Donato Parrotto

Detection of postoperative relapsing/residual cholesteatomas with diffusion-weighted echo-planar magnetic resonance imaging.

Purpose: To assess the capability of echo-planar diffusion-weighted magnetic resonance imaging (MRI) (EPI-DWI) in diagnosing relapsing/residual cholesteatomas after canal wall-up mastoidectomy. Materials and Methods: In a blinded study design, we investigated with MRI, including standard spin-echo sequences, 18 patients evaluated with clinical examination and computed tomography (CT) suspected for relapsing/residual cholesteatoma 7 to 19 months after a canal wall-up mastoidectomy. Images were evaluated by two radiologists blinded to patients’ identities, CT findings, and clinical data set, who decided in a consensus agreement whether there was a pathologic signal increase in the petrous bone in a single-shot EPI-DWI sequence. All the patients underwent a second tympanoplasty or revision surgery of the mastoidectomy cavity within 15 days after magnetic resonance investigation. Sensitivity, specificity, and predictive values were evaluated separately for standard sequences and EPI-DWI. Results: In EPI-DWI, five of six patients with cholesteatoma showed a bright signal, whereas those patients with a noncholesteatomatous tissue showed no anomalies. The only misdiagnosed cholesteatoma was a pearl 2 mm in diameter. Sensitivity, specificity, and positive predictive values, and negative predictive values of EPI-DWI in diagnosing relapsing/residual cholesteatomas were 86, 100, 100, and 92%, respectively. Conclusion: EPI-DWI may be a useful tool in differentiating between cholesteatomatous and noncholesteatomatous tissues after closed cavity mastoidectomy. Further investigations are, however, required to establish the practical utility of EPI-DWI on larger series as a screening modality in the follow-up after closed cavity mastoidectomies.

Modified translabyrinthine approach and hearing preservation.

Objectives/Hypothesis: In 1991, the translabyrinthine approach was modified by sealing the vestibule with bone wax, which allowed preservation of the hearing function in one patient. The present study aimed specifically at evaluating the effectiveness of the modified translabyrinthine approach in preserving hearing function in a group of patients with vestibular schwannoma that involved the internal auditory canal.

Malleostapedotomy in tympanosclerosis patients.

OBJECTIVES To present our personal experience of a series of 10 patients suffering from tympanosclerosis with functional blocking of the stapes or footplate, who underwent malleostapedotomy surgery. The criteria for patient selection for this type of operation, and its results and complications, are discussed. METHODS Prospective study. RESULTS Incus and malleus dysfunction was observed in 70 per cent of cases, either alone or combined with fixation of the stapes. The post-operative hearing results were considered to be satisfactory (i.e. within 20 dB) in 80 per cent of cases. Only one patient had sensorineural hearing loss over 10 dB. CONCLUSIONS Malleostapedotomy has proved its practicability in the treatment of patients with fixed footplate or stapes complicated by ankylosis of the incudomalleolar joint. This procedure can be considered a further, valid technique within the otologists surgical armamentarium.

Partial labyrinthectomy in the treatment of labyrinthine fistula: how we do it

Dear Editor, A labyrinthine fistula due to cholesteatoma represents an alarming complication for the otologist. The surgeon has to balance the aims of removing cholesteatoma and not causing sensorineural hearing loss. Traditionally the choice has been either to conserve or remove the cholesteatoma matrix. Some surgeons have sought to minimise the risk of sensorineural hearing loss by avoiding working on the delicate structures of the membranous labyrinth. On the other hand, leaving residual cholesteatoma may increase the risk of the infective ⁄ inflammatory process spreading from the cholesteatoma matrix to the membranous labyrinth, a factor that may increase the risk of sensorineural hearing loss. Comparison between the two techniques is difficult because of the relative rarity of the condition and because no universally recognised system exists for staging fistulas. Donhoffer and Milewski have suggested a grading system as follows: 1 In type I, there is erosion of the bony labyrinth but an intact endosteum. 2 Type II and III are true fistulae with opening into the perilymphatic space. 3 In type IIa the perilymphatic space is opened but undisturbed. 4 In type IIb the perilymphatic space is disturbed either by ingrowth of cholesteatoma or by active suctioning by the surgeon before recognition of the fistula. 5 In type III there is concomitant involvement or destruction of the underlying membranous labyrinth. Following a careful analysis of the literature on the subject, Copeland and Buchman recommended conservative surgical treatment of the fistula, leaving the matrix in situ and using an open technique. A further treatment possibility that was proposed recently is the immediate removal of the matrix combined with removal of the affected semicircular canal, or ‘partial labyrinthectomy’. While this may seem radical, rationale exists in the principle of eliminating all of the infective and inflammatory processes that could in the future compromise hearing function. Van den Abeele and Offeciers and Kobayashi et al. proposed this alternative strategy that draws from the experiences of Parnes and McClure for the treatment of benign paroxysmal vertigo (occlusion of the posterior semicircular canal) and from those of numerous other authors for the treatment of skull-base lesions while preserving hearing function. This partial labyrinthectomy consists of removing the semicircular canal affected by the fistula together with the cholesteatoma matrix and sealing it immediately afterward. We describe our experience with a group of patients treated using this technique.

Vestibular Evoked Periocular Potentials in Meniere's Disease after Glycerol Testing

Objectives: The present investigation was specifically designed to evaluate the clinical application of vestibular evoked periocular potentials (VEPPs) in the diagnosis of endolymphatic hydrops. Methods: We compared the results of the traditional pure tone audiometry glycerol test with those of the vestibular evoked myogenic potential (VEMP) glycerol test and the VEPP glycerol test in 22 patients affected by unilateral endolymphatic hydrops. Results: Some patients had positive depletive tests with both VEMPs and VEPPs, and other patients had positive tests with either VEMPs or VEPPs. Conclusions: Our outcomes confirmed that vestibular evoked potentials represent a useful additional diagnostic tool in the diagnosis of endolymphatic hydrops. The role of VEPPs in this particular issue was not inferior to that of VEMPs. The outcomes also suggested that not only the saccule, but also the utriculus, may be involved in the genesis of VEPPs.

Acute bilateral facial palsy and Wegener's disease

Wegener’s granulomatosis (WG) is a granulomatous, necrotizing vasculitis with a predilection for the upper respiratory airways, lungs, and kidneys. We describe the case of a patient who experienced acute bilateral facial palsy as the first manifestation of WG and whose diagnosis was achieved by means of a lung biopsy. A 31-year-old man came to our clinic complaining of bilateral aural fullness. Otoscopy revealed a dull tympanic membrane on both sides. Pure-tone audiometry showed a slight bilateral conductive hearing loss. A therapy with antibiotics and corticosteroids did not achieve complete relief. The patient began to feel an intense pain in the left ear 2 weeks later; otoscopy showed a red tympanic membrane. Four days later he developed a sudden left facial palsy (grade VI, House-Brackmann classification); fluid discharge from the ear was noted. A computed tomography (CT) of the temporal bone was performed (Fig 1). A magnetic resonance imaging scan showed soft tissue with a low-intensity signal predominantly on the left side. An urgent facial nerve decompression was performed. Granulation tissue and hypertrophic mucosa completely filled the middle ear and mastoid air cell system. The facial nerve was exposed in all its segments, and it was not directly involved by the granulation tissue. Multiple biopsy specimens showed nonspecific inflammatory granulation tissue. Hematologic examinations, urinalysis, chest x ray, and c-ANCA and p-ANCA results were normal; only ESR was elevated. The patient was discharged with antibiotic and corticosteroid therapy, and improvement of the facial function was observed during the next five days (grade III). Consequently, the therapy was stopped. Three days later, the patient experienced complete facial palsy and a severe conductive hearing loss on the right side and was readmitted to our department. After one day of intravenous corticosteroids, the patient started suffering from respiratory symptoms. Further chest x-ray showed diffuse pulmonary nodules. A thoracic CT scan confirmed the presence of multiple nodules. Transbronchial biopsies of the lesions were consistent with a diagnosis of WG. Subsequently, an elevated ESR and a positive c-ANCA titer confirmed the diagnosis; urinalysis excluded any renal involvement.

Intradural jugular paragangliomas: complications and sequelae.

OBJECTIVES To present the long-term results of a group of patients who underwent surgery for intradural jugular paragangliomas. We discuss the complications, sequelae, and evolution of recurrences and behavior of residual tumors. METHODS From 1989 to 2002, 11 patients with intradural jugular paragangliomas underwent surgery using different approaches. The paragangliomas were grouped according to the classification of Fisch. RESULTS Total removal of the paraganglioma was possible in eight patients, while a subtotal resection was achieved in the other three cases. The tumor remnant remained stable in two patients but continued to grow in one. Recurrence was observed in one patient. There were preoperative deficits in cranial nerves IX to XI in four patients and of XII in two patients. Deficits of cranial nerves IX to XI were acquired as a result of surgery in three cases and of XII in another. Six patients had a pure-tone average of 45 to 75 dB while the others had dead ears. Persistent dysphagia and dysphonia were managed in two patients by injection of the paralyzed cord with fat and subsequent medialization of the vocal cord by thyroplasty. Two patients had a cerebrospinal fluid leak. CONCLUSIONS Surgical results in patients with extensive jugular paragangliomas are consistent and offer an acceptable quality of life even in advanced cases.

Susac syndrome and vestibular-evoked myogenic potentials.

Susac syndrome is a rare disease with fewer than 100 reported cases. It is characterized by encephalopathy, retinal arteriolar branch occlusions, and deafness. The hearing loss is often associated with vertigo or dizziness and caloric testing has demonstrated the presence of vestibular disturbances. This report describes a patient with Susac syndrome whose first symptom was vestibular dysfunction. Although the patient underwent complete audiovestibular testing, only the vestibular-evoked myogenic potentials (VEMPs), measured with an Amplaid MK-12, gave abnormal results. A 24-year-old woman presented in October 2006 with acute onset dizziness. Otoscopy was normal. Audiometry, impedance tests, and auditory brain stem responses were within normal limits. The Hallpike-Fitzgerald test was normal. She had a left-sided VEMP deficit, with delayed latency and reduced amplitude (Fig 1). Magnetic resonance imaging (MRI) showed multiple foci of high T2 signal intensity in both the white and gray matter and leptomeningeal enhancement (Fig 2A and B). A few days after the MRI, the patient complained of hearing loss and neurologic deterioration that consisted of disorientation and incoherent speech. Audiometry revealed a left sensorineural hearing loss that was more pronounced at high frequencies. An initial presumptive diagnosis of viral encephalitis was made. The patient was hospitalized and antiviral therapy was administered. Nevertheless, visual dysfunction developed. Retinal fluorescein angiography disclosed retinal arteriolar branch occlusions in the left eye. Susac syndrome was diagnosed. The antiviral treatment was stopped and prednisone was given. The neurologic deterioration resolved within 10 days. She was discharged with a recommendation that she continue the corticosteroid treatment. She regained a satisfactory quality of life with no particular limitations. MRI done at the last follow-up showed that the hyperintense lesions had disappeared. This case report was approved by Institutional Review Board of University La Sapienza of Rome, Italy.

Vestibular Evoked Myogenic and Periocular Potentials after Vestibular Schwannoma Surgery

Objectives: We analyzed the behavior of preoperative and postoperative vestibular evoked myogenic potentials (VEMPs) and vestibular evoked periocular potentials (VEPPs) in 3 patients who had vestibular schwannoma and underwent modified translabyrinthine surgery. Methods: We compared VEMPs and VEPPs, measured with both air-conducted (AC) and bone-conducted (BC) stimulations. Vestibular evoked potentials were measured both in the immediate postoperative period and some months later. Results: At the immediate postoperative examination, VEPPs were preserved in all 3 patients with both AC and BC stimulations. The VEMPs showed stable or improved parameters in 2 of our patients and were absent in the third patient. At the follow-up examination, VEMPs did not show any significant change with respect to the previous evaluation. In contrast, VEPPs were absent in 2 of our patients with AC stimulation and in 1 patient with BC stimulation. Conclusions: Our results seem to indicate a greater stability and reproducibility of VEMPs compared with VEPPs. In 1 patient, who had a schwannoma of the inferior vestibular nerve, the preservation of VEPPs and the absence of VEMPs in the immediate postoperative period confirm that the saccule represents the origin of VEMPs. Furthermore, these results suggest that not only the saccule, but also the utriculus, could be implicated in the origin of VEPPs.

Selective damage of the vestibular apparatus following toxic effects of streptomycin

Awide range of drugs cause functional damage to or cellular degeneration of the inner ear tissues, particularly at the receptors and neural cells of the VIII cranial nerve. Moreover, the toxic effects of gentamicin and streptomycin on the vestibular apparatus have found clinical application in suppressing the labyrinth in Ménière’s disease refractory to conservative medical treatment. The selectivity of damage to the inner ear structures explains the prevalent audiological or vestibular symptoms of ototoxicity. Some drugs are specifically or predominantly cochleotoxic. This may be evaluated directly by using objective tests, such as auditory brain stem response (ABR) or otoacoustic emissions, or subjective hearing tests. Few studies have evaluated vestibulotoxicity, and these reports were based on histological findings, indirect methods (eg, study of vestibular reflexes), or the direct study of vestibular end-organ dysfunction [vestibular-evoked potentials (VsEPs)]. Theoretically, damage to the inner ear structures caused by ototoxic substances can be selective and limited to a restricted portion of the vestibular apparatus, but this theory has not been validated with any clear, direct clinical evidence. This report describes the case of a patient with bronchopneumonia who was treated with intramuscular streptomycin and underwent post-therapy audiovestibular examination using pretone audiometry, vestibular-evoked myogenic potentials (VEMPs), and caloric tests, demonstrating the presence of selective damage to the vestibular apparatus. A 50-year-old woman presented with bilateral hearing loss and dizziness. Two months earlier, she was given intramuscular streptomycin (1 g daily) for 24 days to treat a lung disease. The therapy was discontinued when the first audiological symptoms appeared. Audiometry revealed bilateral sensorineural hearing loss at high frequencies (right ear: 35 dB at 1000 Hz, 60 dB at 2000 Hz, 75 dB at 4000 Hz, 95 dB at 8000 Hz; left ear: 40 dB at 1000 Hz, 60 dB at 2000 Hz, 85 dB at 4000 Hz, no response at 8000 Hz). The Fitzgerald-Hallpike test (alternating binaural bithermal test) indicated bilateral areflexia. VEMPs were mea-