmental disorders such as autism. With the help of various literature and foreign institutions, we developed our in order to develop a scientific guideline for pediatric impairment that is suited to Korean cultural background and social condition.

Which growth parameters can affect mortality in cerebral palsy?

BACKGROUND: The objective of this study was to identify growth parameters that can affect mortality of cerebral palsy (CP). METHOD: This was a birth cohort study based on the National Health Screening Program for Infants and Children database along with the National Health Insurance Service, which were linked using a personal identifier number. The birth cohort consisted of 2 191 956 subjects, representing 93.5% of live births from 2007-2011, with maximal 10-year follow-up (range, 5-10 years) until October 2016. Subjects with CP were identified. Growth parameters in terms of birth weight, underweight (weight-for-age below the 3rd percentile), rate of body weight gain were collected, along with all-cause mortality after the age of 1 year. RESULT: Prevalence of CP was 2.0 per 1000 live births (95% CI, 1.94-2.06). All-cause mortality after the age of 1 year was 0.09 deaths/1000 person-years (95% CI, 0.08-0.09) in the general population (GP) and 2.85 deaths/1000 person-years (95% CI, 2.32-3.50) in subjects with CP during the follow-up. Therefore, the incidence rate ratio for all-cause mortality was 32.15 (95% CI, 25.72-39.76) in subjects with CP compared to GP. Presence of underweight was significantly associated with higher mortality in both subjects with CP and GP, where the adjusted hazard ratio of death was 2.60 (95% CI, 1.93-3.50) at the age of 18-24 months, 3.12 at 30-36 months, 4.37 at 42-48 months, 5.12 at 54-60 months, and 4.17 at 66-71 months. Birth weight did not affect mortality in both subjects with CP and GP after the age of 1 year (p > 0.05). CONCLUSION: While subjects with CP shows higher mortality, underweight is an important growth parameter that affects all-cause mortality of both subjects with CP and GP. This study urges increased awareness that subjects with CP who are underweight require special care.

Contralateral Involvement of Congenital Muscular Torticollis and Clavicular Fracture.

Congenital muscular torticollis (CMT) is known to concur with some conditions such as developmental dysplasia of the hip or brachial plexus injury, which gives us some insights for pathogenesis of CMT. Although clavicular fracture is the most common fracture in newborns, little is known about concurrence of CMT and clavicular fracture. Our clinical experience led us to realize that concurrence of CMT and clavicular fracture tended to occur on the contralateral side for each other rather than the ipsilateral side. This study aimed to verify contralateral concurrence of CMT and clavicular fracture. This is a retrospective cohort study in a tertiary hospital, including 996 subjects with CMT. Concurrent clavicular fracture was found in 20 of 996 subjects with CMT, with the concurrence rate being 2.01%. Concurrent clavicular fracture and clavicular fracture occurred on the contralateral side for each other in 18 subjects (90%) rather than the ipsilateral side. This contralateral concurrence between side of CMT and clavicular fracture was significant (P = 0.001), with an odds ratio of 81 (P = 0.0032). Concurrent clavicular fracture and clavicular fracture seem to occur significantly more on the contralateral side for each other. Underlying mechanism for consistent contralateral concurrence needs to be verified in the near future.

Rare Concurrence of Congenital Muscular Torticollis and a Malignant Tumor in the Same Sternocleidomastoid Muscle.

While congenital muscular torticollis (CMT) can occur along with other conditions, such as clavicle fracture or brachial plexus injury, these conditions exist outside the sternocleidomastoid muscle (SCM). We present a rare case with concurrence of CMT and a malignant tumor inside the same SCM, along with serial clinical and radiological findings of the atypical features of CMT. The malignant tumor was in fact a low-grade fibromyxoid sarcoma. To the best of our knowledge, the current case is the first of a concurrent condition of CMT inside the SCM. This case suggests that concurrent conditions could exist either inside or outside the SCM with CMT. Therefore, a thorough evaluation of SCM is required when subjects with CMT display atypical features, such as the increase of mass or poor response to conservative therapy. In that case, appropriate imaging modalities, such as ultrasonogram or magnetic resonance imaging, are useful for differential diagnosis.

A Case of Grisel Syndrome Showing No Underlying Laxity of the Atlanto-axial Joint.

Grisel syndrome is a rare, non-traumatic atlanto-axial subluxation associated with an inflammatory or infectious process in the upper neck. According to the two-hit hypothesis, which is widely accepted for the pathogenesis of Grisel syndrome, preexisting ligamentous laxity of the atlanto-axial joint is regarded as the first hit. An inflammatory or infectious process of the atlanto-axial joint acts as the second hit, resulting in non-traumatic atlanto-axial subluxation. We report on a 6-year-old girl with atlanto-axial subluxation following retropharyngeal and cervical lymphadenitis. She was diagnosed with Grisel syndrome, for which an initial computed tomography did not show any preexisting ligamentous laxity of the atlanto-axial joint. A literature review found only 4 case reports on Grisel syndrome with an initially normal atlanto-axial joint. The present case offers some evidence that a single hit, such as inflammatory changes in the atlanto-axial joint, might cause Grisel syndrome, even without underlying ligamentous laxity.

Association between acute gouty arthritis and meteorological factors: An ecological study using a systematic review and meta-analysis.

OBJECTIVES: The aims of this study were as follows: (1) to analyze the literature systematically regarding the seasonal and monthly variation of the occurrence of episodes of acute gouty arthritis, and (2) to investigate the relationship between the occurrence of episodes of acute gouty arthritis and meteorological parameters. METHODS: The present authors systematically reviewed databases for articles published before November 2015. Studies with quantitative data on episodes of acute gouty arthritis by months and/or seasons were included. Meteorological data such as the highest temperature, lowest temperature, diurnal temperature range, change in mean temperature between neighboring days, relative humidity and wind speed for the geographic place(s), and study period where and when each study took place were obtained from meteorological websites. RESULTS: Ten studies published between 1920 and 2015 were included. A meta-analysis by season showed that acute gouty arthritis occurred significantly more frequently in spring than in other seasons. Analysis by month showed an increase in episodes of acute gouty arthritis from March to July, being the highest in July. The trend reversed, and episodes of acute gouty arthritis started decreasing from July to September, being the lowest in September. The change in mean temperature between neighboring days was the only meteorological parameter that was significantly correlated with the number of monthly episodes of acute gouty arthritis. CONCLUSIONS: Acute gouty arthritis seems to develop more frequently during the period in which the temperature increases significantly between neighboring days: spring by season and between March and July by month in the northern hemisphere.

Effectiveness of Ear Splint Therapy for Ear Deformities.

OBJECTIVE: To present our experience with ear splint therapy for babies with ear deformities, and thereby demonstrate that this therapy is an effective and safe intervention without significant complications. METHODS: This was a retrospective study of 54 babies (35 boys and 19 girls; 80 ears; age ≤3 months) with ear deformities who had received ear splint therapy at the Center for Torticollis, Department of Physical Medicine and Rehabilitation, Ajou University Hospital between December 2014 and February 2016. Before the initiation of ear splint therapy, ear deformities were classified with reference to the standard terminology. We compared the severity of ear deformity before and after ear splint therapy by using the physician's ratings. We also compared the physician's ratings and the caregiver's ratings on completion of ear splint therapy. RESULTS: Among these 54 babies, 41 children (58 ears, 72.5%) completed the ear splint therapy. The mean age at initiation of therapy was 52.91±18.26 days and the treatment duration was 44.27±32.06 days. Satyr ear, forward-facing ear lobe, Darwinian notch, overfolded ear, and cupped ear were the five most common ear deformities. At the completion of therapy, the final physician's ratings of ear deformities were significantly improved compared to the initial ratings (8.28±1.44 vs. 2.51±0.92; p<0.001). There was no significant difference between the physician's ratings and the caregiver's ratings at the completion of ear splint therapy (8.28±1.44 vs. 8.0±1.61; p=0.297). CONCLUSION: We demonstrated that ear splint therapy significantly improved ear deformities in babies, as measured by quantitative rating scales. Ear splint therapy is an effective and safe intervention for babies with ear deformities.