A very handy spine!

Behind the retro auricular groove, hidden by the pinna, on the surface of the mastoid, there is, a bony outgrowth, known by any otorhinolaryngologist worthy of the name, located above and behind the entrance to the external auditory canal in front of the supra meatic fossa: Henle's spine. In this historical note the authors retrace the moment of the dicovery of this primordial relief of otological surgery and the life of its discoverer, the German anatomis Jakob Henle, a true malesrtom which mixes science, art politics, and love.

Jugular foramen paragangliomas: management, outcome and avoidance of complications in a series of 75 cases.

Jugular foramen paragangliomas are rare skull base tumours posing multiple complex diagnostic and management problems. We did a study to evaluate surgical technique, outcome and complications in 75 cases of tumours treated by multidisciplinary approach (i.e. combined neurosurgery, neuroradiology, ear, nose and throat surgery and intensive care unit team). Retrospective study on 75 consecutive patients with jugular foramen paragangliomas treated surgically from 1989 to 2005. Preoperative balloon occlusion test was performed in all patients as well as embolization (100%). A combined limited infratemporal and juxtacondylar approach was used in all patients. Gross total resection was achieved in 59 patients (78.7%). The most common complication was represented by lower cranial nerve deficits in five patients (6.6%), which was only temporary in three. Postoperative facial nerve weakness occurred in five cases (6.6%) and resolved in three of them. The remaining two patients underwent facial nerve reconstruction by hypoglossal/facial nerve anastomosis. Four patients (5.3%) had a postoperative cerebrospinal fluid leak, which was successfully treated by lumbar drainage. Two patients (2.7%) died because of complications related to surgical injury of lower cranial nerves: one patient developed aspiration pneumonia and septicemia and the second one developed a large cervico-bulbar hematoma that led to severe respiratory distress and ultimately global cerebral hypoxia. Paragangliomas are rare and complex skull base lesions that may be managed with low morbidity and mortality if a multidisciplinary approach is considered. Facial and lower cranial nerve postoperative deficits can be limited.

[The ultra-rapid cinematography of the larynx, its contributions in speech pathology]. / La cinématographie ultra rapide du larynx, ses apports en phoniatrie.

The development in the medical field of high speed cinematography and its dissemination in the field of speech pathology will probably change the way we view the larynx and diagnose its disorders. So far only the stroboscope could inform us about the appearance of vocal cord vibration but with certain limitations. Indeed the wave motion of the vocal cords is really only apparent motion. It is made possible through the phenomenon of retinal persistence of images and light intermittent vocal folds set to the frequency of the voice and out of phase by a few Hertz. This technique has several disadvantages: The need for the voice to trigger the strobe light; a low number of frames per second (25 fps) recorded; frame loss for the period between unlit two flashes; limitation in the study of the upper voice spectrum (gearing). The ultra-rapid cinematography brings a big difference in design since the digital recording can shoot up to 4000 frames per second with permanent lighting of the larynx. The slow reading of short sequences permits us to view the actual movement of vibrating structures, and to analyze the behavior of the vibrator during the transitional phases of the attack, depreciation and termination of sound. The footage in high resolution permits a detailed analysis of the movements of opening and closing of the vocal cords in phonation and respiration, and the diagnosis of lesions.

Eustachian tube melanoma treated by double surgical approach.

OBJECTIVES: We report a case of an eustachian tube melanoma treated by a double surgical approach. MATERIAL AND METHODS: A 56-year-old man consulted for a recurrent left serous otitis media. RESULTS: CT-scan and MRI showed a lesion filling completely the middle ear, extending along the Eustachian tube up to the nasopharynx, with enhancement after injection. The biopsy of the lesion confirmed the diagnosis of malignant melanoma. After left internal carotid artery occlusion, the removal of the lesion was performed by a double surgical approach: combination of an endoscopic transnasal and a retroauricular approach. Radiotherapy was adjuvant therapy. CONCLUSION: Eustachian tube melanoma is a rare occurrence. Surgical treatment must be radical, what represents a challenge considering the localization; it can be obtained with the double surgical approach proposed in our case report.

Isolate vertigo crisis revealing an endolymphatic sac tumor.

UNLABELLED: Endolymphatic sac tumors are rare entities that have a destructive potential on the temporal bone. They are aggressive tumors presenting as low-grade papillary adenocarcinoma, but there are no reports of metastasis in the literature. The Von Hippel-Lindau disease is a hereditary condition caused by germinal mutations of the tumor-suppressor VHL-gene. We present the case of an endolymphatic sac tumor associated with the Von Hippel-Lindau disease at a 46-year-old patient revealed by an isolate vertigo crisis, discussing the management of the tumor and the clinical, imaging, genetic and histopathologic features of it. CONCLUSIONS: Endolymphatic sac tumors have recently been described as part of the Von Hippel-Lindau disease, a genetic disorder involving the development of hypervascular tumors. The treatment depends on the size of the tumor, however surgical approach is the most successful choice and can be associated or not with radiotherapy.

Role of vestibular input in triggering and modulating postural responses in unilateral and bilateral vestibular loss patients.

The aim of this study was to determine whether the greater medial-lateral (ML) instability observed in patients with compensated unilateral vestibular loss (UVL), tested on a seesaw platform with eyes closed, is task-dependent. UVL patients, categorized into three groups according to time since lesion (1 week, 1 month and 1 year), bilateral vestibular loss patients and age-matched healthy control subjects were tested in three dynamic postural tasks. These tasks involved different supports - a seesaw platform (Satel), a platform generating horizontal linear translations (Synapsys) and foam rubber placed on a static platform - each requiring different somatosensory cues to maintain equilibrium. Displacements of the subjects' center of pressure in both the anterior-posterior (AP) and ML directions were recorded by strain gauges within the platforms. Only tests performed with eyes closed were analyzed. Bilateral vestibular loss patients fell during foam and seesaw trials but not on the platform generating translations. We previously reported that UVL patients had greater postural oscillations on the seesaw platform in the ML compared to AP direction. In this study, we show similar ML/AP differences in patient performance on foam when standing with 'feet close together'. In contrast, these differences were not found when patients were tested on linear translation or on foam standing with feet apart. In conclusion, the postural performance of patients with vestibular loss depends on the exact task used to measure postural stability. UVL patients are less stable when subjected to movement in the ML direction because of the biomechanical constraints of the tasks and/or the availability of proprioceptive information.

[Association of an arachnoid cyst with heterotopic neuroglial tissue in the internal auditory canal]. / Association d'un kyste arachnoïdien et d'une hétérotopie de tissu glial dans le méat auditif interne.

OBJECTIVES: We report a case of an association of an arachnoid cyst with heterotopic neuroglial tissue in the internal auditory canal. MATERIAL AND METHODS: A 66-year-old woman consulted for cochleovestibular syndrome. RESULTS: MRI demonstrated a lesion with spontaneous hypersignal on T1- and T2-weighted images, instigating surgical exploration. We discovered a hematic arachnoid cyst associated with heterotopic neuroglial tissue arising in the internal auditory canal. CONCLUSION: An arachnoid cyst arising within the cerebellopontine angle or the internal auditory canal is a rare occurrence. Clinical manifestations are identical with those produced by a cochleovestibular schwannoma. MRI usually demonstrates a nonenhancing isointense cystic mass with cerebrospinal fluid on all sequences (hypointense on T1-weighted and hyperintense on T2-weighted images). These lesions are usually monitored. Spontaneous hypersignal on T1- and T2-weighted images makes diagnosis difficult, as in our case, leading to surgical exploration.

Endoscopic transsphenoidal surgery for cholesterol granulomas involving the petrous apex.

Surgery for cholesterol granulomas involving the petrous apex has traditionally been performed via a lateral skull base approach. We present a case-series of four cholesterol granulomas treated through the endoscopic-transsphenoid approach over the last 10 years. Drainage was successful and symptomatic improvement was obtained in all cases (follow-up 6 months-10 years). Primary 'sphenoid' lesions, which can be widely drained and remain marsupialised, should be differentiated from primary 'petrous' lesions that can be removed safely through the sphenoid sinus only in case of extension medial to the internal carotid artery (ICA). We feel that the endoscopic transsphenoidal approach is a safe and effective way to access cholesterol granulomas of the petrous apex.

[Dilation of the internal auditory canal and intracanalicular vestibular schwannoma: what are the mechanisms involved?]. / Dilatation du méat acoustique interne et schwannome vestibulaire intracanalaire: quels mécanismes?

OBJECTIVES: To present a unique case of unilateral widening of the internal auditory canal (IAC) with no significant contact with an ipsilateral intracanalicular vestibular schwannoma (VS), raising the issue of the cause(s) of this IAC widening. METHODS: The medical record and radiologic data were reviewed of a patient presenting an enlarged unilateral IAC, which led to the diagnosis of an intracanalicular VS that could not account for the dilation. RESULTS: The patient had a unilateral dilation of the IAC that did not match the ipsilateral VS he had. As a result, this case motivated discussion of whether such dilation of the IAC was congenitally asymmetrical or the result of the mechanisms involved in the widening of the IAC. CONCLUSIONS: Although asymmetry of IAC is a current notion, this case demonstrates a contrario that increased pressure exerted on the walls of the IAC cannot be the only mechanism in such widening.

Kinetics of gentamicin uptake and release in the rat. Comparison of inner ear tissues and fluids with other organs.

The kinetics of entry and release of gentamicin was investigated in fluids and tissues of the inner ear of the rat, as well as in renal cortex, and in organs that do not share susceptibility to the toxic effects of aminoglycosides. Various modes of administration were used to achieve different patterns of drug plasma concentrations. Electrophysiological and histological examinations were performed to correlate pharmacokinetics and ototoxicity. Results show that: the uptake of the drug by the inner ear tissues is dose dependent and manifests a rapid saturation kinetics with a concentration plateau of about 1 micrograms/mg of protein. The low ratio of the perilymph and endolymph to plasma concentrations argues against the concept of an accumulation of the drug in the inner ear over drug levels in plasma, which has been considered as the basic mechanism of ototoxicity. In renal cortex, the kinetics appears similar to that of the inner ear but the concentrations achieved are 10-fold higher than in cochlear tissues. In other organs (liver, heart, lung, and spleen), no saturation could be demonstrated within the duration of the experiment. Ototoxicity seems to be related to the penetration of the drug into compartment(s) from which the half-life of disappearance is extremely slow. Rapid uptake, early saturation, and long exposure of the tissues to the drug may account for the development of toxicity in inner ear and kidney.

[Idiopathic sudden sensorineural hearing loss is not, at this time, an otologic emergency]. / La surdité brusque idiopathique n'est pas, aujourd'hui, une urgence sensorielle.

OBJECTIVES: To evaluate the influence of the treatment delay for sudden sensorineural hearing loss (SSHL) on the final hearing recovery and the prognostic value of the audiometric shapes and the hearing recovery at the end of treatment. PATIENTS AND METHODS: Prospective study including 321 patients admitted in the emergency care center for a recent idiopathic SSHL. Classification according to the audiometric shape in 5 types. All patients received steroids (1 mg/kg per day) and, in case of ascending shape, steroids and mannitol during 6 days. Hearing recovery was evaluated according to the following parameters: 1) hearing recovery (initial PTA-PTA at D6 or D30); 2) incidence of hearing recovery (initial PTA-PTA at D6 or D30)/initial PTA x 100%). RESULTS: Whatever the audiometric type, delay in initiating treatment did not have any influence on the final outcome. Audiometric classification had a good prognostic value, since the ascending shape had a better hearing recovery than descending shape or the sub or total anacusis. Hearing recovery at D6 is a prognostic factor on the final outcome. CONCLUSIONS: Idiopathic SSHL cannot be considered, nowadays, as an otologic emergency.

[Seromucous otitis]. / Les otites séromuqueuses.

OBJECTIVES: Otitis media with effusion are defined as the persistence of middle ear effusion for more than 3 weeks. If the diagnostic is easy, questions remain about pathogeny and treatment. MATERIALS AND METHODS: Literature was reviewed regarding the pathogeny and the best treatment strategy. RESULTS: Except in the case of middle ear effusion due to trauma, effusion is an exudate due to mucous cell metaplasia. The main causal factor is middle ear inflammation, which is secondary to viral or bacterial infection. Inflammation causes dysfunction of the sodium transports in the middle ear. Responsibility of the otitis media with effusion in the genesis of the various chronic otitis media remains controversial. Treatment is justified when otitis media last more than 3 months, that is to say few months observation is required. The aim of treatment is to reduce local inflammation and to treat effusion. Prevention and treatment of local inflammation is difficult. Indeed, it is difficult to avoid rhinitis that is mainly viral. Effusion must be treated in order to avoid local middle ear deterioration and language deficiency. Insertion of tympanostomy tube is the only effective treatment. It decreases middle ear depression and Eustachian tube obstruction and restores the mucociliary clearance. Adenoidectomy and amygdalectomy are not effective in otitis media with effusion but, in association with tympanostomy tube, could decrease recurrence of acute otitis media. CONCLUSION: Otitis media with effusion remains a frequent disorder, for which the only effective treatment is the tympanostomy tube.

Enhanced sodium absorption in middle ear epithelial cells cultured at air-liquid interface.

CONCLUSION: As we demonstrated previously that transcription of alpha-ENaC was correlated with oxygen tension in the culture medium, this study suggests that the increase in alpha-ENaC expression observed under ALI conditions may result from greater oxygenation of ME cells. OBJECTIVE: The physiology of the middle ear (ME) is primarily concerned with keeping the cavities fluid-free, to allow transmission of sound vibrations from the eardrum to the inner ear. ME epithelial cells are thought to play a key role in this process as they actively absorb sodium and water in order to clear any excess fluid present in the cavities. MATERIAL AND METHODS: As an air-liquid interface (ALI) model has been shown to improve differentiation and enhance sodium absorption in other respiratory epithelia, we established an ALI model for ME cells. RESULTS: ME cells cultured under ALI conditions exhibited a fourfold increase in sodium absorption, which was not related to either a metabolic effect or to enhanced morphological differentiation, but instead to an increase in expression of the alpha-subunit of the epithelial sodium channel (alpha-ENaC).

[Idiopathic sudden sensorineural hearing loss: a review]. / Surdités brusques idiopathiques.

Idiopathic Sudden Sensorineural Hearing Loss (ISSHL) remains one of the major unsolved otologic emergencies. It is characterized by the onset of an unilateral sensorineural hearing loss developing within 24 hours, and averaging on pure tone audiogram at least 30 dB HL for three subsequent octave steps, with no marked vestibular symptoms and no identifiable cause. ISSHL is a syndrome covering several heterogeneous entities resulting from different pathogenetic mechanisms. At this time, the audiogram is the unique tool which may help clinicians to identify these entities and provide a classification based on 5 types of hearing loss. Numerous experimental and clinical studies have investigated the mechanisms by which infectious, ischemic, mechanic or immunologic insults may induce cochlear dysfunction. However, extrapolation to humans and rationale therapeutic approaches to ISSHL remain uncertain. SSHL being a diagnosis of exclusion, retrocochlear and neurologic etiologies should be eliminated. No argument allows to consider ISSHL a therapeutic emergency. More precisely, the experimental data presently available on cochlear physiology suggests that a treatment could have some chance to be effective if undertaken within minutes following the onset of ISSHL, a condition never encountered in daily practice. Conversely, it is not justifiable to impute the absence of hearing recovery to a delay in therapy. The various therapeutic strategies currently recommended are highly empirical and should be questionned in terms of cost-effectiveness, the most common being high-dose corticosteroids. New investigation tests are required for improving our approach to ISSHL.

["Endolymphatic" cochleo-vestibular disorders]. / Syndromes cochléo-vestibulaires endolymphatiques.

Certain peripheral vertigos undoubtedly do not raise from neuro-sensorial structures but rather of the secretory structures and can be called "endolymphatic". They deserve to be individualized because concerning different pathogenic mechanisms and certainly are relevant for a specific treatment. We hope that in the next years new investigation tools will allow to explore in a noninvasive way the endolymphatic contents and dysfunctions of these structures.

Cochlear electrical stimulation: influence of age of implantation on Fos immunocytochemical reactions in inferior colliculi and dorsal cochlear nuclei of the rat.

The influence of age at the time of implantation of a stimulating electrode unilaterally in the inner ear on central auditory pathways was investigated in rats deafened shortly after birth. Immunoreactivity for Fos served as a functional marker of neuronal activity. Electrodes were implanted in the left cochlea of rats aged 3 weeks or 4 months. Stimulation lasted 45 minutes, then rats were sacrificed and tissues processed for immunocytochemistry. The younger animals showed significantly more neurons with Fos immunoreactivity bilaterally in the dorsal cochlear nuclei (DCN) and inferior colliculi (IC) than the older rats or control animals with normal hearing receiving the same stimulation. Activity was more prominent in the left DCN and right IC. The results show that electrical stimulation of the inner ear is more effective in younger animals in eliciting gene expression associated with development of a functional network in the auditory pathways. This suggests that deaf children should be provided with cochlear implants as early as possible.

Intratympanic gentamicin injections for Meniere disease: vestibular hair cell impairment and regeneration.

The authors treated 22 patients with intratympanic gentamicin. Vestibular function was measured using caloric and head impulse tests and vestibular evoked myogenic potentials induced by high amplitude sounds and short duration galvanic currents. Roughly one-third of the patients, after initially losing their caloric responses and displaying refixation saccades to head impulse tests, recovered within 2 years of the lesion. Vertigo did not recur in patients in whom the galvanic response was abolished.

The diagnostic value of fine-needle aspiration biopsy under ultrasonography in nonfunctional thyroid nodules: a prospective study comparing cytologic and histologic findings.

Fine-needle aspiration biopsy (FNAB) of thyroid nodules has been proposed as a preoperative screening method to reduce the number of patients with benign nodules referred for surgery. Only a few previously published studies have compared cytologic results with a systematic surgical control; furthermore, the role of ultrasound guidance for FNAB in obtaining representative material has been poorly evaluated. The authors tried to assess the diagnostic value of FNAB performed under ultrasonography in a series of patients with a cold nodule and a systematic surgical control. This prospective study included 132 consecutive patients, 19 men and 113 women, aged from 18 to 82 years. The rate of carcinomas was 13.8%. A very high rate of adequate material was obtained (96.2%). Sensitivity, specificity, and global accuracy were 95%, 87.7%, and 89%, respectively. These results, which are equivalent to those previously published but associated with a low level of insufficient material, emphasize the diagnostic value of thyroid FNAB.

Middle ear adenoma. A tumor displaying mucinous and neuroendocrine differentiation.

Middle ear adenoma (MEA) is a distinctive, rare entity that appears to be derived from the lining epithelium of the middle ear mucosa. We report four cases of MEA displaying the typical histologic growth pattern. Two distinct tumor cell immunophenotypes were identified in all cases; the first type exhibited positivity with anti-epithelial membrane antigen and anti-keratin antibodies, and the second type showed immunoreactivity with anti-keratin, anti-vimentin, and anti-neuron-specific enolase antibodies. Ultrastructural studies revealed bidirectional mucinous and neuroendocrine differentiation, demonstrated by the presence of two distinct cell types containing apically located mucous granules and basally concentrated neuroendocrine granules, respectively. The presence of neuroendocrine differentiation was supported by the immunohistochemical detection of vasoactive intestinal polypeptide in the tumor cells in one case and neuron-specific enolase in three cases. These findings suggest that the potential for mixed mucinous/neuroendocrine differentiation described in other endodermally derived tumors also exists in middle ear mucosa. We also believe that the rare lesions diagnosed as primary carcinoid tumors of the middle ear might in fact be MEA with predominant or only neuroendocrine differentiation. The clinical course of our four cases and our review of the pertinent literature confirm the benign nature of MEA and indicate that these tumors should be treated by complete local excision without additional therapy.

Ion transports in the middle ear epithelium.

Ion transports in the middle ear epithelium have been recently characterized. Experimental data using cell culture have found the existence of a sodium transepithelial transport that drives a water flow. This is thought to play a key role in the maintain of air-filled and fluid-free cavities. Impairment of this process is involved in the pathogenesis of secretory otitis media, which is the main cause of acquired hearing loss. Several modulations of this transport have been evidenced: (i) reactive oxygen species induced an endogenous synthesis of prostaglandin E2 (PGE2), which in turn increased the cAMP level and modulated ion transport rate; (ii) steroids increased the expression of the alpha subunit sodium channel mRNA, which changes paralleled the modulation of ion transport in the middle ear epithelium; (iii) moderate hypoxia selectively and reversibly decreased the rate of sodium transport, as a result of a parallel decrease in alpha epithelial sodium channel subunit mRNA level. These modulations may explain the course of middle ear pathology. However, the development of an in vivo model has become mandatory to assess the relevance of these data in the pathophysiology of the middle ear.