Endothelin-1 gene polymorphism in sudden sensorineural hearing loss.

Objectives/hypothesis: Endothelin-1 is a potent vasoconstrictor peptide that is widely distributed throughout the mammalian body including the spiral modiolar artery, vestibule, and cochlea. This study aimed to investigate the association between the Lys198Asn (G/T) polymorphism (rs5370) of the endothelin-1 gene and sudden sensorineural hearing loss (SSNHL).

Study Design: Case-control study.

Polymorphisms in genes encoding aquaporins 4 and 5 and estrogen receptor α in patients with Ménière's disease and sudden sensorineural hearing loss.

Aims: The etiologies of Ménière's disease and idiopathic sudden sensorineural hearing loss (SSNHL) remain unclear. The homeostasis of the water and blood circulation in the inner ear is essential for maintaining its hearing and equilibrium functions, and aquaporins and estrogen are involved in the fluid or ion balance in the inner ear. We investigated the associations between genetic polymorphisms in aquaporin 4 (AQP4, rs2075575), aquaporin 5 (AQP5, rs3736309), and estrogen receptor α (ERα1, rs2234693; ERα2, rs9340799) and susceptibility to Ménière's disease or SSNHL.

Progressive hearing loss following acquired cytomegalovirus infection in an immunocompromised child.

We report a rare case of progressive hearing loss after acquired CMV infection in a child with Langerhans cell histiocytosis (LCH). A 5-month-old female was diagnosed as having LCH. When she was 14 months old, she received an unrelated donor umbilical cord blood transfusion for the treatment of intractable LCH.

Children with severe or moderate obstructive sleep apnoea syndrome show a high incidence of persistence after adenotonsillectomy.

Conclusion: Persistent obstructive sleep apnoea syndrome (OSAS) occurs in approximately 20% of normal-weight children after adenotonsillectomy (T&A) and, in nearly 70% of them, it is caused by adenoid regrowth. Patients with severe or moderate OSAS showed a high incidence of persistent disease even after T&A. Allergic disease, severity and large adenoid size are associated with adenoid regrowth and persistent disease.

Relationship between adenoid size and severity of obstructive sleep apnea in preschool children.

Objective: To investigate the contributions of adenoid and tonsil sizes to obstructive sleep apnea syndrome (OSAS) in normal-weight children in two age categories: preschool and schoolchildren.

Methods: Fifty-eight normal-weight (body mass index z-score<2) symptomatic children with OSAS (apnea-hypopnea index ≥ 2) were evaluated. The patients were divided into two age categories: preschool (age<6; n=33) and schoolchildren (age ≥ 6; n=25).

Contribution of complement factor H Y402H polymorphism to sudden sensorineural hearing loss risk and possible interaction with diabetes.

Sudden sensorineural hearing loss (SSNHL) is one of the most common diseases encountered by otolaryngologists; however, the etiology is unclear. The aim of this study was to assess the association between SSNHL and polymorphism of complement factor H (CFH) Y402H, which is implicated in age-related macular degeneration. We conducted a case-control study, in which the cases were 72 SSNHL patients and the controls were 2161 residents selected randomly from the resident register.

Polymorphisms in genes involved in inflammatory pathways in patients with sudden sensorineural hearing loss.

Although the etiology of idiopathic sudden sensorineural hearing loss (SSNHL) remains unclear, the pathologically increased permeability of blood vessels, elucidated by gadolinium-enhanced magnetic resonance imaging (MRI), suggests the involvement of inflammation. Because SSNHL is considered a multifactorial disease, possibly caused by interactions between genetic factors and environmental factors, the authors investigated the associations of polymorphisms of inflammatory mediator genes with susceptibility to SSNHL. The authors compared 72 patients affected by SSNHL and 2010 adults (1010 men and 1000 women; mean age 59.

Endolymphatic hydrops and blood-labyrinth barrier in Ménière's disease.

Conclusions: The blood-labyrinth barrier is impaired in association with the hydrops grade in Ménière's disease.

Objectives: To investigate the relationship between endolymphatic hydrops and the clinical characteristics of patients with Ménière's disease revealed by 3 T magnetic resonance imaging (MRI).

Methods: A double dose of gadoteridol (Gd; 0.

Pathogenetic role of increased nasal resistance in obese patients with obstructive sleep apnea syndrome.

Background: The role of increased nasal resistance in obstructive sleep apnea syndrome (OSAS) remains controversial. The aim of this study was to examine the pathogenetic role of nasal obstruction in obese patients with OSAS.

Methods: Patients with OSAS (n = 125) at a university hospital were divided into three groups according to body mass index (BMI): nonobese (BMI < 25 kg/m(2)), mildly obese (25 kg/m(2) < or = BMI < 30 kg/m(2)), and obese (BMI > or = 30 kg/m(2)).

3 Tesla magnetic resonance imaging obtained 4 hours after intravenous gadolinium injection in patients with sudden deafness.

Conclusion: 3 Tesla (3T) magnetic resonance imaging (MRI) performed 4 h after intravenous gadolinium (Gd) injection provides sufficient anatomic resolution of the inner ear fluid spaces in sudden deafness. The signal intensity ratio (SIR) between the cochlea and cerebellum may be a good indicator of disruption of the blood-labyrinthine barrier.

Objectives: We evaluated the inner ear 4 h after intravenous Gd injection to determine whether 3T MRI enables the acquisition of images of the affected inner ear in sudden deafness.

Endolymphatic hydrops revealed by intravenous gadolinium injection in patients with Ménière's disease.

Conclusion: Visualization of endolymphatic hydrops became possible after intravenous gadolinium (Gd) injection in patients with Ménière's disease.

Objective: To visualize endolymphatic hydrops after intravenous Gd injection.

Methods: Gd (gadoteridol; 0.

Pharyngeal morphology: a determinant of successful nasal surgery for sleep apnea.

Objectives/hypothesis: To estimate the effectiveness of nasal surgery on the occurrence of sleep apnea, and to analyze the pharyngeal morphology of apnea patients whose sleep-disordered breathing was ameliorated postoperatively.

Study Design: Prospective study.

Methods: Thirty-five consecutive patients with apnea and nasal obstruction underwent polysomnography and a morphological examination of the upper airway before and after nasal surgery, which included septoplasty, inferior turbinectomy, and/or functional endoscopic sinus surgery.

Morphological examination of upper airway in obstructive sleep apnea.

Objectives: An aim of this study was to assess the predictive power of an otorhinolaryngological examination of the upper airway to identify risk factors of obstructive sleep apnea syndrome (OSAS) in the patients.

Methods: We examined 141 consecutive patients with OSAS. The morphological features were assessed by the designated otorhinolaryngologist while the subjects were sitting relaxedly with tidal breathing.

Significance of a screening device (Apnomonitor 5) for sleep apnea syndrome.

Objectives: The objective was to evaluate the significance of a portable sleep-monitoring device (Apnomonitor 5, Chest Co., Tokyo, Japan) to diagnose sleep apnea syndrome (SAS).

Methods: The Apnomonitor 5 comprised an oronasal thermistor, a pulse oximeter, chest and abdominal belts to monitor the circumferences of the chest and abdomen, a microphone to monitor tracheal sound, a position detector, and an integrative unit.

Recombination of a maternal pericentric inversion results in 22q13 deletion syndrome.

We describe a 10-month-old boy with 22q13 deletion syndrome. Chromosomal analysis showed a partial duplication of 22p11.2-pter and a terminal deletion of 22q13.