Optimising the pre-treatment process before mobile ear surgery for chronic suppurative otitis media in Wolisso and Attat, Ethiopia.

BACKGROUND: Chronic suppurative otitis media is a major cause of long-standing hearing impairment in many Sub-Saharan African countries. METHODS: Attempts were made to optimise the pre-treatment process before mobile ear surgery for chronic suppurative otitis media in Wolisso, a semi-urban community in the Oromia region, and in Attat, a rural community in the Gurage region, both in the south-west of Ethiopia, between 2008 and 2010. This included special training for ENT nurses, and the use of a strict scheduling regime and improved topical treatment. RESULTS AND CONCLUSION: This strategy allowed effective middle-ear surgery to be carried out using simple means and with a mobile ear surgery team, the latter of which is only transiently but regularly on site.

Sclerosing bone dysplasias with involvement of the craniofacial skeleton.

In this review we provide a complete overview of the existing sclerosing bone dysplasias with craniofacial involvement. Clinical presentation, disease course, the craniofacial symptoms, genetic transmission pattern and pathophysiology are discussed. There is an emphasis on radiologic features with a large collection of CT and MRI images. In previous reviews the craniofacial area of the sclerosing bone dysplasias was underexposed. However, craniofacial symptoms are often the first symptoms to address a physician. The embryology of the skull and skull base is explained and illustrated for a better understanding of the affected areas.

Localization of the gene for hyperostosis cranialis interna to chromosome 8p21 with analysis of three candidate genes.

Hyperostosis cranialis interna (HCI) is a rare autosomal dominant disorder characterized by intracranial hyperostosis and osteosclerosis, which is confined to the skull, especially the calvarium and the skull base. The rest of the skeleton is not affected. Progressive bone overgrowth causes nerve entrapment that leads to recurrent facial nerve palsy, disturbance of the sense of smell, hearing and vision impairments, impairment of facial sensibility, and disturbance of balance due to vestibular areflexia. The treatment is symptomatic. Histomorphological investigations showed increased bone formation with a normal tissue structure. Biochemical parameters were normal. Until today the disease has been described in only three related Dutch families with common progenitors and which consist of 32 individuals over five generations. HCI was observed in 12 family members over four generations. Patients are mildly to severely affected. Besides HCI, several bone dysplasias with hyperostosis and sclerosis of the craniofacial bones are known. Examples are Van Buchem disease, sclerosteosis, craniometaphyseal dysplasia, and Camurati-Engelmann disease. However, in these cases the long bones are affected as well. Linkage analysis in a family with HCI resulted in the localization of the disease-causing gene to a region on chromosome 8p21 delineated by markers D8S282 and D8S382. Interesting candidate genes in this region are BMP1, LOXL2, and ADAM28. Sequence analysis of these genes did not reveal any putative mutations. This suggests that a gene not previously involved in a sclerosing bone dysplasia is responsible for the abnormal growth in the skull of these patients.

Neurophysiologic, audiometric and vestibular function tests in patients with hyperostosis cranialis interna.

OBJECTIVE: Hyperostosis cranialis interna (HCI) is an autosomal dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina. The aim of this study is to describe the value of several neurophysiological, audiometric and vestibular tests related to the clinical course of the disorder. METHODS: Ten affected subjects and 13 unaffected family members were recruited and tested with visual evoked potentials, masseter reflex, blink reflex, pure tone and speech audiometry, stapedial reflexes, otoacoustic emissions, brainstem evoked response audiometry and electronystagmography. RESULTS: Due to the symmetrical bilateral nature of this disease, the sensitivity of visual evoked potentials (VEPs), masseter reflex and blink reflex is decreased (25-37.5%), therefore reducing the value of single registration. Increased hearing thresholds and increased BERA latency times were found in 60-70%. The inter-peak latency I-V parameter in BERA has the ability to determine nerve encroachment reliably. 50% of the patients had vestibular abnormalities. No patient had disease-related absence of otoacoustic emissions, because the cochlea is not affected. CONCLUSION: In patients with HCI and similar craniofacial sclerosing bone dysplasias we advise monitoring of vestibulocochlear nerve function with tone and speech audiometry, BERA and vestibular tests. VEPs are important to monitor optic nerve function in combination with radiological and ophthalmologic examination. We do not advise the routine use of blink and masseter reflex.

Imaging features and progression of hyperostosis cranialis interna.

BACKGROUND AND PURPOSE: HCI is a unique autosomal-dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina, whereby the mandible is affected to a lesser extent. The aim of this study is to describe the specific radiologic characteristics and course of the disorder. MATERIALS AND METHODS: CT scans of affected individuals within 1 family were analyzed and compared with scans of their unaffected family members and with an age- and sex-matched control group. Linear measurements were performed of the inner table, the medulla, and the outer table of different skull locations, and attenuation (density) measurements of the same regions were recorded. Neuroforamina widths were recorded as well. RESULTS: There was significant thickening of the skull in the frontal, parietal, temporal, and occipital regions, which was mainly due to thickening of the inner table of the skull. The attenuation of the deposited hyperostotic bone was lower than normal cortical bone. CONCLUSIONS: HCI is the only genetic bone dysplasia known that is confined to the craniofacial area. The hyperostotic bone is less attenuated than normal cortical bone. The observed radiologic abnormalities explain the possible impairment of the olfactory, optic, trigeminal, facial, and vestibulocochlear nerves.

Phenotypic manifestations and management of hyperostosis cranialis interna, a hereditary bone dysplasia affecting the calvaria and the skull base.

Hyperostosis cranialis interna is a hereditary bone disorder that is characterized by endosteal hyperostosis and osteosclerosis of the calvaria and the skull base (OMIM 144755). The progressive bone overgrowth causes entrapment and dysfunction of cranial nerves I, II, V, VII, and VIII, its first symptoms often presenting during the second decade. This study analyzes the clinical course of 13 affected individuals of three related families (32 individuals). The disorder appears to have an autosomal-dominant transmission pattern. Facial and vestibulocochlear nerve dysfunction are most frequently reported. Surgical decompression of the accessible impaired cranial nerves is advised in the early symptomatic period or even in the presymptomatic period in high-risk individuals.

Facial nerve decompression via middle fossa approach for hyperostosis cranialis interna: a feasible therapeutic approach.

UNLABELLED: Hyperostosis cranialis interna is an autosomal dominant disorder characterised by endosteal hyperostosis and osteosclerosis of the skull base and calvaria, leading to compression and dysfunction of cranial nerves I, II, VII and VIII. CASE REPORT: We report the use of bilateral surgical decompression of the internal auditory canals to treat hyperostosis cranialis interna in an eight-year-old girl presenting with bilateral facial palsy due to hyperostosis cranialis interna. INTERVENTION AND OUTCOME: Using a middle fossa craniotomy approach, both internal auditory canals were unroofed and cranial nerves VII and VIII were decompressed, with a one-year interval between sides. The mimic function recovered. One year post-operatively, the right and left facial sides had been restored to House-Brackmann grades I and II, respectively. CONCLUSION: This is the first report of the use of surgical decompression of the internal auditory canal in a case of hyperostosis cranialis interna. Surgical decompression of the internal auditory canal is recommended therapeutically, but may also be performed prophylactically in younger patients with hyperostosis cranialis interna.

Synchronous bilateral epithelial-myoepithelial carcinoma of the parotid gland: case report and review of the literature.

Synchronous bilateral malignancy in the parotid glands is extremely rare. The English literature reveals nine case reports. The most common synchronous bilateral malignancies are acinic cell carcinoma. Epithelial-myoepithelial carcinoma is an uncommon neoplasm comprising 1% of all salivary gland tumours. In this case report, we describe, to our best of knowledge, the first case of a patient with a synchronous bilateral epithelial-myoepithelial carcinoma of the parotid gland. The clinical histopathological and immunohistochemical peculiarities are elucidated. Imaging studies like ultrasonography are mandatory for both parotid glands and upper necks in the clinical presence of a unilateral parotid gland tumour.

Marked differences in survival rate between smokers and nonsmokers with HPV 16-associated tonsillar carcinomas.

Oncogenic human papillomavirus (HPV) is a causative agent in a subgroup of head and neck carcinomas, particularly tonsillar squamous cell carcinomas (TSCC). This study was undertaken because controversial data exist on the physical status of HPV-DNA and the use of p16(INK4A) overexpression as surrogate HPV marker, and to examine the impact of HPV and tobacco consumption on the clinical course of TSCC. Tissue sections of 81 TSCC were analyzed by HPV 16-specific fluorescence in situ hybridization (FISH) and p16(INK4A)-specific immunohistochemistry. Results were correlated with clinical and demographic data. HPV 16 integration was detected by FISH as punctate signals in 33 out of 81 (41%) TSCC, 32 of which showed p16(INK4A) accumulation. Only 5 out of 48 HPV-negative tumors showed p16(INK4A) immunostaining (p < 0.0001). The presence of HPV furthermore correlates significantly with low tobacco (p = 0.002) and alcohol intake (p = 0.011), poor differentiation grade (p = 0.019), small tumor size (p = 0.024), presence of a local metastasis (p = 0.001) and a decreased (loco)regional recurrence rate (p = 0.039). Statistical analysis revealed that smoking significantly increases the risk of cancer death from TSCC and that non-smoking patients with HPV-containing TSCC show a remarkably better disease-specific survival rate. HPV 16 is integrated in 41% of TSCC and strongly correlates with p16(INK4A) overexpression, implicating the latter to be a reliable HPV biomarker. Patients with HPV-positive tumors show a favorable prognosis as compared to those with HPV-negative tumors, but tobacco use is the strongest prognostic indicator. These findings indicate that oncogenic processes in the tonsils of non-smokers differ from those occurring in smokers, the former being related to HPV 16 infection.

Primary temporal bone secretory meningioma presenting as chronic otitis media.

We report an extremely rare case of a secretory meningioma primarily involving the temporal bone. A 56-year old female patient presented to us with a history of a chronic otitis media and unilateral hearing loss. Diagnostic investigations revealed a tumor arising from the temporal bone without signs of intracranial involvement. Histopathological examination showed a meningioma of the secretory type. The tumor was partially resected and serial imaging at follow-up revealed no extension of the tumor. No new symptoms developed 1 year after surgery. Secretory meningioma is a rare meningioma subtype and extracranial presentation in the temporal bone is very unusual. We present the first case of a primary temporal bone secretory meningioma in the otorhinolaryngological literature. As radical as possible surgical excision with serial imaging at follow-up is recommended.

Paraneoplastic syndromes in patients with primary malignancies of the head and neck. Four cases and a review of the literature.

Paraneoplastic syndromes rarely affect patients with head and neck cancer. Four patients with different histological types of head and neck cancer are presented in which the primary malignancy was preceded and/or accompanied by a paraneoplastic syndrome. In the first patient erythrodermia preceded the diagnosis of a nasopharyngeal carcinoma. The second patient presented with a B cell lymphoma of the nasopharynx in association with the syndrome of inappropriate secretion of arginine vasopressine (Schwartz-Bartter syndrome). In the third patient paraneoplastic polyarthritis had been diagnosed 5 months before a hypopharyngeal carcinoma was diagnosed. In the last patient the paraneoplastic anti-Hu positive encephalomyelitis was associated with a primary malignancy in the larynx with neck metastases. Diagnostic procedures, treatment and follow-up of these patients are reported and accompanied by a review of the literature.

Infiltrating aggressive epitheliosis of the temporal bone: a newly described disease entity.

Two patients with a highly destructive histologic nondysplastic squamous epithelial lesion of the temporal bone are described. Both tumors were characterized by the production of copious amounts of keratin material which progressively extended in the temporal bone, surrounding bony structures, soft tissue and intracranially, with concomitant functional disorders. Only small amounts of epithelial cells were found. CT scanning revealed extensive and progressive destruction of the temporal bone, skull base in both patients, clivus and arch of atlas in one patient, besides intracranial and intracerebellar abscess formation. Despite extensive surgical therapy, the disorder was unmanageable. Histology showed a normal squamous epithelial basal cell layer with remarkable acanthosis and extensive hyperkeratosis. Material obtained at autopsy demonstrated intracerebellar extension of keratin material in one patient and in the hemicerebellectomy specimen of the other patient. To our knowledge, this disease entity has not been reported before.

Compliance, quality of life and quantitative voice quality aspects of hands-free speech.

CONCLUSIONS: With the use of a new automatic stoma valve (ASV) it appears possible to rehabilitate patients who have previously been unsuccessful in acquiring hands-free speech. As well as making daily ASV use possible for an additional group of patients, this new device was also appreciated by many patients as an additional rehabilitation tool for specific occasions. Despite statistically significant improvements in aspects of voice and breathing using this novel ASV, improvement of peristomal adhesion is probably the main factor needed to further increase success rates. Nevertheless, our results show that it makes sense to keep trying to achieve hands-free speech, even if previous attempts have failed. OBJECTIVE: To make a long-term (6 months) assessment of compliance and aspects of voice, breathing and quality of life using a new ASV: the Provox FreeHands heat and moisture exchanger (HME). MATERIAL AND METHODS: This was a prospective clinical multicentre trial in 79 laryngectomized patients (8 regular ASV users, 58 previously unsuccessful users and 13 new users). Data were collected at baseline and after 1 and 6 months by means of European Organization for Research and Treatment of Cancer Quality of Life questionnaires and specific structured questionnaires concerning compliance, skin adhesion, voicing and pulmonary aspects. An objective assessment of voice parameters (maximum phonation time, maximum phonation time while counting, dynamic loudness range and number of pauses in a standard read-aloud text) was made for comparison of different stoma occlusion methods (digital occlusion via an HME and two different ASVs). A subjective assessment of overall voice quality was made. RESULTS: After 6 months, 19% of patients used the new ASV on a daily basis (mean 5 h/day), while 57% used it on an irregular basis as an additional rehabilitation tool for special occasions. Two-thirds of the study group indicated that they would continue to use the new ASV after the study period. With respect to the objective parameters, statistically significantly better maximum phonation times and dynamic loudness ranges were observed with the new ASV compared to the Blom-Singer ASV. However, the best results for all the objective parameters were obtained with digital occlusion via the Provox HME.

Thymic cyst in the neck.

A rare case of a thymic cyst in the neck containing both thymus and parathyroid tissue in a 7-year-old boy is presented. The clinical presentation, diagnostic evaluation, surgical management and histopathological features are described. The embryology of cervical thymic cysts and the differential diagnosis of cystic neck masses in children are briefly reviewed. The diagnosis is seldom made preoperatively. Surgical resection is the treatment of choice for definitive diagnosis, resolution of symptoms and cure.

Quality of life assessment in laryngectomized individuals: do we need additions to standard questionnaires in specific clinical research projects?

OBJECTIVES: To assess, whether the EORTC questionnaires QLQ-C30 and QLQ-H&N35 give enough detailed information to study specific quality of life (QoL) related issues in laryngectomized individuals. DESIGN: Multicentre, prospective clinical trial; baseline measurement with EORTC questionnaires and an additional questionnaire, focusing on specific postlaryngectomy problems. SETTING: Head and Neck Department of one Comprehensive Cancer Center and three Academic Medical Hospitals in the Netherlands. PARTICIPANTS: Eighty laryngectomized individuals, selected to participate in a prospective clinical trial on hands-free tracheoesophageal speech. MAIN OUTCOMES MEASURES: Comparison of QoL dimensions, as assessed with the standard EORTC questionnaires, with the information obtained with additional questions, aimed at discovering in more detail, e.g. voice and respiratory problems in laryngectomized individuals. RESULTS: Based on the EORTC QoL questionnaires a good overall and voice specific QoL-level was found. However, the additional questionnaire showed that especially concerning voice and respiration more specific information was obtained. For example, despite an overall satisfaction with many aspects of the voice in more than three-quarters of the patients, speaking in a noisy environment was reported by 63% of the patients as being a serious problem, and a significant relation could be established between pulmonary and voicing problems (r = 0.28, P < 0.05), something also undetectable with the EORTC questionnaires. CONCLUSIONS: These findings underline the necessity to develop and use more specific additional questionnaires as an adjunct to the existing EORTC questionnaires, when studying specific symptoms in laryngectomized individuals, especially in order to detect intervention related changes over time.

Help-seeking behaviour of hearing-impaired persons aged > or = 55 years; effect of complaints, significant others and hearing aid image.

OBJECTIVE: To determine factors influencing help-seeking behaviour in hearing-impaired persons aged > or = 55 years MATERIAL AND METHODS: The study comprised 1,419 persons (480 females, 939 males) aged > or = 55 years who participated voluntarily in a driving test. Pure-tone audiometry was performed in all subjects. Participants without a hearing aid with an average hearing loss of > or = 30 dB in the better ear for the frequencies 0.5, 1, 2 and 4 kHz were asked how they perceived their hearing by means of a structured questionnaire. The questionnaire provided insight into why some hearing-impaired people seek help from a general practitioner (GP) and others do not. Four factors were examined. RESULTS: A total of 483/1.419 (34%) participants tested had a hearing loss exceeding 30 dB. Fewer than half of these had visited their GP with complaints of hearing impairment. Of those who had not sought help from a GP, 57% nevertheless judged their hearing as poor. Social pressure exerted by significant others was less in the non-consulters group. Hearing aid image did not differ significantly between the two groups. CONCLUSION: The help-seeking behaviour of hearing-impaired elderly people is related to the degree of hearing disability and the social pressure exerted by significant others as well as the willingness to try hearing aids.