En Hamac tympanoplasty and canalplasty for optimal type 1 tympanoplasty outcomes.

OBJECTIVE: Multiple tympanoplasty techniques have been developed with numerous differences in grafting and approach. This study aimed to improve type 1 tympanoplasty outcomes by using the 'en hamac' technique as well as performing a complete canalplasty for anterior perforations. METHOD: A retrospective review was performed using the prospective Otology-Neurotology Database tool for otological surgery. All primary type 1 tympanoplasty cases performed for tympanic membrane perforations from 2010 to 2016 were selected for analysis, all performed by one author. Minimal clinical and audiometric follow up was 18 months. RESULTS: Tympanic membrane perforation closure was achieved in 62 of the patients (96.88 per cent). None of the en hamac cases had residual or recurrent perforation (p = 0.02). The mean remaining air-bone gap was 8.50 dB. The remaining air-bone gap was less than 10 dB in 72.55 per cent, 10-20 dB in 25.49 per cent and more than 20 dB in 1.96 per cent. CONCLUSION: Using the en hamac technique for anterior perforations as well as systematically performing a complete canalplasty provides multiple surgical advantages with excellent post-operative results.

Transoral robotic surgery for parapharyngeal lesions: a case series of four benign tumours.

OBJECTIVES: The parapharyngeal space (PPS) is an anatomically complex space in the vicinity of vital structures. With the introduction of the daVinci robot in head and neck surgery, the surgical robotic system is now being used to gain direct access to the parapharyngeal space and to excise the tumors endoscopically. This study evaluates the outcomes of four patients with benign PPS tumors treated with a transoral robotic surgery approach in a single centre. MATERIAL AND METHODS: All patients with benign tumors of the PPS who underwent transoral resection (between January 2012 and June 2014) using the robot were included in this retrospective study. RESULTS: The study population comprised of two males and two females with a mean age of 52 (range 34-66 years). The parapharyngeal mass was successfully transorally removed in all cases. Overall, mean length of stay was 3.25 days with mean time to oral diet of one day. No intraoperative, perioperative or postoperative complications were encountered. The histological diagnosis was pleomorphic adenoma in two cases (50%). The other two cases were: schwannoma and angioma. There were no recurrences on radiological investigations during a mean follow-up of 14.5 months. MRI scan showed a stable residual fibrotic lesion in case of the angioma. The preoperative complaints of mucus in the throat, painless swelling of the soft palate or throat burden of all patients resolved after surgery. CONCLUSION: With the assistance of the surgical robotic system, benign tumors within the PPS can be excised safely without neck incisions. Further long-term evaluation is needed to define patient selection and the role of TORS for PPS neoplasms.

Congenital laryngeal cyst: an unusual cause of stridor in the neonate.

BACKGROUND: Congenital laryngeal cysts are a rare, but potentially fatal, cause of airway obstruction in infants and children. Most laryngeal cysts are acquired. Here, we describe a congenital laryngeal cyst, its treatment, and its presentation immediately after birth. CASE REPORT: A newborn child developed stridor very shortly after birth, due to a large, congenital saccular cyst. The diagnosis was based on a laryngoscopy and imaging studies, which detected an extralaryngeal extension through the cricothyroid membrane. The cyst was removed with an endoscopic procedure and a CO2-laser. Afterwards, the cyst recurred, but was successfully removed with an external approach. CONCLUSION: Clinical manifestations of laryngeal cysts in neonates shortly after birth are rare. Here, we present an interesting clinical case. We also concisely reviewed the literature on the epidemiology, clinical presentation, diagnosis, classification, and therapy for laryngeal cysts.

Imaging of intralabyrinthine schwannomas: a retrospective study of 52 cases with emphasis on lesion growth.

BACKGROUND AND PURPOSE: Only a few case reports and small series of intralabyrinthine schwannomas (ILSs) have been reported. The purpose of this study was to assess prevalence, MR characteristics, location, clinical management, and growth potential/patterns of ILSs in the largest series reported. MATERIALS AND METHODS: Lesion localization, MR characteristics, lesion growth, and clinical management were reviewed in 52 patients diagnosed with an ILS between February 1991 and August 2007 in 2 referral centers. The number of ILSs and vestibulocochlear schwannomas in the cerebellopontine angle/internal auditory canal was compared to assess the prevalence. RESULTS: ILSs most frequently originate intracochlearly, are hyperintense on unenhanced T1-weighted images, enhance strongly after gadolinium administration, and are sharply circumscribed and hypointense on thin heavily T2-weighted 3D images. The scala tympani is more frequently or more extensively involved than the scala vestibuli. Follow-up MR imaging, available in 27 patients, showed growth in 59% of subjects. Growth was seen from the scala tympani into the scala vestibuli and from the scala vestibuli to the saccule and vice versa. Twelve lesions were resected, and the diagnosis of ILS histopathologically confirmed. CONCLUSION: ILSs can account for up to 10% of all vestibulocochlear schwannomas in centers specializing in temporal bone imaging, grow in more than 50%, and are most frequently found intracochlearly, often anteriorly between the basal and second turn. Cochlear ILSs most often originate in the scala tympani and only later grow into the scala vestibuli. Growth can occur from the cochlea into the vestibule or vice versa through the anatomic open connection between the perilymphatic spaces in the scala vestibuli and around the saccule.

Post-traumatic pulsatile tinnitus: the hallmark of a direct carotico-cavernous fistula.

Following trauma to her right frontal region, a 68-year-old woman suffered bilateral, benign, paroxysmal, positional vertigo and a left-sided, longitudinal petrosal bone fracture, with secondary facial palsy and ossicular luxation. From the onset, the patient complained of pulsatile, left-sided tinnitus. After eight weeks, she developed left-sided ocular symptoms, progressing from conjunctival hyperaemia and orbital oedema to an abducens nerve palsy, and ultimately to heart failure. The case and the final diagnosis of carotico-cavernous fistula are discussed. Guidelines are proposed for a diagnostic approach to pulsatile tinnitus and for the optimal management of patients presenting with pulsatile tinnitus associated with ocular symptoms.

Reparative granuloma related to perilymphatic fistula.

Reparative granuloma is defined as an 'exaggeration of the normal reparative process' (Schuknecht) after stapes surgery, often resulting in a destruction of the labyrinth. It is ascribed to a foreign body reaction, yet there are insufficient histological data to prove this and although rare, the problem is still with us. We encountered two patients in whom the history contains elements evoking a perilymphatic fistula. Cerebrospinal fluid causes irritation of the middle ear mucosa. This is demonstrated in an exemplary way in case of a spontaneously developed cerebrospinal fluid leak from a defect in the tegmen tympani. The leak itself may be very small, but is almost always surrounded by a large mass of granulation tissue. Since the chemical composition of perilymph and cerebrospinal fluid is known to be identical, a persistent perilymphatic leak might likewise be responsible for the formation of a granuloma in the middle ear, eventually invading and destroying the labyrinth. Laboratory experiments to investigate this hypothesis are recommended.

Eosinophilic fungal rhinosinusitis (EFRS): a distinct CT/MRI-entity? A European experience.

OBJECTIVE: To determine the value of radiological features in the diagnosis of Eosinophilic Fungal Rhinosinusitis (EFRS). STUDY DESIGN: Retrospective review of the radiological materials of 65 patients with documented Eosinophilic Fungal Rhinosinusitis treated at the same institution. METHODS: Evaluation by the ENT surgeon and the head and neck radiologist. RESULTS: EFRS was more common in female patients in this series. Fifty-four (83%) patients were above 30 years of age, with a peak of 18 patients (27.7%) in the seventh decade. All the patients except one (98.5%) showed bilateral mucosal thickening on unenhanced CT scans. Thirty-eight patients (58%) showed increased intrasinus attenuation on unenhanced CT scans. Thirty-seven patients (57%) showed opacification of at least one sinus; 25 (38%) showed osteitis; 11 (17%) had erosion of the sinus wall and only one patient showed minor expansion of an involved sinus. In 6 patients, typical hyperattenuation patterns on CT scans, together with distinctive MRI images, were highly suggestive of EFRS. CONCLUSION: Our data show that hyperattenuation on CT images with bone window settings suggests the presence of EFRS. This hyperattenuation is more clearly seen with soft-tissue window settings. When necessary, adjunctive MRI can provide information which might be highly predictive for the diagnosis of EFRS. However, non-specific imaging findings of chronic rhinosinusitis (CRS) should also be seen as possible EFRS pathology.

Varicella zoster virus: beyond facial paralysis.

J. Ramsay Hunt's hypothesis that herpes zoster oticus results from a reactivation of the herpes zoster virus in the geniculate ganglion, has been supported by the demonstration of varicella zoster viral DNA in the geniculate ganglion of the side with facial paralysis in patients with Ramsay Hunt syndrome, with the use of the polymerase chain reaction. Similarly, DNA of the varicella zoster virus has been identified in the spiral and vestibular ganglion as well. We report on three patients with cochleovestibular symptoms as the first manifestations of Ramsay Hunt syndrome. A 64-year old woman and a 72-year old man presented with vertigo and an auricular herpetiform eruption. Only the woman developed later on a mild facial paralysis. A 58-year old man presented with an acute cochleovestibular syndrome, serologically proven to be a varicella zoster viral reactivation, which was followed three weeks later by the typical cutaneous recrudescence. We believe that these cases result from reactivation of latent varicella zoster virus in the spiral and/or vestibular ganglion. As the varicella zoster virus is dormant in the non-neuronal satellite cells, the facial symptoms in our patients as well as the high incidence of cochleovestibular symptoms in classical Ramsay Hunt syndrome can be explained by viral transmission across the nerves inside the internal auditory canal. Therefore, we think there are grounds to recommend a prompt treatment with an antiviral and a corticosteroid agent, not only in case of an acute facial paralysis but also when confronted with an acute cochleovestibular syndrome.

Ramsay Hunt syndrome: pathophysiology of cochleovestibular symptoms.

Ramsay Hunt's hypothesis that herpes zoster oticus results from reactivation of the varicella zoster virus (VZV) in the geniculate ganglion is supported by the detection of viral genome in archival temporal bones of normals and Ramsay Hunt patients by the polymerase chain reaction. Ramsay Hunt syndrome is characterized by the presence of cochleovestibular symptoms in association with facial paralysis. VZV has also been demonstrated in the spiral and/or vestibular ganglion. Two cases are reported in which cochleovestibular symptoms outweighed the facial nerve symptoms, presumably representing VZV reactivation in the spiral and/or vestibular ganglion. From these observations and the known dormancy of VZV in non-neuronal satellite cells, it is argued that the cochleovestibular symptoms in Ramsay Hunt syndrome may result from VZV transmission across the nerves inside the internal auditory canal and that prompt treatment with an antiviral-corticosteroid combination might be justified in the management of any acute non-hydropic cochleovestibular syndrome.

Symmetrical ethmoidal metastases from ductal carcinoma of the breast, suggesting transcribrosal spread.

Symmetrical ethmoidal metastases from ductal carcinoma of the breast, suggesting transcribrosal spread. While half of breast cancers develop metastases, the appearance of metastatic disease in paranasal sinuses from this origin is very rare. Eighteen other cases were found in the literature, dating from 1939 till now. A case of metastatic breastcancer presenting as a subacute therapy-resistant pansinusitis is described. The perfect symmetry was misleading. Bilateral ethmoidal biopsies were compatible with metastases from a ductal adenocarcinoma. Further investigation revealed meningeal carcinomatosis in the supra-orbital region and locoregional recurrence in the mastectomy scar and axilla. Comparing these 19 cases in chronological order, it was noticed that symptoms at time of diagnosis shift from those of space occupying lesions to those suggestive for sinusitis. This shift could be explained by earlier diagnosis. High index of suspicion is the key to diagnosis. Earlier diagnosis does not result in longer survival since in most cases patients have already widespread disease and die within one year. Most authors mention the role of the vertebral venous plexus in hematogeneous spreading of tumor cells. Another pathway of hematogenous spread is via (occult) lung metastases. This case prompts the hypothesis of transcribrosal spread from meningeal involvement.

CT and MR imaging of congential abnormalities of the inner ear and internal auditory canal.

The embryology of the inner ear must be known as many of the inner ear malformations present as a result of the arrest during the various stages of embryology. These malformations are described in this "embryologic" perspective and specific names for certain malformations are no longer used. Both CT and MR can be used to look at inner ear malformations but often both techniques are complementary. However, CT is preferred when associated middle- or external ear malformations must be excluded. Magnetic resonance is preferred when subtle changes in the membranous labyrinth or abnormalities of the nerves in the internal auditory canal must be visualised. The CT and MR technique must however be adapted as more and more subtle congenital malformations can only be seen when the right technique is used. The heavily T2-weighted gradient-echo or fast spin-echo MR techniques are mandatory if malformations of the inner ear must be excluded. The purpose of this paper is to describe the techniques used to study these patients and to give an overview of the most frequent and important congenital malformations which can be found in the inner ear and internal auditory canal/cerebellopontine angle.

Spontaneous cerebrospinal fluid otorrhea from a tegmen defect: transmastoid repair with minicraniotomy.

Spontaneous cerebrospinal fluid (CSF) otorrhea is a rare condition that presents in 2 clinical categories. In congenital labyrinthine malformations, it leads to bouts of meningitis in a hearing-impaired child. In the adult age group, a spontaneous CSF leak almost always results from a dural and bony defect in the tegmen area. Possible pathogenic mechanisms include progressive sagging and rupture of dura through a congenital tegmen dehiscence and progressive bone erosion by aberrant arachnoid granulations. These patients usually present with a middle ear effusion, resulting in clear discharge after myringotomy with tube insertion. Based on 4 patients with a CSF leak from a tegmen defect, this report reviews the clinical findings and diagnostic approach. The surgical management by a 5-layer closure using a transmastoid approach with minicraniotomy is outlined. This procedure offers a relatively simple and reliable method for repair without the inherent risks of a middle fossa craniotomy.

Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging.

PURPOSE: To introduce aplasia or hypoplasia of the vestibulocochlear nerve (VCN) as a possible cause of hearing loss and to identify the magnetic resonance (MR) imaging characteristics of this entity. MATERIALS AND METHODS: In seven patients with congenital deafness or unexplained sensorineural hearing loss, MR imaging enabled diagnosis of aplasia or hypoplasia of the VCN. Axial (0.7-mm) three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) images and parasagittal reconstruction images perpendicular on the course of the VCN were obtained. Twenty normal inner ears were also studied; their findings were compared with those of the patients. RESULTS: The facial nerve and inferior and superior vestibular and cochlear branches of the VCN were identified on the MR images in the 20 normal inner ears. Aplasia of the VCN was detected in two patients with normal labyrinths but with a severe stenosis of the internal auditory canal. A common VCN with absence of the cochlear branch was found bilaterally in two patients with a congenital malformation of the labyrinth. A common VCN with absence or hypoplasia of the cochlear branch was found in three patients with normal internal auditory canals and labyrinths. CONCLUSION: Submillimetric gradient-echo images (eg, 3DFT-CISS) should always be used to exclude aplasia or hypoplasia of the cochlear branch of the VCN in all cochlear implant candidates and patients with congenital deafness. This entity, which can occur with or without associated labyrinthine malformation, should be confirmed in two planes.

Inner ear malformations in patients with sensorineural hearing loss: detection with gradient-echo (3DFT-CISS) MRI.

The sensitivity of different MRI sequences in the detection of inner ear malformations in patients presenting with sensorineural hearing loss (SNHL) and/or vertigo was evaluated. We studied 650 patients presenting with SNHL and/or vertigo, clinically not suspected of having inner ear malformations. The sensitivity of T1-weighted, Gd-enhanced T1-weighted and (when available) T2-weighted spin-echo images, and three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) gradient-echo images, to unexpected malformations was assessed. Inner ear malformations were found in 15 (2.3%) of these patients. Enlargement of the endolymphatic duct and sac was the most frequent malformation, found in 11 patients. The 3DFT-CISS images showed all lesions; the other sequences were less sensitive and the pathology was missed, partially or only retrospectively seen in 11 of the 15 patients. Therefore, in addition to the routine unenhanced and Gd-enhanced T1-weighted and T2-weighted images, thin gradient-echo (3DFT-CISS) images are necessary to detect all clinically unexpected inner ear malformations in patients presenting with vertigo and/or SNHL.

Catecholamine-secreting paragangliomas at the skull base.

Paragangliomas (glomus tumors) comprise 15% of all neoplasms at the skull base. Despite extensive growth, these tumors usually do not secrete active biogenic substances into the circulation in sufficient quantities to produce symptoms. When they do secrete large amounts of catecholamines, they will cause symptoms that mimic a pheochromocytoma. The still confusing nomenclature of paragangliomas is reviewed, and the clinical work-up, surgical treatment, and follow-up of five patients with catecholamine-secreting paragangliomas of temporal bone (3), infratemporal fossa (1), and nasopharynx (1) are presented and discussed.

Experimental evidence of ototoxicity of ear drops. A review of the literature.

Evidence of ototoxicity as derived from laboratory experiments on animal models and from a few prospective controlled studies in man is reviewed. Data are gathered for the different components and antibiotics contained in ototopical preparations. An attempt is made to answer the question whether these laboratory findings are valid in the clinical situation in man.

Magnetic resonance examination of the inner ear and cerebellopontine angle in patients with vertigo and/or abnormal findings at vestibular testing.

The inner ears of 167 patients with vertigo and/or abnormal findings at vestibular testing were studied using magnetic resonance (MR). Pathology potentially explaining vertigo was found in 54 patients, and was detected in the posterior fossa (28%), the internal auditory canal (28%) and the membranous labyrinth (44%). The overall percentage of pathology and the percentage of pathology found in the membranous labyrinth was high and was probably influenced by the referral pattern in our hospital where high resolution MR of the inner ear (three dimensional Fourier transformation-constructive interference in steady state sequence; 3DFT-CISS sequence) is available. Unenhanced, and especially gadolinium (Gd)-enhanced T1-weighted spin-echo images, are needed to detect most of the pathology inside the internal auditory canal, and some of the lesions inside the membranous labyrinth. 3DFT-CISS images are the only images that can show fibrous obliteration of the intralabyrinthine fluid spaces, and are therefore necessary to recognise most of the intralabyrinthine pathology. Finally, T2-weighted spin-echo images are best suited to demonstrate cerebellar or brain stem infarction. An additional MR-angiography sequence (three dimensional Fourier transformation-fast imaging with steady precession; 3DFT-FISP) is used when vascular compression of the cochleovestibular nerve is suspected. MR is the method of choice to look for pathology in patients with vertigo, and allows detection of pathology that remains invisible with other imaging techniques. However, well adapted sequences are needed to detect these lesions.

[Hearing loss following lumbar puncture: case report and literature study]. / Gehoorsdaling na lumbaal punctie: gevalsbespreking met litteratuurstudie.

This report describes a patient with permanent hearing loss after spinal anesthesia. The literature is reviewed, particularly concerning incidence and possible causes. The incidence is surprisingly high and related to the size of the needle. The role of the cochlear aqueduct as a transmitter of a decreased fluid pressure in the subarachnoid space to the perilymph of the labyrinth is discussed.

Constructive interference in steady state-3DFT MR imaging of the inner ear and cerebellopontine angle.

PURPOSE: To assess the value of a three-dimensional Fourier transformation MR technique "CISS" (constructive interference in steady state) in imaging the inner ear. SUBJECTS: We studied 50 normal inner ears (40 axial, 10 coronal) and 10 pathologic inner ears in 60 patients. RESULTS: The cochlea, semicircular canals, and vestibulum were visualized in detail. Cranial nerve VII and the cochlear, superior vestibular, and inferior vestibular branch of cranial nerve VIII were identified in 90%, 94%, 80%, and 88% of the cases, respectively. A vascular loop was recognized inside the internal auditory canal in 6%, and in the porus in 30%, of the cases. The high signal of the cerebrospinal fluid and labyrinthine fluids (perilymph and endolymph) on the CISS images made excellent delineation of tumors in the cerebellopontine angle and internal canal possible and allowed detection of tumoral labyrinth involvement. The thin sections, high resolution of the images, and capability of producing multiplanar and three-dimensional reconstructions often offered additional information. CONCLUSIONS: The CISS sequence allows detailed study of the normal and pathologic inner ear and promises to be highly valuable in the demonstration of the vascular loop.