Protective effects of anise in prevention of cerebral ischemia reperfusion injury in rats.

The aim of this study is to determine the protective efficacy of anise in cerebral ischemia and reperfusion injury in rats. In this study, 28 Wistar Albino rats, weighing 250-300 grams (g), were used. Four groups were formed with 7 rats in each group. Group 1 (n=7): Control group, Group 2 (n=7): Anise group, 5 mL/kg/day of anise aqueous extract prepared according to Gamberini's protocol was given orally by gavage for 30 days. Group 3 (n=7): Cerebral ischemia reperfusion (CIR) group, at the beginning of the experiment, 30 minutes of cerebral ischemia and 1 hour of reperfusion were induced and the animals were sacrificed by exanguination. Group 4 (n=7): Anise+ CIR group, After administering 30 days of anise's aqueous extract, CIR was induced and the study was terminated. TOS values of the Anise+ CIR group was significantly lower than that of the CIR group (p<0.05). Il-6 and TNF-α values of the CIR group were significantly higher than the Anise+ CIR group (p<0,05). Our study revealed that anise ameliorates oxidative damage and inflammation due to cerebral ischemia/reperfusion, by reducing the levels of inflammatory cytokines (TNF-α, Il-6).

Clinical characteristics of 47 children with pigmentary mosaicism: A cross-sectional study.

BACKGROUND: Pigmentary mosaicism (PM), also known as Blaschkoid dyspigmentation, is a rare pigmentary anomaly. Although several case reports have been published describing extracutaneous manifestations associated with PM, there are very few studies on the clinical characteristics of patients with PM. AIM: To describe the clinical characteristics of patients with PM. PATIENTS AND METHODS: This descriptive cross-sectional study was conducted among 47 children examined by a dermatologist and a pediatrician. The pattern and location of the PM, type of pigmentation and extracutaneous manifestations were documented. RESULTS: The most common pattern of PM was narrow-band PM, followed by broad-band and checkerboard patterns. The trunk was the most affected region, followed by the legs and arms. PM manifested as hypopigmentation in 51.1% of cases, as hyperpigmentation in 27.6%, and as hypo/hyperpigmentation in 21.2%. Accompanying diseases were present in 40.4% of patients: neuropsychiatric diseases were the most common, followed by endocrinological or hematological diseases and growth/developmental delay. CONCLUSION: PM has been associated with several extracutaneous findings but there is still some debate whether these associations reflect different PM phenotypes or whether they are simply coincidental. Our study suggests that extracutaneous involvement in PM patients is frequent, thus warranting careful examination of PM patients.

MANAGEMENT PRINCIPLES OF INCIDENTAL THYROID 18F-FDG UPTAKE IDENTIFIED ON 18F-FDG PET/CT IMAGING.

Purpose: The purpose of this study is to determine the clinical significance of incidental thyroid 18F-FDG PET/CT uptake in oncology patients with the focus achieving the most appropriate management of this challenging situation. Materials and method: Two thousand five hundred and eighty 18F-FDG PET/CT studies performed at our institute in the past 4 years were retrospectively reviewed. Patients with incidental FDG uptake in the thyroid gland were further analysed. Results: The prevalence of incidental FDG uptake in thyroid gland was 7.6% (129 patients). 26 patients (20.1%) had diffuse 18F-FDG PET/CT uptake, 103 patients (79.1%) had nodular uptake in thyroid gland. All diffuse uptake patients who were further examined diagnosed to be a benign condition. 53 patients in the nodular uptake group were further examined and the final histopathology examinations revealed an 18.8% malignancy rate. SUV max values ranged from 2 to 21.8 with a significant highness in malignant lesions. Conclusion: 18F-FDG PET/CT uptake in the thyroid gland may be diffuse or nodular. Diffuse uptake needs no further examination as it usually accompanied by benign thyroid disorders. Patients with nodular uptake whose general condition is good should be further examined due to high rates of malignancy.

Evaluation of follow-up colonoscopy in acute colonic diverticulitis for detection of advanced adenoma and colon cancer: a retrospective cohort study.

BACKGROUND: Due to variable rates of colon carcinoma or advanced adenoma mimicking an acute diverticulitis episode, the necessity of colonoscopy to detect colon cancer or advanced adenoma remains to be explored. This study investigated the incidence and predictive factors of colon cancer or advanced adenoma following acute diverticulitis. METHODS: We evaluated retrospectively all consecutive patients with an episode of computed tomography-proven acute diverticulitis between June 2016 and August 2019. A follow-up colonoscopy was performed. Demographic and clinical parameters were recorded. Patients with clinically substantial colonic neoplasia (colon cancer or advanced adenoma) were classified as Group A, while Group B included patients without clinically significant colonic neoplasia. The incidence of clinically significant colonic neoplasia in acute diverticulitis patients was regarded as the primary outcome. RESULTS: The mean age of 233 patients with acute diverticulitis was 58.6 ± 12.7 years. Complicated diverticulitis was detected in 39 patients (16.7 %). Sixteen patients (6.9 %) were assigned to Group A and 217 patients (93.1 %) to Group B. The age of the patients in group A was significantly higher than in Group B (p =0.001). Age above 50 and 65 years was also significantly associated with clinically significant colonic neoplasia (p =0.015 and p =0.012, respectively). The other variables did not influence the development of clinically significant colonic neoplasia (p >0.05). CONCLUSIONS: Colonoscopy examination following an episode of acute diverticulitis may not be recommended for all patients due to the rare occurrence of colon cancer or clinically significant colonic neoplasia in those younger than 50 years. HIPPOKRATIA 2021, 25 (2):69-74.

Significance of Gastric Wall Thickening Detected in Abdominal CT Scan to Predict Gastric Malignancy.

BACKGROUND: Early diagnosis of gastric cancer is one of the most important parameters affecting the survival of the disease. In this study, we aimed to stress the importance of antrum wall thickness in CT examination. METHOD: The study included 111 patients between ages of 18 and 95 who had antral wall thickening in computed tomography and also had endoscopic evaluation performed in the same clinic. The patients were divided into two groups as benign and malignant according to the pathology results. The thickness of the antrum wall in computed tomography, hemoglobin and albumin levels, and age was compared among these two groups. Parameters with significant differences were further analyzed by multivariate analysis using logistic regression analysis. RESULTS: Of the 111 patients included in the study, 57 were male and 54 were female. Mean age was 65 years. Fifty-one patients were classified as benign and 60 patients as malignant. Mean age of the malignant patients was 70, while that of benign patients was 59 (p < 0.05). Antrum wall thickness was 13.68 ± 3.27 mm in malignant patients and 9.22 ± 2.17 mm in benign patients (p < 0.05). Similarly, hemoglobin level was significantly different in malignant and benign patients (10.78 ± 1.57 g/dl and 12.64 ± 1.43 g/dl, respectively; p < 0.05). Albumin levels were 3.36 ± 0.57 mg/dl in malignant patients and 3.97 ± 0.57 mg/dl in benign patients (p < 0.05). CONCLUSION: Evaluation of antrum wall thickness, age, hemoglobin, and albumin values together may contribute to distinguishing the benign and malignant pathologies involving this region in patients with suspected stomach wall thickening in abdominal CT scan.

Colonic Wall Thickening Reported in Abdominal CT: Does It Always Imply Malignancy?

AIM/BACKGROUND: Early diagnosis of patients with colon cancer is one of the most important parameters affecting the survival of patients. In this study, we aimed to examine the effect of the age, hemoglobin (Hb), albumin, neutrophil lymphocyte ratio (NLR), thrombocyte lymphocyte ratio (PLR), and mean platelet values (MPV) on the separation of benign and malignant diseases in patients with suspected colon wall thickness (CWT) observed in abdominal computed tomography (CT) examination. METHOD: The study included 116 patients between the ages of 18 and 95 who had CT examination where the colon wall could be evaluated and who also had colonoscopy. Patients suspected for CWT in CT with difficulties in differential diagnosis were divided into two groups according to colonoscopic-histopathological evaluations. Normal or benign pathological causes were included in the first group, while malignant causes constituted the second group. Whether the two groups differed in terms of CWT, Hb, age, albumin, NLR, PLR, and MPV values was investigated with descriptive statistics. RESULTS: One hundred and sixteen patients (74 males, 42 females) with CT examinations and colonoscopic sampling were included in the study. After colonoscopic and histopathological evaluations, there were 64 cases in the first group and 52 in the second group. According to the results of the univariate analysis and multivariate logistic regression, CWT, Hb, NLR, and MPV were identified to be independent variables for determining colon cancer. CONCLUSION: A combined evaluation of Hb, NLR, and MPV values in patients with CWT in abdominal CT examination may contribute to the separation of benign and malignant pathologies.

Oxidative stress parameters in localized scleroderma patients.

Localized scleroderma (LS) (morphea) is a chronic, inflammatory skin disease with unknown cause that progresses with sclerosis in the skin and/or subcutaneous tissues. Its pathogenesis is not completely understood. Oxidative stress is suggested to have a role in the pathogenesis of localized scleroderma. We have aimed to determine the relationship of morphea lesions with oxidative stress. The total oxidant capacity (TOC), total antioxidant capacity (TAC), paroxonase (PON) and arylesterase (ARES) activity parameters of PON 1 enzyme levels in the serum were investigated in 13 LS patients (generalized and plaque type) and 13 healthy controls. TOC values of the patient group were found higher than the TOC values of the control group (p < 0.01). ARES values of the patient group was found to be higher than the control group (p < 0.0001). OSI was significantly higher in the patient group when compared to the control (p < 0.005). Oxidative stress seems to be effective in the pathogenesis. ARES levels have increased in morphea patients regarding to the oxidative stress and its reduction. Further controlled studies are required in wider series.

Seguridad y eficacia de la cirugía retrógrada intrarrenal en pacientes de diferentes grupos de edad / Safety and efficacy of retrograde intrarenal surgery in patients of different age groups

Objetivos: Evaluar la eficacia y seguridad de la cirugía intrarrenal retrógrada (CIRR) para tratar los cálculos renales en pacientes de diferentes grupos de edad. Pacientes y métodos: Se realizó un análisis retrospectivo de 947 pacientes que se sometieron a CIRR para cálculos renales entre enero de 2008 y enero de 2014. La edad en la CIRR se analizó tanto como una variable continua como categórica, y los pacientes fueron clasificados en 3 grupos de edad; ≤ 15 años en la cirugía (grupo i, n = 51), 16-60 años (grupo ii, n = 726) y> 60 años (grupo iii, n = 170). Se compararon los 3 grupos con respecto a las características del cálculo, los parámetros operativos y los resultados postoperatorios. Resultados; La tasa de ausencia de cálculos fue del 78,4% en el grupo i, 77,5% en el grupo ii, y 81,1% en el grupo iii (p = 0,587). Un análisis de regresión logística multivariante mostró que solo el tamaño del cálculo y el número de cálculos tuvieron una influencia significativa en las tasas de ausencia de cálculos después de CIRR. Se produjo un 13,7% de complicaciones intraoperatorias en el grupo i, 5,6% en el grupo ii, y 7,6% en el grupo iii. Las tasas de complicación global en niños eran más altas que en pacientes adultos, pero las diferencias no fueron estadísticamente significativas. Se descubrió que solo el tiempo de operación esataba asociado con el aumento del riesgo de complicaciones intraoperatorias. Las complicaciones médicas perioperatorias se desarrollaron en 8 pacientes (0,8%) en el grupo ii y 2 pacientes (1,1%) en el grupo iii. Un hombre de 48 años de edad murió a causa de shock séptico 5 días después de la cirugía. Conclusiones; Se observó que la CIRR era un procedimiento seguro y eficaz en todos los grupos de edad de pacientes con cálculos, por lo tanto, la edad no debe ser considerada como un factor limitante

Objectives: To assess the efficacy and safety of retrograde intrarenal surgery (RIRS) to treat renal stones in different age groups of patients. Patients and methods: We performed a retrospective analysis of 947 patients who underwent RIRS for renal calculi between January 2008 and January 2014. Age at RIRS was analysed both as a continuous and categorical variable and patients were categorized into three age groups; aged ≤ 15 years at surgery (group i, n = 51), 16 - 60 years (group Ii, n = 726) and > 60 years (group iii, n = 170). We compared the 3 groups with the regard to stone characteristics, operative parameters and postoperative outcomes. Results: The stone-free rate was 78.4% in group i, 77.5% in group ii, and 81.1% in group iii (P = .587). A multivariate logistic regression analysis showed that only stone size and stone number had significant influence on the stone-free rates after RIRS. Intraoperative complications occurred 13.7% in group i, 5.6% group ii, and 7.6% in group iii. Overall complication rates in children were higher than adult patients but the differences were not statistically significant. We found that only operation time was associated with the increased risk of intraoperative complications. Peroperative medical complications developed in 8 patients (.8%) in group ii and 2 patients (1.1%) in group iii. A 48-year-old man died from septic shock 5 days after the surgery. Conclusions: RIRS was observed to be a safe and effective procedure in all age groups of patients with stone disease, therefore age should not be considered as a limiting factor

Evaluation of the intraocular pressure changes during micturition in patients with benign prostatic hyperplasia.

OBJECTIVE: To evaluate the effect of the Valsalva maneuver (VM) during micturition on intraocular pressure (IOP) in patients with benign prostatic hyperplasia (BPH). PATIENTS AND METHODS: IOP were measured before and during micturition in uroflowmetry testing by Tonopen-Avia (Reichert Inc., NY, USA) in 35 eyes of 35 patients with BPH. International Prostate Symptoms Score (IPSS) questionnaire was used for quantification of BPH symptoms. The subjects were divided into two groups according to IPSS questionnaire: patients with low IPSS scores and patients with high IPSS scores. RESULTS: The mean IOP before and during micturition in patients with BPH were 15.26 ± 4.39 mmHg and 16.23 ± 4.43 mmHg, respectively (p = 0.047). When patients with BPH were assigned to two distinct groups according to the IPSS scores, IOP did not differ in patients with low IPSS scores (p = 0.590), whereas mean IOP was significantly higher in patients with high IPSS scores (p = 0.007). CONCLUSIONS: The Valsalva maneuver, performed during micturition, can lead to significant IOP increase in patients with BPH, particularly in advanced disease.

Safety and efficacy of retrograde intrarenal surgery in patients of different age groups.

OBJECTIVES: To assess the efficacy and safety of retrograde intrarenal surgery (RIRS) to treat renal stones in different age groups of patients. PATIENTS AND METHODS: We performed a retrospective analysis of 947 patients who underwent RIRS for renal calculi between January 2008 and January 2014. Age at RIRS was analysed both as a continuous and categorical variable and patients were categorized into three age groups; aged ≤ 15 years at surgery (group i, n=51), 16 - 60 years (group Ii, n=726) and>60 years (group iii, n=170). We compared the 3 groups with the regard to stone characteristics, operative parameters and postoperative outcomes. RESULTS: The stone-free rate was 78.4% in group i, 77.5% in group ii, and 81.1% in group iii (P=.587). A multivariate logistic regression analysis showed that only stone size and stone number had significant influence on the stone-free rates after RIRS. Intraoperative complications occurred 13.7% in group i, 5.6% group ii, and 7.6% in group iii. Overall complication rates in children were higher than adult patients but the differences were not statistically significant. We found that only operation time was associated with the increased risk of intraoperative complications. Peroperative medical complications developed in 8 patients (.8%) in group ii and 2 patients (1.1%) in group iii. A 48-year-old man died from septic shock 5 days after the surgery. CONCLUSIONS: RIRS was observed to be a safe and effective procedure in all age groups of patients with stone disease, therefore age should not be considered as a limiting factor.

Evaluation of some antioxidant enzyme activities (SOD and GPX) and their polymorphisms (MnSOD2 Ala9Val, GPX1 Pro198Leu) in fibromyalgia.

OBJECTIVE: Fibromyalgia syndrome (FMS) is a pain syndrome in which common pain in muscle-skeletal system, sleeping disorder and fatigue symptoms coexist. The aim of the present study was to determine SOD and GPX enzyme levels in FMS as well as to investigate possible associations between FMS and Ala9Val polymorphism of MnSOD2 and Pro198Leu polymorphism of GPX1. PATIENTS AND METHODS: The study included 127 women FMS patients and 56 healthy subjects. Total SOD and total GPX enzyme activities were determined in patient and control groups. In addition, frequencies of Ala9Val polymorphism of MnSOD2 and Pro198Leu polymorphism of GPX1 were also detected. RESULTS: SOD enzyme activity was higher in FMS group compared to control (p < 0.001). GPX enzyme activity, on the other hand, was not different between FMS and control groups. No significant differences were found between genotype and allele frequencies of GPX1 and MnSOD2 polymorphisms. CONCLUSIONS: Elevated total SOD and unchanging total GPX1 activities in FMS patients could be the reason for increased oxidative stress and lipid peroxidation in FMS. Genotype and allele frequencies of Ala9Val polymorphism of MnSOD2 and Pro198Leu polymorphism of GPX1 in FMS have been studied first time in the present study, and no associations were found between them and FMS.

The effects of carvedilol on ischemia-reperfusion injury in the rat testis.

OBJECTIVE: To analyze the oxidative damage and histopathological alterations caused by ischemia-reperfusion (I/R) injury and ameliorative effects of carvedilol (CVD) in the rat testis. MATERIALS AND METHODS: Twenty-one male rats were randomized into 3 groups as follows: Group I (n = 7); control (sham) group, Group II (n = 7); I/R group, in which I/R injury was performed by torsing the left testis 720 ° clockwise for 2 hours and detorsing for 2 hours. Group III (n = 7); CVD treatment group; in addition to I/R process, one-dose of CVD was administered (2mg/kg, i.p) 30 min. before detorsion. Levels of antioxidant enzymes, superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) and levels of malondialdehyde (MDA) and protein carbonyl (PC) were determined in testicular tissues and serum of rats. Testicular tissues were also examined histopathologically and Johnsen scores were determined. RESULTS: Activities of SOD and GSH-Px in serum and testicular tissues were increased by I/R, but administration of CVD decreased these levels (p < 0.001 and p = 0.001). Significantly increased MDA levels in serum and testicular tissues were decreased by CVD treatment (p < 0.001 and p = 0.001). Concerning PC levels in serum and testicular tissues, there was no statistically significant difference between the groups (p = 0.989 and p = 0.428). There was not a statistically significant difference in terms of mean Johnsen scores between the groups (p = 0.161). CONCLUSIONS: Administration of CVD decreased oxidative damage biochemically in the rat testis caused by I/R injury, but histopathologically no change was observed betwe¬en all of the groups.

The effects of carvedilol on ischemia-reperfusion injury in the rat testis

Objective: To analyze the oxidative damage and histopathological alterations caused by ischemia-reperfusion (I/R) injury and ameliorative effects of carvedilol (CVD) in the rat testis. Materials and Methods: Twenty-one male rats were randomized into 3 groups as follows: Group I (n = 7); control (sham) group, Group II (n = 7); I/R group, in which I/R injury was performed by torsing the left testis 720º clockwise for 2 hours and detorsing for 2 hours. Group III (n = 7); CVD treatment group; in addition to I/R process, one-dose of CVD was administered (2mg/kg, i.p) 30 min. before detorsion. Levels of antioxidant enzymes, superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) and levels of malondialdehyde (MDA) and protein carbonyl (PC) were determined in testicular tissues and serum of rats. Testicular tissues were also examined histopathologically and Johnsen scores were determined. Results: Activities of SOD and GSH-Px in serum and testicular tissues were increased by I/R, but administration of CVD decreased these levels (p < 0.001 and p = 0.001). Significantly increased MDA levels in serum and testicular tissues were decreased by CVD treatment (p < 0.001 and p = 0.001). Concerning PC levels in serum and testicular tissues, there was no statistically significant difference between the groups (p = 0.989 and p = 0.428). There was not a statistically significant difference in terms of mean Johnsen scores between the groups (p = 0.161). Conclusions: Administration of CVD decreased oxidative damage biochemically in the rat testis caused by I/R injury, but histopathologically no change was observed between all of the groups. .

Evaluation of total oxidant and antioxidant status in localized and generalized vitiligo.

BACKGROUND: Vitiligo is an acquired depigmentation disorder, and oxidative stress is suggested to have a major role in its aetiopathogenesis. AIM: To assess whether oxidative stress has a greater role in generalized than in localized vitiligo. METHODS: We assessed 31 patients with active vitiligo (17 localized, 14 generalized) and 38 healthy controls. Serum total oxidant status (TOS), total antioxidant status (TAS) and oxidative stress index (OSI) were determined. RESULTS: Patients with vitiligo had significantly lower TAS and higher TOS and OSI values than controls. Both localized and generalized vitiligo were associated with lower TAS and higher TOS and OSI values, compared with controls, and all three did not differ with vitiligo type. CONCLUSIONS: A systemic oxidative stress exists in patients with vitiligo. These results indicate that the global antioxidant capacity of patients might have been exhausted through a defence mechanism against oxidative processes. The imbalance in TOS/TAS status may have an important role in the aetiopathogenesis of vitiligo, regardless of the clinical variant of the disease.

Lich-Gregoir ureteral reimplantation with fixation of ureter during detrusorraphy as a reliable outpatient anti-reflux procedure.

PURPOSE: We report our experience with a Lich-Gregoir reimplantation (LGR) technique that included fixation of the ureter during detrusorraphy to preserve the integrity of the newly created submucosal tunnel, performed as an outpatient anti-reflux procedure. METHODS: Ninety-seven children (mean age: 7.9 years) with primary VUR underwent LGR between 1995 and 2008. Twenty-one had bilateral VUR, 8 had ureteral duplication, 9 had megaureter, and 13 had previous subureteric injections. Through a Pfannenstiel incision the ipsilateral ureter was mobilized up to the ureterovesical junction without disturbing ureterotrigonal integrity. A 3-5 cm detrusorotomy was extended from the ureterovesical junction towards the umbilicus, the ureter was placed on the mucosa, and detrusorraphy was performed with a loosely running 3/4-0 polyglactin suture that passed through the detrusor on one side of the incision, taking a 1-2 mm "bite" of the ureteral adventitia, and detrusor on the other side. Postoperative ultrasonograms and voiding cystograms (VCUG) were obtained at 1 and 3 months, respectively. Children were then followed up for clinical symptoms of urinary tract infection with annual ultrasonograms. RESULTS: The mean operative time was 56 min. Eighty-one percent of the children were treated as outpatients. Transient voiding difficulties occurred in 2 children with a bilateral procedure. Reflux was cured in 116 ureters (98%) with a mean follow-up of 58 months (range: 6-128). Ipsilateral obstruction was not evident during ultrasound surveillance. CONCLUSIONS: LGR is an outpatient procedure with a high success rate and a low morbidity. Integrity of the newly created submucosal tunnel is important for long-term success, which may be facilitated by our modified detrusorraphy technique.

Strategies to improve tolerability of rivastigmine: a case series.

Cholinesterase inhibitors, which act by inhibiting the degradation of acetylcholine in the brain, form the mainstay of therapy for AD. Predominant adverse events are cholinergic in nature, the most frequent being gastrointestinal side effects. Here we present a case series of patients with dementia who were treated with non-standard titration regimens of oral rivastigmine in order to achieve improved tolerability.

Tolosa Hunt syndrome: a case report. Clinical and magnetic resonance imaging findings.

A 36-year-old woman was admitted with a left abducens nerve palsy. MR showed enlargement of the left cavernous sinus. The patient was treated with 80 mg oral methyl prednisolone. Clinical findings improved within a month. Two months later, she was readmitted with left oculomotor and right abducens nerve palsy. MR showed significant increase in the volume of the abnormal area in the left cavernous sinus and a new lesion within the right cavernous sinus. After intravenous gadolinium DTPA, there was enhancement in both cavernous sinuses. Methyl prednisolone therapy was again started. After one month of treatment neurological examination was normal. Follow-up MR findings were similar to previous ones.

The effect of physeal traction applied to the triradiate cartilage on acetabular growth.

Physeal distraction was applied with an external fixator to the triradiate cartilage of dogs with the aim of increasing the capacity of the acetabulum. The force was continued for from 2 to 6 weeks and the consequent changes were evaluated with regard to function and structure by radiography and microscopy. The distraction, without producing epiphysiolysis and destruction of the cartilage, resulted in expansion of the pelvic bones. The depth and volume of the acetabulum were increased, but the acetabular angle was decreased. Distraction also caused proliferation of the lacunar cells and the number of mammillary processes in the cartilage columns increased. Distraction can therefore be applied to the triradiate cartilage to enlarge the capacity of the acetabulum without producing epiphysiolysis.

Larsen's syndrome with mixed-type hearing loss.

Mixed-type hearing loss is an extremely rare feature of Larsen's syndrome, a complex of symptoms characterized by multiple joint dislocation and "flat" faces. In this report, a patient with bilateral mixed-type hearing loss and Larsen's syndrome is presented.