Cochlear nerve canal stenosis and associated semicircular canal abnormalities in paediatric sensorineural hearing loss: a single centre study.

OBJECTIVE: This study aimed to evaluate the association between cochlear nerve canal dimensions and semicircular canal abnormalities and to determine the distribution of bony labyrinth anomalies in patients with cochlear nerve canal stenosis. METHOD: This was a retrospective study in which high-resolution computed tomography images of paediatric patients with severe-to-profound sensorineural hearing loss were reviewed. A cochlear nerve canal diameter of 1.5 mm or less in the axial plane was classified as stenotic. Semicircular canals and other bony labyrinth morphology and abnormality were evaluated. RESULTS: Cochlear nerve canal stenosis was detected in 65 out of 265 ears (24 per cent). Of the 65 ears, 17 ears had abnormal semicircular canals (26 per cent). Significant correlation was demonstrated between cochlear nerve canal stenosis and semicircular canal abnormalities (p < 0.01). Incomplete partition type II was the most common accompanying abnormality of cochlear nerve canal stenosis (15 out of 65, 23 per cent). CONCLUSION: Cochlear nerve canal stenosis is statistically associated with semicircular canal abnormalities. Whenever a cochlear nerve canal stenosis is present in a patient with sensorineural hearing loss, the semicircular canal should be scrutinised for presence of abnormalities.

Adenoid cystic carcinoma of the sinonasal tract: outcome of endonasal endoscopic surgery at five-year follow up.

OBJECTIVE: To study the outcome of endonasal endoscopic surgery for adenoid cystic carcinoma of the sinonasal tract over a five-year follow-up period. DESIGN: Retrospective analysis. METHODS: Four consecutive patients with adenoid cystic carcinoma of the sinonasal tract, who had undergone endonasal endoscopic surgery, were reviewed regarding age at diagnosis, sex, primary site, tumour-node-metastasis staging, treatment modalities, histopathological findings, duration of follow up, distant metastases and treatment outcome. RESULTS: All patients were diagnosed at an advanced stage and had post-operative adjuvant radiotherapy. Three patients underwent endoscopic endonasal resection and one endoscopic assisted craniofacial resection. The most common primary site was the ethmoid sinus (three patients). Three patients had no evidence of recurrence. One patient who had undergone partial clearance via endoscopic endonasal resection developed cervical node metastases a year after treatment; this patient also developed distant metastases. CONCLUSION: Adenoid cystic carcinoma is difficult to treat. Sinonasal tract tumours can be resected via endoscopic endonasal resection or endoscopic assisted craniofacial resection, but prolonged follow up is advisable. Radiotherapy is an important adjuvant treatment.

Duplicate internal auditory canals with facial and vestibulocochlear nerve dysfunction.

OBJECTIVE: We report an extremely rare case of duplication of the internal auditory canal associated with dysfunction of both the facial and vestibulocochlear nerves. We also review the literature regarding the integrity of the facial and vestibulocochlear nerves in such cases. CASE REPORT: A 34-year-old man presented with unilateral, right-sided, sensorineural hearing loss and facial nerve palsy since childhood. Facial nerve function was observed to be House-Brackmann grade III. Computed tomography and magnetic resonance imaging demonstrated ipsilateral duplicate, vacant internal auditory canals. Based on the clinical presentation, we interpreted these radiological findings as aplasia of the vestibulocochlear nerve and severe hypoplasia of the facial nerve. CONCLUSION: To our best knowledge, this is the first report of vestibulocochlear nerve aplasia and severe facial nerve hypoplasia in a case of ipsilateral duplication of the internal auditory canal. High resolution gradient echo magnetic resonance imaging sequences are advocated for assessment of neural integrity in patients with an abnormal internal auditory canal and facial and/or vestibulocochlear nerve dysfunction.

c-erbB-4 protein expression in human breast cancer.

The Type 1 family of growth factor receptors includes epidermal growth factor receptor (EGFR), c-erbB-2, c-erbB-3 and c-erbB-4. Overexpression of the first two members is associated with poorer prognosis in patients with breast carcinoma. In this study we examined the expression of c-erbB-4 protein using the monoclonal antibody HFR-1. A total of 127 consecutive cases of primary operable invasive breast carcinoma presenting between 1975 and 1977 were studied. All patients were managed by simple mastectomy or conservation surgery with radiotherapy and no adjuvant therapy given. Long-term follow-up was maintained. Routine, formalin-fixed, paraffin-embedded tumour samples were used and sections were stained immunohistochemically using the Duet StreptABC method. Immunoreactivity was classified using a simple semi-quantitative scoring method. Protein expression was generally low but definite positive cytoplasmic, membranous and nuclear reactivity was identified in 58%, 41% and 25% of cases respectively. Expression at all three sites demonstrated significant inverse associations were histological grade. In addition, membrane accentuation correlated inversely with the Nottingham Prognostic Index (NPI), while cytoplasmic reactivity showed a positive association with c-erbB-3 expression. No significant associations were found with disease-free interval or survival. The results of this study demonstrate that higher levels of c-erbB-4 protein expression are associated with a more differentiated histological phenotype in contrast to the other members of the Type 1 family. Larger series with extended follow-up will be required to ascertain definitively the prognostic value of c-erbB-4 expression in breast carcinoma.