Lateral semicircular canal fistula in cholesteatoma: diagnosis and management.

The objective of this retrospective study was to present the authors' experience on the management of labyrinthine fistula secondary to cholesteatoma. 695 patients, who underwent tympanoplasty for cholesteatoma, in a University Hospital between 1993 and 2013 were reviewed, to select only those with labyrinthine fistulas. 42 patients (6%) had cholesteatoma complicated by fistula of the lateral semicircular canal (LSCC). The following data points were collected: symptoms, pre- and postoperative clinical signs, surgeon, CT scan diagnosis, fistula type, surgical technique, preoperative vestibular function and audiometric outcomes. Most frequent symptoms were unspecific, such as otorrhea, hearing loss and dizziness. However, preoperative high-resolution computed tomography predicted fistula in 88 %. Using the Dornhoffer and Milewski classification, 16 cases (38 %) were identified as stage 1, 22 (52 %) as stage II, and 4 (10 %) as stage III. The choice between open or closed surgical procedure was independent of the type of fistulae. The cholesteatoma matrix was completely removed from the fistula and immediately covered by autogenous material. In eight patients (19 %), the canal was drilled with a diamond burr before sealing with autologous tissue. After surgery, hearing was preserved or improved in 76 % of the patients. There was no statistically significant relationship between the extent of the labyrinthine fistula and the hearing outcome. In conclusion, a complete and nontraumatic removal of the matrix cholesteatoma over the fistula in a one-staged procedure and its sealing with bone dust and fascia temporalis, with sometimes exclusion of the LSCC, is a safe and effective procedure to treat labyrinthine fistula.

Preoperative computed tomography scan may fail to predict perilymphatic gusher.

OBJECTIVES: A stapes gusher is a very rare event in ear surgery, but the consequences for hearing can be dramatic. It can usually be predicted by characteristic radiologic abnormalities. We report 2 cases of gusher without any abnormalities seen on the preoperative computed tomography scans. METHODS: The first case was in a 30-year-old man with a bilateral mixed hearing loss. The gusher occurred after a stapedotomy performed with a microdrill. The oval window was plugged with a vein graft, and a fluoroplastic piston was inserted. The second case was in a 39-year-old woman with a family history of hearing loss who presented with a bilateral mixed hearing loss. The footplate was fractured during the stapedotomy drilling and was covered with a temporalis fascia graft that was fixed with a fluoroplastic piston. RESULTS: The first patient had no cerebrospinal fluid leakage and no vertigo or tinnitus. He did have a sensorineural hearing loss. The second patient had dizziness and tinnitus. Postoperative magnetic resonance imaging scans were performed, but again no features were identified that might have predicted these cases. CONCLUSIONS: Surgeons should be reminded that a preoperative computed tomography scan may fail to detect the risk of a perilymphatic gusher.

Rhinoscleroma: a French national retrospective study of epidemiological and clinical features.

BACKGROUND: Rhinoscleroma (RS) is a rare, chronic, granulomatous disease of the upper respiratory tract that is associated with infection with Klebsiella rhinoscleromatis. RS is more common in certain geographic regions than in others, but other risk factors and the pathogenesis of RS remain unclear. METHODS: We sent a standardized questionnaire to all pathologists and otolaryngology specialists in French University Hospitals and asked whether they had seen patients with RS in the previous 16 years (1990-2005). We then retrospectively reviewed the files of all patients identified. RESULTS: We collected 11 cases of RS, with a median patient age at diagnosis of 35.7 years (range, 5-72 years). The 3 patients with a familial history of RS presented with early-onset forms of RS; 1 had an uncommon aggressive presentation of the disease with ischemic stroke. Two unrelated consanguineous families were identified, 1 of which included 2 affected siblings. Two patients with sporadic disease were positive for HIV infection. All patients were living in France, but most were immigrants from areas where RS is endemic (North Africa, 3 of the 11 patients; West Africa, 4 patients; and Turkey, 1 patient). The probable duration of exposure to K. rhinoscleromatis in endemic areas varied widely: 0-28 years. Clinical features and outcome also varied considerably among cases. Biopsies had been performed for all patients and revealed granulomas containing Mikulicz cells. Cultures of biopsy tissue were positive for K. rhinoscleromatis in 5 of the 11 cases. Prolonged antibiotic treatment was administered to all patients, as follows: ciprofloxacin (7 patients), third-generation cephalosporins (2), tetracycline (2), and clofazimine (2). Eight of the 11 patients did not experience relapse during extended periods of follow-up (1.3-12 years). Relapses in 3 patients were confirmed by a second biopsy. CONCLUSIONS: The occurrence of early-onset RS in multiplex and/or consanguineous families suggests that genetic control of the host response to K. rhinoscleromatis may be involved in the pathogenesis of RS in endemic areas.

Adenocarcinoma of Ethmoid: a GETTEC retrospective multicenter study of 418 cases.

OBJECTIVE: To determine risk factors and evaluate the treatment of ethmoid adenocarcinoma. Epidemiologic data were recorded and compared with the literature. MATERIALS AND METHODS: A multicenter and retrospective study. The medical records of 418 patients who had presented with ethmoid adenocarcinoma at 11 French hospitals from 1976 to 2001 were analyzed to determine the clinical characteristics and treatment of the disease. RESULTS: The gender ratio was 2.8 men per 1 woman. Toxic exposure was classic for this lesion, exposure to wood and leather for most cases. The mean age was 63 years (range 31-91). Symptoms were nonspecific and based on clinical rhinologic signs. Nasal endoscopy after mucosal retraction was found useful to evaluate the extension of the lesion and to perform biopsies. Computed tomography scan and magnetic resonance imagery must be carried out prior to treatment to define extra nasal extension. The survival rate was significantly influenced by the size of the lesion (T4, N+) and extension to brain or dura. Surgery with postoperative radiotherapy remains the treatment of choice. Total excision must be a major priority, as confirmed in our series. CONCLUSION: This retrospective study was, to our knowledge, the largest ever reported in the literature. This series confirmed the risk factor of this lesion as well as the lesion's influence on the survival rate. Surgery is the most important part of the treatment. Local recurrences were responsible for the poor prognosis of this lesion.

Rhinoscleroma of the sinuses.

INTRODUCTION: Rhinoscleroma is a rare chronic, granulomatous disease of the respiratory tract. OBJECTIVE: The aim of this paper was to report 2 unusual cases of rhinoscleroma and to review the literature. MATERIAL: We present two cases of sinus rhinoscleroma diagnosed and treated at the ENT Department of a French University hospital. RESULTS AND CONCLUSION: Rhinoscleroma primarily affects the nasal cavity but the nasopharynx (18%-43%), larynx (15%-40%), trachea (12%) and bronchi (2% to 7%) can also be involved. However, the paranasal sinuses are usually free of disease. Rhinological signs are generally the first reported by patients. CT scan and MRI are useful for diagnosis. Diagnosis of rhinoscleroma was based on histological characteristics and presence of Klebsiella rhinoscleromatis on biopsy cultures. In most cases treatment involves prolonged antibiotic therapy with aesthetic surgical reconstruction when necessary. However, rhinoscleroma is difficult to eradicate and its recurrence rate is high.

Laryngeal sensation recovery by reinnervation in rabbits.

OBJECTIVES/HYPOTHESIS: To assess the possibilities of restoring laryngeal sensation in an animal model by way of the internal branch of the superior laryngeal nerve (ibSLN) bilateral section and anastomosis to itself or to transposition nerves (i.e., lingual, glossopharyngeal, and great auricular nerves). STUDY DESIGN: Prospective study using New Zealand rabbits. METHODS: Six groups of rabbits were operated on and evaluated: healthy controls (n = 6); section without reinnervation (denervated group, n = 7); section and reinnervation with ibSLN (SLN-SLN group, n = 9); and section and anastomosis with the lingual nerve (lingual group, n = 7), the glossopharyngeal nerve (glossopharyngeal group, n = 6), and the great auricular nerve (GA group, n = 7). After 9 months, recovery of a laryngeal closure reflex was assessed by stimulation of the epiglottis and nerve anastomosis. RESULTS: Laryngeal sensation was restored in 14.3% in the denervated group, 66.6% in the SLN-SLN group, 71.4% in the lingual group, 100% in the GA group (P < .001), 50% in glossopharyngeal group. Some anastomoses were severed. When anastomosis was intact, a laryngeal closure reflex was observed in 91.7% of the rabbits of the SLN-SLN group (P < .001), 80% in the lingual group (P < .001), 100% in the GA group (P < .05) and 100% of the glossopharyngeal group. CONCLUSIONS: Rehabilitation of supraglottic laryngeal sensation is feasible by way of anastomosis of the ibSLN to itself, but also to the lingual, glossopharyngeal, and great auricular nerves. These results suggest that this type of procedure may be useful in humans to prevent aspiration and subsequent pneumonia as related to lesions of the laryngeal reflex pathway. It could also be considered one stage toward the functional rehabilitation of a transplanted larynx.

"Bleeding polyp" of the nasal septum: an uncommon lesion in adults.

Inflammatory hemangioma of the nasal septum, known as "bleeding polyp," is an uncommon lesion in adults. We report the case of a 20-year-old woman who presented with left nasal hemangioma revealed by unilateral epistaxis and obstruction. The patient had a medical history of septoplasty performed 5 years earlier. This benign tumor can be spontaneous or posttraumatic and occurs in young postpubertal adults or patients over 40 years of age, without a sex preponderance. The site of origin is most frequently the cartilaginous septum. The appearance and clinical signs often mimic malignancy, and a biopsy for histologic confirmation of the diagnosis is crucial. The capillary hemangioma is more frequently observed than the cavernous type. This tumor does not present spontaneous involution, and treatment is based on a surgical excision including the mucosa and the underlying perichondrium, after computed tomographic and/or nuclear magnetic resonance examination.

Intralabyrinthine schwannomas: a case series with discussion of the diagnosis and management.

OBJECTIVE: By extracting cases of intralabyrinthine schwannomas (ILS) from the sum of all vestibular schwannomas, we aim to identify and analyze unique features of its presentation. This allows us to refine the management protocol of this rare condition. DESIGN: This is a retrospective study of all patients seen in the Department of Otorhinolaryngology-Head and Neck Surgery, Rouen University Hospital, with either ILS or ILS with intracanalicular extension (ILS-IAC) between 2001 and 2011. A literature search was performed and results combined to draw conclusions on management strategies. METHOD: Three patients with ILS and 6 patients with ILS-IAC were identified. We retrieved data on age, sex, symptoms, audiometry, imaging, and management. Pure tone audiometry and speech discrimination score were assessed and hearing classification recorded. Facial nerve function and vestibular function were documented throughout. The diagnostic and surveillance imaging (MRI with or without CT) were reviewed. RESULTS: The average age at presentation was 62.8 years and the sex ratio was (male: female) 4:5. An ipsilateral hearing loss was observed in all patients. Eight of 9 patients had tinnitus at presentation, 2 had rotatory vertigo, and 1 patient had a facial palsy and hemifacial spasm. In 2 cases, the labyrinthine extension was initially missed. The patient presenting with a large tumor and facial palsy was operated on without delay. The others underwent MRI surveillance, with 4 requiring surgery at a later stage. No postoperative facial palsies were encountered other than the one that had been present preoperatively. CONCLUSION: Frequency of ILS is underestimated because of poor diagnostic criteria. These tumors have often been described as having features, which resemble Ménière's disease, which is not found in our series. In the absence of tumor progression or disabling symptoms, their management is surveillance/medical, and when surgery is considered, facial paralysis and recurrence rates seem low. The treatment of IAC-ILS differs from that of ILS.

Nasolacrimal duct oncocytoma: an unusual cause of chronic epiphora.

Epiphora has traditionally fallen under the purview of ophthalmologists. However, owing to the development of endoscopic dacryocystorhinostomy, this condition has been increasingly observed in otolaryngologic practice. We report the case of a woman with a 4-month history of right epiphora and dacryocystitis. Nasal endoscopy revealed the presence of a tumor at the inferior meatus. Histopathologic examination of a biopsy specimen identified the tumor as an oncocytoma. Surgical excision via a lateral rhinotomy approach was performed. At 3 years postoperatively, the patient was disease-free. Lacrimal sac and nasolacrimal duct tumors are rare, but they should be considered as a possible etiology in patients with acquired epiphora because most of these tumors are malignant.

Role of radiotherapy in the treatment of nasoethmoidal adenocarcinoma.

OBJECTIVE: To assess the efficacy of radiotherapy in the treatment of nasoethmoidal adenocarcinoma. DESIGN: Multicenter, retrospective study. SETTING: Eleven French hospitals. PATIENTS: The medical records of 418 patients who presented with nasoethmoidal adenocarcinoma from January 1, 1976, through December 31, 2001, were evaluated. A total of 324 patients were treated with a combination of surgery and radiotherapy, and 55 were treated with surgery only. MAIN OUTCOME MEASURES: Survival rates, disease recurrence, and postoperative complications. RESULTS: The 5-year Kaplan-Meier survey revealed survival rates of 64.5% for the surgery-only group and 70.8% for the combined-treatment group. In the surgery-only group, 28 patients (51%) had disease recurrence (24 local, 2 regional, and 2 distant). Of the 55 patients in the combined-treatment group, 31 patients (56%) had disease recurrence (29 local, 1 regional, and 1 distant). Immediate postoperative complications in the combined-treatment group were hemorrhages in 2 patients, meningitis in 3 patients, and cerebrospinal fluid leakage in 4 patients, but no deaths occurred. In the surgery-only group, 1 patient had meningitis, 2 had cerebrospinal fluid leaking but no hemorrhage, and 5 died postoperatively. CONCLUSION: The results of this retrospective study suggest that radiotherapy can be used to treat nasoethmoidal adenocarcinoma, but its usefulness should be confirmed with further prospective studies.

Unusual presentation of oropharyngeal Kaposi's sarcoma.

BACKGROUND: The aim of this article is to focus ear, nose, and throat (ENT) practitioners on a pathology rarely seen within the head and neck mucosa. METHODS: A 62-year-old black African man was referred to our ENT department for dysphagia and hoarseness. Physical ENT examination revealed a smooth normal mucosal mass on the left lateral pharyngeal wall, which masked the inlet of the larynx, and bilateral cervical lymph nodes but no other mucosal lesions. Anti-human immunodeficiency virus antibodies were found to be negative. Hemoglobin, hematocrit, white blood cell count, and platelet count were normal. Serum protein electrophoresis was also normal. A CT scan confirmed the lesion and lymph node involvement. Treatment consisted of a transoral endoscopic resection with ligature of the pedicle, with the patient under general anesthesia. The patient's improved medical condition permitted 11 cycles of bleomycin (15 mg/3 weeks). RESULTS: A 5-month fibroscopic follow-up control showed no recurrence, and total clinical regression was observed after 1 year. Three years later, all ENT lesions had disappeared, with no adenopathy or mucosal lesions. CONCLUSION: Kaposi's sarcoma is a pathology that the ENT practitioner must keep in mind when patients present with a laryngopharyngeal mass. The symptomatic cases were all surgically treated; however, death subsequently occurred.

Squamous cell carcinoma of the nasal columella: a retrospective study of 66 cases from the GETTEC.

Squamous cell carcinoma of the nasal columella (SCCNC) is a rare disease. We aimed to define a strategy for the diagnosis and management of nasal columella squamous cell carcinoma. Medical records of 66 patients presenting with columella squamous cell carcinoma in nine French hospitals, from 1980 to 2003, were evaluated to determine the clinical characteristics and current treatment of the disease. Mean age was 69 years. The sex ratio was one female for three males. Majority of the lesions were T1 N0 according to the classification international union against cancer. Patients underwent one of the three treatments: surgery alone for the T1 lesions, radiotherapy for tumors of T2-T3 and combined (surgery and radiotherapy) for T4 lesions. The 5-year Kaplan-Meier survival test was 39% and no difference was found between the therapeutic groups. Thirty-five (53%) tumor recurrences were observed with a median time of 43 months. As regards TNM classification and treatment, no significant difference between the two groups disease free/recurrence was found. Prognosis of early lesions was considered better than the advanced lesions. SCCNC is difficult to manage and has a poor prognosis. No therapeutic solution has yet been confirmed in the treatment of this pathology.