Extramedullary plasmacytoma of temporal bone: report of 2 cases and review of literature.

OBJECTIVE: The aim of the study was to report 2 cases of extramedullary plasmacytoma (EP) with localization to middle ear that were diagnosed and managed in our department. METHODS: The first patient was a 60-year-old man with progressive hearing loss, tinnitus, recurrent episodes of otalgia, and otorrhea in his right ear during the last 2 years. The second patient was a 66-year-old man who presented with aural fullness in his right ear and a mild pain in the region of right mastoid. Mild hearing loss and episodes of dizziness with sensation of falling for about 6 months were reported in the clinical history. CONCLUSIONS: The prognosis of EP is considered favorable in regard to the solitary bone plasmacytoma and multiple myeloma (MM). The differential diagnosis of EPs from other plasma cell dyscrasias and especially from MM is considered essential. It is a radiosensitive tumor and, especially for EPs of temporal bone, the combined treatment of surgery resection and postoperative radiation seems to provide the best local control and the lower risk of occurrence. The therapeutic strategy includes a close follow-up of the patients because of the risk of occurrence and/or dissemination into MM. The 10-year survival rate reaches 70% with the appropriate therapy.

The tympanic segment of the facial nerve: anatomical study.

There is a conroversy in the literature about the length of the proximal tympanic segment of the facial nerve (PTSFN). The objective of the current study is to measure the length of the tympanic segment of the facial nerve (TSFN) and of its proximal (PTSFN) and distal (DTSFN) segments, in normal human temporal bones. Moreover, we will explore if these lengths are correlated. If a form of a functional relationship can be established, it could offer insights in partially predicting or estimating the length of the TSFN as well as of its proximal and distal portions. Direct measurements were obtained in 40 normal human temporal bones, which were examined by surgical dissection. Relationships between these measurements were established using Pearson's correlation method (two-tailed). The length of the TSFN was on average 10.97 mm. The length of the PTSFN was on average 5.25 mm and of the DTSFN was 5.72 mm. No significant statistical differences according to gender or side (right or left) were detected. It was determined that the length of the TSFN was in linear correlation with positive direction with its proximal (PTSFN) and distal (DTSFN) segments. Also the PTSFN length was in linear correlation with positive direction with the DTSFN length. The length of the PTSFN comprises about one-half of the TSFN length. The existence of a definite correlation between the lengths of the TSFN, PTSFN, and DTSFN implies the existence of a form of functional interrelationship. This could facilitate prediction and identification of the TSFN and PTSFN lengths from the easily identifiable DTSFN length during surgery.

Auditory processing disorder and brain pathology in a preterm child with learning disabilities.

BACKGROUND: Auditory processing disorders involve deficits in the processing of information in the auditory domain that are not due to higher order language, cognitive or other related factors. PURPOSE: To evaluate the possibility of structural brain abnormalities in preterm children manifesting as auditory processing disorders. RESEARCH DESIGN: A case report of a young girl, preterm at birth, with language difficulties, learning problems at school, and additional listening problems. RESULTS: A diagnosis of a central auditory processing disorder was made on the basis of severe deficits in three nonspeech temporal tests (the frequency and duration pattern and the random gap detection tests). Her brain MRI revealed large porencephalic cysts and thinning of the corpus callosum. CONCLUSIONS: The observed auditory deficits would be compatible with a pressure effect of the cysts at a brainstem or higher level for the random gap detection test, and with the thinning of the corpus callosum for the pattern tests, the latter requiring interhemispheric transfer of information. The case highlights that preterm children with learning difficulties may suffer from an auditory processing disorder, in the presence of structural brain abnormalities that are due to birth and neonatal complications.

Sudden sensorineural hearing loss: long-term follow-up results.

OBJECTIVE: This study describes the long-term outcome of patients suffering from sudden sensorineural hearing loss (SSHL) after an initial combined therapy of steroids with vasodilators. STUDY DESIGN AND SETTING: Eighty cases were retrospectively reviewed to evaluate the long-term audiometric data and to statistically assess the value of specific clinical parameters. The persistent long-term otoneurologic manifestations associated with SSHL were also reported. RESULTS: In the long-term period, hearing level remained stable 2 months after the onset of SSHL. Patients complained of persistent tinnitus (36%), fluctuant hearing loss (8.7%), dizziness (8.7%), and classical Ménière's triad in 2.5% of cases. Profound and total SSHL associated with vertigo are poor prognostic indicators. CONCLUSIONS: In terms of cost-effectiveness, a steroid-vasodilator therapy for SSHL for >2 months is not recommended because no additional effect was evidenced. Persistent otoneurologic manifestations, mainly tinnitus or dizziness are relatively common, particularly in patients with severe to total SSHL. EBM RATING: C-4.

Hearing assessment in pre-school children with speech delay.

OBJECTIVE: The purpose of this study was to detect any underlying hearing loss among the healthy pre-school children with speech delay. METHODS: 76 children, aged from 1 to 5 years, underwent a thorough audiological examination consisting of tympanometry, free field testing, otoacoustic emission recordings and auditory brainstem responses (ABRs). If hearing was normal, then they were evaluated by a child neurologist-psychiatrist. RESULTS: According to our findings, the children were classified into 3 groups; those with normal hearing levels (group I, 52 children, 68.4%), sensorineural hearing loss (group II, 22 children, 28.9%) and conductive hearing loss (group III, 2 children, 2.6%). In group I, speech delay was attributed to pervasive developmental disorder (PDD), which represents high-functioning autistic children (37 cases). Other causes were specific language impairment (SLI)-expressive (3 cases), bilingualism (2 cases), and unknown etiology (10 cases). More than half (59%) of the children diagnosed with PDD evidenced significant language impairment limited to more than two words. Children with SLI-expressive and bilingualism used a maximum of two words. In group II, 13 children suffered from profound hearing loss in both ears, 3 from severe, 3 had profound hearing loss in one ear and severe in the other, 2 from moderate, and 1 had moderate in one ear and severe in the other. No child had mild sensorineural hearing loss. The children with profound hearing loss in at least one ear had total language impairment using no word at all (10 cases), or a maximum of two words (6 cases). When hearing loss was moderate to severe, then the speech vocabulary was confined to several words (more than two words-6 cases). Only two children suffering from conductive hearing loss both presented with complete lack of speech. CONCLUSION: A great number of healthy pre-school children with speech delay were found to have normal hearing. In this case, the otolaryngologist should be aware of the possible underlying clinical entities, especially of psychiatric nature. The children with profound sensorineural hearing loss exhibited more severe speech delay than those with moderate to severe. Regardless of etiology, the early identification and intervention contribute to positive outcome in this critical period of childhood for language development.

Olfactory neuroblastoma. A report of 3 cases.

Olfactory neuroblastoma is a rare malignant tumour, usually diagnosed at advanced stages. We studied 3 patients who were treated at our Institute between 1991 and 1999. One patient presented with a stage A and 2 with a stage B tumour. One patient presented with coma due to inappropriate secretion of antidiuretic hormone associated with a stage B tumour. All 3 patients were treated with complete surgical resection via a lateral rhinotomy approach and postoperative radiotherapy. There was no involvement of the cribriform plate. One patient developed a metachronous regional metastasis and was treated with neck dissection and radiotherapy. All 3 patients are free from recurrence with a follow-up period of 9 years, 18 months and 1 year, respectively. Combination therapy is the cornerstone of treatment for olfactory neuroblastoma. Complete surgical resection is the most important prognostic factor and can be accomplished via lateral rhinotomy for early stage tumours.