Pediatric stapedectomy: does cause of fixation affect outcomes?

OBJECTIVE: To compare outcomes of stapedectomy in patients with congenital stapes fixation versus juvenile otosclerosis. METHODS: A retrospective chart review was performed from January 1, 1999 until January 1, 2011 to identify patients under 18 years old who underwent a stapedectomy. Age, gender, pre- and postoperative audiograms, intraoperative findings including etiology of stapes fixation, prosthesis type, and complications were recorded. RESULTS: Twenty-two children were identified who had undergone a stapedectomy (two patients underwent sequential bilateral surgery) resulting in a total of 24 ears. The cause of fixation included juvenile otosclerosis (n=7) and congenital stapes fixation (n=17). The overall mean pre-operative air-bone gap (ABG) was 34.7 dB (SD: 13.5) compared to a postoperative mean ABG of 9.0 (SD: 9.3) (p<0.001). The mean postoperative ABG of 9.6 (SD: 10.5) in the congenital stapes fixation group was similar to the mean postoperative ABG of 7.2dB (SD: 5.4) in children with juvenile otosclerosis (p=0.6). Two patients developed delayed profound sensorineural hearing loss approximately two weeks after surgery. One patient with profound sensorineural hearing loss recovered to a profound mixed hearing loss with a speech discrimination score of 80%. CONCLUSIONS: Pediatric stapedectomy has comparable results to stapedectomy in adults regardless of the cause of stapes fixation; however, delayed sensorineural hearing loss may be higher in the pediatric population.

Characterization of the larynx in ephrin-B2 knockout mice: a novel animal model for laryngeal clefts.

OBJECTIVE: To identify and classify laryngeal clefts in a novel mouse model. DESIGN: In vivo animal study. SETTING: Academic research laboratory. SUBJECTS: 129/CD1 mice with the ephrin-B2 gene disrupted by the ß-galactosidase (lacZ) gene were humanely killed at embryonic day 18 (E18) and evaluated for the presence and characterization of a laryngeal cleft. Homozygous and heterozygous lacZ knockout mice as well as wild-type littermates were evaluated. MAIN OUTCOME MEASURES: Microsurgical dissection of the oral cavity and pharynx allowed for a pseudoendoscopic view of the larynx to determine the presence or absence of a cleft. The specimens were also histologically sectioned and examined for characterization and classification of the cleft. RESULTS: A laryngeal cleft was identified in 12 of 27 ephrin-B2 homozygous lacZ knockout mice (44%). Laryngeal clefts were not identified in heterozygous ephrin-B2 knockout mice or in wild-type littermates. CONCLUSIONS: Disruption of ephrin-B2 reverse signaling results in laryngeal clefts in lacZ knockout mice. This presents a novel mouse model in which future investigations into etiology of laryngeal clefts may be examined.

Pediatric otogenic lateral sinus thrombosis recanalization.

OBJECTIVE: To describe the recovery outcomes in pediatric patients with otogenic lateral sinus and internal jugular vein thrombosis. METHODS: An inpatient database from a tertiary care pediatric hospital was queried from 1999 to 2010 for the diagnosis code [325] thrombosis of intracranial venous sinus. Demographics, extent of thrombosis, surgical intervention, use of anticoagulation, and the presence of recanalization on follow-up imaging was collected. RESULTS: Fifteen patients (10 male, 5 female) were identified with otogenic lateral sinus thrombosis. Eleven patients (73.3%) had evidence of thrombus in the transverse sinus, while 10 patients (66.7%) had thrombus in the internal jugular vein, and one patient (6.7%) had thrombus in the cavernous sinus. Five patients (33%) had otitic hydrocephalus. Twelve patients (80%) underwent operative manipulation of the lateral sinus including: three decompressions 20%, three needle aspirations 20%, and six venotomies with evacuation of clot or pus 40%. Twelve patients were anticoagulated with low molecular weight heparin, and three patients were not anticoagulated. Ten of fourteen patients (71.4%) who underwent follow-up magnetic resonance venography had evidence of partial (57.1%) or complete (14.3%) recanalization of the lateral sinus. All five patients with otitic hydrocephalus recovered as determined by a normal fundoscopic exam and recovery of abducens paresis. CONCLUSION: Recanalization of the lateral intracranial venous sinus occurred in the majority of the patients in this series. The role of operative intervention and/or anticoagulation remains unclear.

Otalgia.

Otalgia, pain in the ear, can be a consequence of otologic disease (primary or otogenic otalgia), or can arise from pathologic processes and structures other than the ear (secondary or referred otalgia). In children, ear disease is far and away the most common cause of otalgia, but in adults, secondary or referred otalgia is more common. Otalgia can be the only presenting symptom of several serious conditions, and its etiology should be fully explored. Unfortunately, its workup is complex and no simple algorithm exists. This article outlines the common causes, presentation, and treatment options for primary and secondary otalgia.