Superior Canal Dehiscence Involving the Superior Petrosal Sinus: A Novel Classification Scheme.

OBJECTIVES: To highlight superior semicircular canal dehiscence (SSCD) involving the superior petrosal sinus (SPS), and to propose a novel classification system for SPS associated SSCD with potential surgical implications. STUDY DESIGN: Multicenter retrospective review. SETTING: Three tertiary referral centers. PATIENTS: All patients diagnosed with SPS associated SSCD (1/2000 to 8/2016). Radiographic findings and clinical symptoms were analyzed. INTERVENTION: Surgical repair or observation. MAIN OUTCOME MEASURE: Radiographic findings and clinical symptoms were analyzed. RESULTS: Thirty-three dehiscences (30 patients) involving the SPS were identified. The average age at the time of presentation was 52.5 years (median, 56.9; range, 4.9-75.3 yr), and 53.3% of patients were men. Three patients had bilateral SPS associated SSCD. The most common associated symptoms at presentation were episodic vertigo (63.6%), subjective hearing loss (60.6%), and aural fullness (57.6%). Four distinct types of dehiscence were identified: class Ia. SSCD involving a single dehiscence into an otherwise normal appearing SPS; class Ib. SSCD involving a single dehiscence into an apparent venous anomaly of the SPS; class IIa. SSCD involving two distinct dehiscences into the middle cranial fossa and the SPS; class IIb. SSCD involving a single confluent dehiscence into the middle cranial fossa and the SPS. CONCLUSIONS: SSCD involving the SPS represents a small but distinct subset of SSCD cases. This scenario can create a unique set of symptoms and surgical challenges when intervention is sought. Clinical findings and considerations for surgical intervention are provided to facilitate effective diagnosis and management.

Clinical features of otolith organ-specific vestibular dysfunction.

OBJECTIVE: To elucidate the clinical features and vestibular symptoms of patients with otolith organ dysfunction in the presence of normal function of the semicircular canals. METHODS: We reviewed the clinical records of 277 consecutive new patients with balance disorders who underwent testing of cervical and ocular vestibular evoked myogenic potentials (cVEMPs and oVEMPs) as well as caloric testing and video head impulse testing (vHIT). RESULTS: We identified 76 patients who showed normal caloric responses and normal vHIT findings in each SCC plane, but abnormal responses in cVEMP and/or oVEMP testing. Benign paroxysmal positional vertigo (BPPV) was the most common diagnosis. 37% of patients could not be categorized into any of the established clinical entities that could cause a balance disorder and did not show sensorineural hearing loss. The most common clinical manifestation in the idiopathic cases was recurrent rotatory vertigo with a duration of 1-12 h. CONCLUSIONS: The most common diagnosis of otolith organ-specific vestibular dysfunction was BPPV. The most common clinical manifestation in the idiopathic cases was recurrent rotatory vertigo. SIGNIFICANCE: Specific dysfunction of the otolith organs occurs in association with some of the undiagnosed patients with recurrent rotatory vertigo.

Radiologic classification of superior canal dehiscence: implications for surgical repair.

OBJECTIVE: Surgical access to repair a superior canal dehiscence (SCD) is influenced by the location of the bony defect and its relationship to surrounding tegmen topography as seen on computed tomography. There are currently no agreed-upon methods of characterizing these radiologic findings. We propose a formal radiologic classification system of SCD based on dehiscence location and adjacent tegmen topography. STUDY DESIGN: Retrospective case review SETTING: Tertiary, neurotology referral center PATIENTS: We identified 298 patients with superior canal dehiscence on CT from February 2001 to October 2013. Of these, 251 had symptomatic superior canal dehiscence syndrome and were included in the study. INTERVENTION: Patients underwent high-resolution temporal bone CT scans with creation of axial, coronal, Pöschl, and Stenver reformatted images to examine the superior semicircular canal. Two residents-in-training and a head and neck radiologist independently read the scans. MAIN OUTCOME MEASURES: CT scans were assessed for (1) superior canal dehiscence or "near" dehiscence, (2) defect location relative to the skull base, (3) surrounding tegmen defects, (4) geniculate ganglion dehiscence, (5) superior petrosal sinus-associated dehiscence (SPS), (6) low-lying tegmen, and (7) the distance between the outer table of the temporal bone and the arcuate eminence.

Intralabyrinthine schwannomas: imaging diagnosis and classification.

BACKGROUND AND PURPOSE: ILS is a rare lesion that has a different management from the more common "acoustic" schwannoma. To date, only 137 cases have been reported. We present a classification scheme based on labyrinthine anatomy to describe and localize these lesions. Treatment and prognosis hinge on the appropriate localization of these tumors; thus, a concise terminology that can be used by both the otolaryngologist and radiology communities is desirable. MATERIALS AND METHODS: After approval of the institutional review board, a retrospective study of all patients with the diagnosis of ILS imaged between 1996 and 2010 was performed. Clinical and imaging data were collected. Patients were imaged with thin-section high-resolution T2 and contrast-enhanced MR imaging. RESULTS: There were 45 patients with a diagnosis of ILS. Forty-three had complete histories. There were 18 male and 25 female patients with an age range of 21-78 years with a mean age of 53 years. The most common presenting symptom was progressive sensorineural hearing loss. Lesions were characterized on the basis of their location. Intracochlear was most common (14/45) followed by transmodiolar (13/45), intravestibular (7/45), vestibulocochlear (5/45), transmacular (4/45), and transotic (2/45). Sixteen patients underwent surgical resection. The remaining patients were followed clinically and by serial MR imaging. CONCLUSIONS: ILS is an uncommon but under-reported tumor. We characterized the MR imaging appearance of these tumors by using high-resolution techniques. In addition, an anatomically based classification system is presented that will help the radiologist accurately describe ILS within the inner ear and help the surgeon determine which tumors are potential surgical candidates.

Posterior petrous meningiomas: 82 cases.

OBJECT: The aim of this study was to discuss posterior petrous meningiomas--their classification, clinical manifestations, surgical treatments, and patient outcomes. METHODS: A retrospective analysis was performed in 82 patients with posterior petrous meningiomas for microsurgery. According to the anatomical relationship with the posterior surface of the petrous bone and with special reference to the internal auditory canal (IAC), posterior petrous meningiomas were classified into three types: Type I, located laterally to the IAC (28 cases); Type II, located medially to the IAC, which might extend to the cavernous sinus and clivus (32 cases); and Type III, extensively attached to the posterior surface of the petrous bone, which might envelop the seventh and eighth cranial nerves (22 cases). Sixty-eight (83%) of 82 cases involved total resection. The rate of anatomical preservation of facial nerve was 97.5%, whereas the functional preservation rate was 81%. The rate of hearing preservation was 67%. All Type I tumors were completely resected, and the rate of anatomical preservation of facial nerve was 100% and functional preservation was 93%. Regarding Type II lesions, 75% of 32 cases involved total resection; the rate of anatomical preservation of facial nerve was 97% and functional preservation was 75%. For Type III lesions, 73% of 22 cases were totally resected. The rate of anatomical preservation of facial nerve in patients with this tumor type was 95%, whereas functional preservation was 73%. CONCLUSIONS: Clinical manifestations and surgical prognoses are different among the various types of posterior petrous meningiomas. It is more difficult for Types II and III tumors to be resected radically than Type I lesions, and postoperative functional outcomes are significantly worse accordingly. The primary principles in dealing with this disease entity include preservation of vital vascular and central nervous system structures and total resection of the tumor as much as possible.

"Spontaneous" perilymph fistula: a case report.

A surgically documented case of barotrauma-induced perilymph fistula is presented in this case report. A brief review of the literature on this entity confirms the difficulty of making a definitive preoperative diagnosis in most instances. Clinical, audiometric, radiologic, and intraoperative findings are presented, and the classic presumed mechanisms for this uncommon cause of sudden sensorineural hearing loss are discussed. The presence of intact evoked otoacoustic emissions in an ear demonstrating a severe cochlear-type loss was considered helpful in narrowing the differential diagnosis in this case, and may suggest a productive avenue for future study.

Intralabyrinthine schwannomas: diagnosis, management, and a new classification system.

OBJECTIVE: To outline the diagnosis and management of intralabyrinthine schwannomas and to propose a new classification system to further define them. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center at a university hospital. PATIENTS: Twenty-eight patients seen at the institution with intralabyrinthine schwannomas between 1996 and 2002 were included in the study. INTERVENTIONS: Diagnosis was made with magnetic resonance imaging in all but one case. Our ability to detect these tumors has been greatly enhanced by the introduction of high-resolution T2-weighted magnetic resonance imaging. Treatment options were observation with serial magnetic resonance imaging versus complete surgical removal via a translabyrinthine or transotic approach. Surgery was indicated for dizziness caused by the tumor or extralabyrinthine growth. MAIN OUTCOME MEASURES: Clinical features, audiology, radiology, and management outcomes were evaluated. RESULTS: Eight patients have undergone successful surgery with removal of their tumors and resolution of symptoms. One patient chose to have stereotactic radiotherapy. Of the 20 patients who were managed with observation and serial magnetic resonance imaging, only 1 has shown significant growth requiring surgical removal. CONCLUSION: Intralabyrinthine schwannomas are uncommon tumors that mimic the clinical features of many other neurotologic conditions. A high index of suspicion and precise imaging are often required to detect these tumors. Surgical treatment is indicated for specific indications and will be needed in the minority of patients with this disorder. The classification system that we propose is helpful in both the diagnosis and the management of these tumors.

On classification capability of neural networks: a case study with otoneurological data.

We investigated the capability of multilayer perceptron neural networks and Kohonen neural networks to recognize difficult otoneurological diseases from each other. We found that they are efficient methods, but the distribution of a learning set should be rather uniform. Also it is important that the number of learning cases is sufficient. If the two mentioned conditions are satisfied, these neural networks are similarly efficient as some other machine learning methods. The conditions are known in the theory of neural networks [1,2], but not often taken seriously in practice. Both networks functioned as well, excluding the case with several input variables, where the Kohonen neural networks surpassed the perceptron.

[Possibilities for preserving hearing in labyrinth fistulas of different degrees of severity]. / Möglichkeiten des Hörerhalts bei Labyrinth-Fisteln von unterschiedlichem Schweregrad.

The labyrinthine fistula continues to be one of the most common complications in ears with cholesteatoma. Fifty-one patients with labyrinthine fistula were identified in a series of 1243 cases with cholesteatoma operated upon between 1989 and 1993 at the University ENT Clinic Wuerzburg. The surgical management concept comprised of removing the cholesteatoma matrix, categorizing the fistula type, and immediate covering of the labyrinthine capsule defect with bone dust, perichondrium and fibrin glue. The classification system of the fistulae used in the study used the depth of labyrinthine involvement as a criterion and also took into account the possibility of intentional or accidental damage to the labyrinth during surgical manipulation (Fig. 1). From 1991 on, patients were treated with 500 mg of Presnisolon 21 hydrogen succinate in a single intravenous dose, at the time the fistula was corrected. The postoperative hearing results were graded based on the extend of preservation of inner ear function at or near the preoperative level. None of the patients who had corticosteroid therapy suffered a profound sensory neural hearing loss (Fig. 2, 3), whereas five ears without steroids and a deep fistula lost function completely. The study concluded that corticosteroids have a beneficial impact on postoperative outcome in cases with severe injury to the membranous labyrinth.

Vertigem na criança / Vertigo in childhood

É feita uma breve revisäo sobre vertigem na infância. Os autores apresentam uma classificaçäo e däo especial atençäo para a vertigem paroxística benigna

Idiopathic bilateral vestibulopathy.

We report the clinical features of 22 patients with acquired bilateral vestibulopathy of unknown cause. All had either absent or markedly decreased responses to both caloric and rotational testing. They presented with dysequilibrium and imbalance, worse at night; most reported oscillopsia but none had associated hearing loss or other neurologic symptoms. Nine reported prior prolonged episodes of vertigo consistent with the diagnosis of bilateral sequential vestibular neuritis. Of the remaining 13, none had exposure to known ototoxins or a positive family history. Idiopathic bilateral vestibulopathy is an important cause of progressive imbalance in adults and should be considered even though hearing is normal.

Endolymphatic hydrops. An overview and classification.

Endolymphatic hydrops (EH) is a pathological condition which is the final common manifestation of a variety of otologic insults. In this paper we develop a classification which, on the basis of clinical and pathological data, distinguishes symptomatic and asymptomatic forms. Clinical case histories and temporal bone studies are presented to illustrate and substantiate this classification. The symptomatic form becomes evident by the hallmark symptoms of episodic vertigo and fluctuating hearing loss. The asymptomatic form is silent. Interconversion from one form to another may occur over time. Each of the forms can be subdivided, according to etiology, into embryopathic, acquired, and idiopathic types. The embryopathic type comprises those cases in which some noxious influence interferes with prenatal labyrinthine development. The acquired type includes those cases in which a documented insult, be it inflammatory or traumatic, is suffered by a previously normal labyrinth. The inflammation may be viral, bacterial, or spirochetal in nature, while the traumatic event may be either accidentally or surgically sustained. The idiopathic type includes cases in which the instigating event precipitating the EH is unknown. Menière's disease represents merely one example of the EH group of disorders, namely symptomatic idiopathic EH.

Classification of endolymphatic hydrops.

Ménière's disease is but one member of a family of disorders linked by the common pathophysiologic substrate of endolymphatic hydrops. The authors evaluated both clinical cases and temporal bone specimens and conclude that endolymphatic hydrops is a pathologic condition that is the final common manifestation of a variety of otologic insults. They present a logical classification which, on the basis of clinical data, distinguishes symptomatic and asymptomatic forms. The hallmarks of the symptomatic form are fluctuating hearing loss and episodic vertigo. The asymptomatic form is clinically silent. Each form may be further subclassified into embryopathic, acquired, and idiopathic types. The embryopathic type comprises those cases in which a noxious influence disrupts labyrinthine development in utero. The acquired type includes those cases in which a documented insult, either inflammatory or traumatic, is suffered by a previously normal labyrinth. The idiopathic type encompasses all those cases in which the event precipitating the endolymphatic hydrops is unknown. Ménière's disease is redefined as idiopathic, symptomatic endolymphatic hydrops.

Perilymph fistulas.

A perilymph fistula is an abnormal communication between the fluids surrounding the membranous labyrinth and the middle ear space. Because of the potential hazards of meningitis, permanent hearing loss, and occasionally incapacitating vestibular symptoms, early recognition and prompt repair of the perilymph leak is important. One hundred thirty three cases of perilymph fistulas are presented, stressing the clinical characteristics, evaluation, and management of patients with this otologic entity. The historical and contemporary literature on this subject is reviewed. A pathophysiological basis for perilymph fistula formation is presented, based upon certain anatomic, physiologic and mechanical principles involving the temporal bone and surrounding structures. For the purpose of publication, the Materials and Methods and Evaluation sections of the paper have been omitted. These are available from the author upon request in mimeographed form. The interested reader may wish to refer to items 80 and 133 in the Bibliography, previous publications by the author in which the bulk of the data used in the omitted sections can be found.

Congenital anomalies of the inner ear: introducing a new classification of labyrinthine anomalies.

In a literature review of 108 articles a special effort was made to find and classify inner ear anomalies and diseases associated with inner ear anomalies. This study showed the following. Most of the diseases associated with inner ear anomalies are also associated with anomalies in other parts of the body. Hereditary characteristics comprise the most common etiological factor among the diseases associated with inner ear anomalies. Among anomalies observed in the cochlea, the vestibule, and the semicircular canals, anomalies in the cochlea are most frequently associated with various diseases and were observed in 30 of 40 diseases. Anomalies of the vestibule were observed in 25 diseases, and those of the semicircular canals in 18 diseases. Anomalies in both the osseous and the membranous labyrinth were most frequently associated with the diseases studied, as they were observed to occur with 10 of the 43 diseases. In this paper a new classification system for labyrinthine anomalies is introduced, based on this study of the literature.

[Medico-legal assessment of vestibular disturbances (author's transl)]. / Die Begutachtung vestibulärer Störungen Eine Orientierungshilfe zur Bemessung der MdE.

In the medico-legal assessment of vestibular disturbances the degree of handicap in daily and occupational situations is to be considered. Therefore a five degree classification of everyday performances (physiological stimuli of daily life) involving the vestibular system and a grading of vestibular disorders based on subjective and objective symptoms have been compiled. A diagram relating intensity of vestibular disturbance with performance gives the percentage of reduction in working capacity (MdE).