Cephalometric findings among children with velopharyngeal dysfunction following adenoidectomy-A retrospective study.

OBJECTIVES: To characterise the craniofacial structure by cephalometry, especially the skull base and nasopharyngeal space, in children who underwent adenoidectomy and developed persistent velopharyngeal dysfunction (VPD). DESIGN: Retrospective study. SETTING: Speech and swallowing clinic of a single academic hospital. PARTICIPANTS: Thirty-nine children with persistent VPD following adenoidectomy (mean age 8.0±3.6 years) and a control group of 80 healthy children. MAIN OUTCOME MEASURES: Cephalometric landmarks were chosen; craniofacial linear and angular dimensions were measured and analysed. RESULTS: The linear dimensions of the nasopharyngeal area were shorter in the VPD group, S-Ba (41.6±4.2 mm, P<.05) and S-Ptm (42.4±5.1 mm, P<.05). The anterior skull base, N-S, was similar (68.1 mm±6.8). The velum length, Ptm-P was significantly shorter in the VPD group (27.8±4.3 mm, P<.001). The Ba-S-Ptm angle was significantly larger in the VPD group (63.5±5.6°, P<.001). There was no significant difference in cranial base angle (CBA), Ba-S-N, between the two groups. CONCLUSIONS: Cephalometry may provide information regarding persistent postoperative VPD. The nasopharyngeal space angle and velar length appear to be risk factors for persistent VPD after adenoidectomy.

Evaluation of noise-induced hearing loss by auditory steady-state and auditory brainstem-evoked responses.

OBJECTIVES: Noise-induced hearing loss (NIHL) may result from occupational noise exposures and is considered as an 'Occupational Disease'; therefore, it is compensable. To verify the existence and severity of the work-related hearing loss, there is a need of an objective, reliable auditory measure in cases of arbitration of financial disputes to resolve any medicolegal aspects. The objective of the study was to compare between the ABR and ASSR for predicting the behavioural threshold in subjects with normal hearing or NIHL. DESIGN: The study included 82 subjects regularly exposed to high levels of occupational noise, with normal hearing and NIHL. ABR to clicks and to tone bursts were recorded followed by multiple-frequency ASSR. Physiological and behavioural thresholds were compared for specific frequencies (1000, 2000 Hz) and average of high-frequency range (2000 and 4000 Hz). In addition, Pearson correlations and the specificity and sensitivity of each measure were also calculated using receiver operating characteristic (ROC) curves. RESULTS: In the NIHL group, there was a significantly smaller difference between the behavioural threshold and click-ABR than the ASSR in high-frequency range. Pearson correlations were significantly higher for click-ABR. Analysis of specific frequencies yielded a smaller difference between behavioural and ASSR than tone-burst-ABR thresholds, with a slightly better correlation for ASSR than tone-burst-ABR. Higher sensitivity but lower specificity was suggested for ASSR than ABR. CONCLUSIONS: ASSR is associated with high-frequency specificity, shorter test sessions and good correlations with behavioural thresholds, making it a potentially better measure than ABR for predicting audiograms in subjects with NIHL. These findings have diagnostic implications, especially in cases of workers' compensation when subjects may be uncooperative.

Anti-estrogen therapy for hereditary hemorrhagic telangiectasia - a long-term clinical trial.

STATEMENT OF PROBLEM: Hereditary hemorrhagic telangiectasia (HHT) is associated with recurrent epistaxis in 90% of the cases. Good response to hormone treatment has been documented, although its use remains controversial. The aim of this study was to examine the efficacy of an anti-estrogenic agent, tamoxifen, in the treatment of HHT-associated epistaxis. METHOD OF STUDY: Out of 46 patients with diagnosis of epistaxis due to HHT who started treatment with tamoxifen 20 mg/d, 38 patients completed a mean of 23.4 months of treatment. All patients filled out a self-assessment questionnaire of rhinologic Quality of Life and epistaxis grading scale. MAIN RESULTS: The bleeding score and the Quality of Life score improved. Hemoglobin concentration also improved. None of the patients needed blood transfusions during the treatment period. Only one patient had minor side effects of the drug. PRINCIPAL CONCLUSIONS: Tamoxifen appears to be an effective agent for the treatment of epistaxis due to HHT.

The impact of positive resection margins in partial laryngectomy for advanced laryngeal carcinomas and radiation failures.

OBJECTIVES: To investigate the prognostic significance of positive margin and disease course in partial laryngectomy for advanced laryngeal carcinoma and radiation failure. DESIGN: Retrospective case control study. SETTING: A major tertiary referral centre. PARTICIPANTS: Patients who underwent partial laryngectomy for advanced laryngeal carcinoma or for radiation failure with at least 2 years of follow-up. MAIN OUTCOME MEASURES: Margin status versus primary treatment and salvage treatment versus disease-free survival and overall survival. RESULTS: Twenty nine patients with sufficient follow-up data were found. Twelve patients had histological positive margin. Five of the 12 patients with a histological positive margin failed surgery as did four patients with clear margins. A positive margin had no effect on disease-free survival (P = 0.287) but was associated with poorer overall survival (P = 0.051). Of 11 patients treated primarily with surgery, recurrence was documented in one of eight with a positive margin and none of three with clear margins. Of 18 patients who underwent surgery secondary to radiation failure, recurrence was documented in all four with a positive margin and 4 of 14 with negative margins. Extended frontolateral resection, performed only in radiation failures, was associated with worse disease-free survival. CONCLUSIONS: Non-irradiated patients with involvement of a single margin after partial laryngectomy may be spared total laryngectomy if adjuvant radiation is administered. Patients who fail radiation should undergo radical partial laryngectomy, with conversion to total laryngectomy in those with a positive margin.

[(123)I]-FP-CIT SPECT and olfaction test in patients with combined postural and rest tremor.

Mixed-type tremors pose a clinical diagnostic challenge. The aim of the study was to better characterize patients with combined postural and rest tremor. Patients were categorized into four groups: essential tremor (ET) (n = 7), combined rest + postural tremor (n = 17), PD (n = 17), and control subjects (n = 9). All underwent the University of Pennsylvania Smell Identification Test (UPSIT). The mixed-tremor group was also evaluated with SPECT imaging using the dopamine transporter (DaT) ligand (123)I-labeled FP-CIT. There was no significant difference in olfaction scores between the mixed tremor and essential tremor groups (23.2 +/- 6.6 vs 21.7 +/- 4.9) or between these groups and controls (27.2 +/- 5.0). The patients with PD had significantly lower scores than all the other groups (13.7 +/- 5.4, p < 0.001). Of the 12 patients with mixed tremor evaluated by SPECT, 9 had normal findings. This study suggests that rest tremor is part of the spectrum of ET, even in patients with long-standing disease. However, in a minority of patients, there might be transformation of ET-PD.

Dysfunction of the auditory efferent system in patients with traumatic brain injuries with tinnitus and hyperacusis.

OBJECTIVES: Tinnitus, hyperacusis and difficulty listening in background noise are common symptoms reported by patients with traumatic brain injury (TBI). The aim of this study was to explore the function of the auditory system in TBI patients with and without auditory complaints but having normal pure-tone audiograms. METHODS: The study consisted of 24 TBI patients with and 10 TBI patients without auditory complaints. In addition, 15 normal controls were included in the study. The function of the auditory system was tested by recording transient otoacoustic emissions (TEOAE) during the presentation of increasing levels of white noise in the contralateral ear. RESULTS: Most of the TBI patients with auditory complaints (87%) showed absent or significantly reduced effect of the auditory efferent system as compared with the TBI patients without auditory complaints and to normal controls. However, the global amplitude of the TEOAE was significantly higher in TBI patients with auditory complaints compared to those without. CONCLUSIONS: Due to its role in peripheral and central auditory activity, dysfunction of the efferent system may be at least partially responsible for these auditory complaints. This study underscores the importance of testing and evaluating the functional integrity of the medial efferent system by an objective and non-invasive method in patients with TBI.

The effects of passive smoking on olfaction in children.

The effect of passive smoking on odor identification was studied in 10 children exposed to passive smoke at home. All had at least one parent who smoked at least one pack of cigarettes a day. The control group consisted of 10 children of nonsmoking parents. Ten odorants were tested: vinegar, ammonia, peppermint, roses, bleach, vanilla, cough drops, turpentine, licorice and mothballs. Each child was presented with five test trays containing all 10 odorants in random order. Of the total of 500 odors presented, the control group correctly identified 396 (79%) and the study group, 356 (71%) (p < 0.005). This work demonstrates that children exposed to passive smoke have difficulty identifying odors in comparison to children raised in relatively smoke-free environments. Since the study group tend to misidentify four of the 10 odorants tested--vanilla, roses, mothballs and cough drops--we suggest that these four odorants should suffice in testing odor identification in children.

[The endoscopic approach to isolated sphenoid sinus disease].

UNLABELLED: We reviewed the records of 38 patients aged 16 to 76 years with isolated sphenoid sinus disease who were treated by intranasal endoscopic sphenoidotomy at our hospital during the period 1992-1997. Diagnosis was made on the basis of history rigid nasal endoscopy and computed tomography (CT) scan of the sinuses. Headache was the main symptom in 29 (76%) patients. Other complaints were rhinitis, cough, nasal obstruction and nasal bleeding. Sphenoidotomy was performed endoscopically, directly through the area of the natural ostium. Acute or chronic sinusitis was found in 57% of the patients, cysts in 13%, polyps in 10%, mucocele in 8%, and fungal infection, pituitary adenoma, inverted papilloma and adenocarcinoma, in 3% each. Surgical results were excellent. The endoscopic approach proved to be both safe and effective. CONCLUSION: Sphenoid sinus disease is mostly inflammatory in origin. The endoscopic approach to the sphenoid sinus is currently the most appropriate method of surgery for an isolated sphenoid lesion.

Resolved sudden hearing loss as a presenting symptom of retrocochlear lesion.

OBJECTIVE: To determine whether acoustic neuroma-induced sudden hearing loss is associated with hearing recovery and, if so, to characterize its clinical, audiometric and imaging manifestations. METHODS: The files of 72 patients with sudden hearing loss evaluated between 1989 to 2001 were reviewed. All patients underwent pure tone audiometry, acoustic reflex and auditory brain revoked response (ABR) test. Magnetic resonance imaging (MRI) was performed followed by a second hearing test after one month. The findings were compared between patients with and without evidence of tumors on imaging, and between patients with tumors with and without recovery. RESULTS: Twenty-five patients (35%) had a diagnosis of acoustic tumor. Of these, six (24%) recovered hearing after one month. Five of them had small intracanicular tumors and one had a small extracanicular tumor. There was variability in the hearing loss. Five had a pathological ABR and one had normal ABR. CONCLUSIONS: We conclude that recovery from hearing loss does not exclude acoustic tumors and these patients therefore require full evaluation including MRI.

Cholesteatoma prevention by local treatment with vitamin A.

HYPOTHESIS: In view of the proven effectiveness of vitamin A for the treatment of hyperkeratosis, it was hypothesized that vitamin A may also be a feasible therapeutic option for cholesteatoma, which has similar histologic features. BACKGROUND: Cholesteatoma is a major cause of chronic ear disease, often requiring surgery. The recurrence rate may exceed 20%. Hyperkeratosis is a major sequela of tympanomastoid surgery, causing chronic ear hygiene problems. METHODS: The external auditory canals of 40 Mongolian gerbils were ligated to induce cholesteatomas. The ears were divided into three groups by type of treatment: group I, vitamin A drops (n = 40); group II, Cortisporin drops (n = 20); group III, no treatment (control subjects) (n = 20). Examination for the development and grade of cholesteatomas was performed after 9 months. RESULTS: Cholesteatomas developed in 26 ears (65%) of group I, 12 ears (60%) of group II, and 20 ears (100%) of group III. The differences were significant between groups I and III and groups II and III for both rate of cholesteatomas and severity of grade. There were no significant differences between groups I and II. CONCLUSION: Local treatment reduces the risk of cholesteatomas in ligated gerbilline ears. Cortisporin and vitamin A are equally effective as local treatments.

Carbon dioxide laser and silver halide infrared transmitting fibers for tympanoplasty: an experimental animal model.

OBJECTIVE: This study evaluates the effectiveness and safety of fiberoptic carbon dioxide (CO2) laser welding for graft closure of tympanic membrane perforations in an animal model. STUDY DESIGN AND SETTING: Tympanic membrane perforation was surgically induced in 11 eardrums of 7 given pigs. A lumbar facial graft was placed over the wound, and albumin drops served as a biologic solder. CO2 laser energy, transmitted through silver halide infrared transmitting fibers, was used for "spot-welding" along the circumference of the graft. The welded sites were evaluated by using a surgical microscope as well as by evaluating the sites histologically. RESULTS: Healing started 3 to 4 days after surgery and was completed within 3 weeks with the formation of a neotympanum. Some inflammation with granulation tissue was noted in 5 eardrums. CONCLUSIONS AND SIGNIFICANCE: These preliminary results indicate that CO2 laser tympanoplasty with a fiberoptic delivery system may be a promising new technique for the clinical setting.

Effects of passive smoking on odour identification in children.

BACKGROUND AND OBJECTIVE: The effect of passive smoking on odour identification in children has rarely been reported. This study assessed the ability of such young subjects to identify a variety of odours. METHODS: The study population consisted of 20 children, 10 who were exposed to passive smoke at home and 10 with nonsmoking parents. Ten odourants were tested: vinegar, ammonia, peppermint, roses, bleach, vanilla, cough drops, turpentine, licorice, and mothballs. Each child was presented with five test trays containing all 10 odourants in random order. RESULTS: Of the total of 500 odours presented, the control group correctly identified 396 (79%) and the study group identified 356 (71%) (p < .005). The study group tended to misidentify 4 of the 10 odourants tested, namely, vanilla, roses, mothballs, and cough drops-56 of 200 (28%), compared with 96 of 200 (48%) in the control group. This was a highly significant finding (p < .0005). CONCLUSION: This work demonstrated that children exposed to passive smoke have difficulty identifying odours in comparison with children raised in relatively smoke-free environments. The identification of four odourants, vanilla, roses, mothballs, and cough drops, was particularly diminished in this study group.

Connections of the facial and vestibular nerves: an anatomic study.

The facial and vestibulocochlear nerves emanate from the brain stem and then run parallel to each other within the internal auditory canal prior to their more peripheral distribution. Although anatomic connections between the facial and cochlear nerves have been described, reports outlining facial-vestibular anastomoses are few and may be found primarily in the non-English literature. The present study documents the existence of vestibulofacial neural connections as part of an anatomic dissection of 17 fresh human temporal bones.

Mucus retention cyst of the maxillary sinus: the endoscopic approach.

OBJECTIVE: To present our experience of endoscopic surgery for symptomatic mucus retention cyst of the maxillary sinus. DESIGN: Retrospective study. SETTING: Teaching hospital, Israel. PATIENTS: 60 patients with 65 symptomatic cysts of the maxillary sinus who were operated on endoscopically. Only patients with large cysts that filled at least 50% of the sinus space were included. INTERVENTION: A rigid nasal endoscope was used in all cases; most of the cysts were removed through the natural sinus ostium. RESULTS: Cysts recurred in only two patients during the first postoperative year. There were no complications from the procedure. CONCLUSION: The endoscopic approach to the treatment of maxillary sinus cyst is associated with a low rate of recurrence (3% in this study) and no complications, and we recommend it as the surgical procedure of choice.

Clinical characterization of genetic hearing loss caused by a mutation in the POU4F3 transcription factor.

OBJECTIVES: To describe the detailed auditory phenotype of DFNA15, genetic hearing loss associated with a mutation in the POU4F3 transcription factor, and to define genotype-phenotype correlations, namely, how specific mutations lead to particular clinical consequences. DESIGN: An analysis of clinical features of hearing-impaired members of an Israeli family, family H, with autosomal dominant-inherited hearing loss. SETTING: Department of Human Genetics and Molecular Medicine, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel; Department of Audiology, Rabin Medical Center, Petah Tiqwa, Israel; and audiological centers. PARTICIPANTS: Clinical features of 11 affected and 5 unaffected individuals older than 40 years from family H were studied. Mutation analysis was performed in 6 presymptomatic individuals younger than 30 years; clinical features were analyzed in 4 of these family H members. INTERVENTIONS: Hearing was measured by pure-tone audiometry and speech audiometry on all participating relatives of family H. Immittance testing (tympanometry and acoustic reflexes), auditory brainstem response, and otoacoustic emissions were done in a selected patient population. RESULTS: The patients presented with progressive high-tone sensorineural hearing impairment, which became apparent between ages 18 and 30 years. The hearing impairment became more severe with time, eventually causing significant hearing loss across the spectrum at all frequencies. CONCLUSIONS: Our results indicate that POU4F3 mutation-associated deafness cannot be identified through clinical evaluation, but only through molecular analysis. Intrafamilial variability suggests that other genetic or environmental factors may modify the age at onset and rate of progression.

Spontaneous pneumocephalus in the posterior fossa in a patient with a ventriculoperitoneal shunt: case report.

OBJECTIVE AND IMPORTANCE: A unique case of spontaneous pneumocephalus is described. It appeared a few years after the uneventful introduction of a cerebrospinal fluid shunt and was probably attributable to a defect of the posterior mastoid plate. CLINICAL PRESENTATION: A 65-year-old man presented with a subacute onset of vertigo, vomiting, and atactic gait instability. The patient had undergone a ventriculoperitoneal shunt implantation 2 years previously for communicating hydrocephalus. A computed tomographic scan revealed a posterior fossa pneumatocele without hydrocephalus. INTERVENTION: A simple mastoidectomy was performed. Detection of the area of the bone defect was followed by mastoid obliteration with abdominal fat. CONCLUSION: Clinicians should be aware that pneumocephalus can occur spontaneously, with or without obvious shunt problems. Treatment should be directed toward the area through which air penetrated the posterior fossa.

Sudden hearing loss as a presenting symptom of acoustic neuroma.

PURPOSE: Retrocochlear pathological findings may be suggested by findings on the brainstem response and acoustic sensorineural reflex (AR) tests. We describe the incidence of acoustic neuroma presenting as sudden hearing loss (SHL) and the effectiveness of the discrimination (DISC) test, the brainstem-evoked response, and AR test in predicting acoustic neuroma in patients with SHL. METHODS: We retrospectively reviewed the charts of all adult patients who presented to our center with sensorineural SHL between 1989 and 1995. Two groups were defined: those with cerebellopontine angle (CPA) tumor and those with negative imaging findings. Results of the brainstem-evoked response, AR, and DISC tests were compared. RESULTS: Forty patients were admitted with sensorineural SHL, of whom 19 (47.5%) had a CPA tumor. The latter group showed a significantly lower mean age and better results for the low frequencies on pure tone audiometry, as well as better brainstem-evoked response test results than the patients with negative imaging findings. There was also a significant difference between the groups for both the affected and unaffected ears on the discrimination test. CONCLUSION: Acoustic tumors may be a more common cause of sudden sensorineural hearing loss than previously suspected. The DISC test is a useful screening tool for acoustic tumor, whereas the brainstem-evoked response test shows poorer results in affected patients with sensorineural hearing loss than in other subgroups with different signs of acoustic neuroma. We recommend that young patients presenting with mild SHL who have normal results on the AR and brainstem-evoked response tests undergo magnetic resonance imaging to rule out CPA tumor.

Cochlear histopathologic analysis in diabetic rats.

HYPOTHESIS: This study aimed to show whether sensorineural hearing loss in diabetes mellitus is secondary to changes in the organ of Corti and stria vascularis. BACKGROUND: The high rate of sensorineural hearing loss in diabetes mellitus has led to much speculation as to whether there is an association between the two. METHODS: A well-established diabetic animal model was used to examine the inner ear histopathologically. RESULTS: No statistical difference in the inner and outer hair cells or in the stria vascularis was shown between the genetic-diabetic rats and control subjects. CONCLUSIONS: If there is hearing loss with diabetes, its pathogenesis does not involve damage to the hair cells or stria vascularis.

[The parapharyngeal space: tumors and surgical approaches].

The potential parapharyngeal space contains the great vessels of the neck, cranial nerves IX-XII, the sympathetic chain and lymph nodes. Surgical access is difficult and the infrequency of tumors in it make surgery of this part of the head and neck a challenge, 8 women and 2 men (mean age 54.7, range 31-70 years) with tumors of the parapharyngeal space operated on during the past 6 years are presented. The most common surgical approach was the cervical submandibular, used in 6 of our patients with relative ease and minimal complications. Tumors of deep lobe parotid origin should be operated through a transparotid approach. Large benign or malignant tumors have a better prognosis when the transcervical transmandibular approach is used, providing wider access.