Hearing loss in Turner syndrome.

Objective: To address the characteristics of hearing loss in patients with Turner syndrome (TS), we evaluated hearing levels of patients with TS and analyzed causative factors.

Study Design: Thirty-three patients with TS (8 to 40 years of age) were studied through the use of audiological measurements, and causative factors were explored.

Results: Twenty cases (35 of 66 ears tested) showed high-frequency (8 kHz) sensory neural hearing loss (HFQ-SNHL).

[Congenital laryngeal stridor].

We reviewed the diagnosis, complications and treatment of congenital laryngeal stridor (CLS), in 97 patients who consulted our clinic between 1991 and 2001. The 97 patients were diagnosed with laryngeal malacia (32%), vocal cord paralysis and laryngeal stenosis (22%), a neoplastic disease like hemagioma and papilloma (11%), or cystic disease (7%). The cases with vocal cord paralysis, laryngeal stenosis or laryngeal cysts were usually diagnosed within 2 months of birth based on severe dyspnea.

Mondini dysplasia and recurrent bacterial meningitis in a girl with relapsing Langerhans cell histiocytosis.

We report a case of a girl with Langerhans cell histiocytosis (LCH) of multifocal bone disease, who developed recurrent bacterial meningitis and unilateral sensorineural hearing loss during the relapsing course of the disease. Mondini dysplasia, a congenital inner ear anomaly, was suspected by high resolution computed tomographic scan and the dysplasia with cerebrospinal fluid leakage was confirmed by surgery in the ipsilateral ear showing hearing loss. Although rare, congenital inner ear anomalies such as Mondini dysplasia should be kept in mind in pediatric patients with hearing impairment and/or recurrent bacterial meningitis during chemotherapy for various types of neoplasms including LCH.

[Congenital choanal atresia and nasal stenosis].

The most common form of nasal obstruction in neonates is soft tissue edema, but congenital bony nasal anomalies are recognized as an important cause of newborn airway obstruction. We reviewed 20 cases of congenital bony nasal abnormalties such as choanal atresia and nasal stenosis referred to National Children's Hospital between 1996 and 2001. Of the 20, 8 involved choanal atresia (6 bilateral and 2 unilateral) and 12 nasal stenosis (5 nasal cavity stenosis, 2 pyriform aperture stenosis and 5 unknown).