Search

Treball Final de Grau en Medicina. Codi: MD1158. Curs acadèmic: 2020/2021 El vértigo es un síntoma que se percibe como una ilusión de movimiento generalmente rotacional. Este síntoma, cada vez más prevalente a medida que aumenta la edad de los pacientes, es común a muchas patologías otoneurológicas. Por esta razón, el diagnóstico diferencial de todas las entidades nosológicas que comprenden al vértigo periférico compone la base diagnóstico-terapéutica de la patología vertiginosa. La complejidad de la fisiopatología en Otoneurología evoca un reto en la evidencia de los tratamientos para cada patología que se incluye el presente protocolo asistencial. El objetivo de este trabajo consiste en diseñar un protocolo con el fin de estandarizar la toma de decisiones en el manejo terapéutico de los pacientes que cursen con vértigo periférico en el Departamento de Salud del hospital de La Fe. En cuanto a la metodología, se realizó una revisión integradora de la literatura científica. Los artículos fueron seleccionados de diversas bases de datos Medline/PubMed y Cochrane, recursos de síntesis y metabuscadores, analizando su calidad con las herramientas CASPe y AGREE, a su vez, han sido adaptados a las características del área y departamento de salud concreto. Así pues, se ha elaborado un protocolo para el tratamiento del vértigo periférico del adulto que recoge el amplio abanico de opciones terapéuticas que deben contemplarse según la etiología de este, y también su versión reducida, que favorece su uso en la práctica clínica diaria. Vertigo is a symptom experienced as an illusion of movement, usually rotational. This symptom, which becomes more and more prevalent as patients get older, is common to many otoneurologic diseases. Therefore, the differential diagnosis of all the nosological entities that include peripheral vertigo forms the diagnostic-therapeutic basis of vertiginous pathology. The complexity of the physiopathology in Otoneurology evokes a challenge in the evidence of the treatments for each pathology included in this care protocol. The aim of this work is to design a protocol to standardise decision-making in the therapeutic management of patients with peripheral vertigo in the health department of La Fe hospital. In terms of methodology, an integrative review of the scientific literature was carried out. The articles were selected from multiple Medline/PubMed and Cochrane databases, synthesis resources and meta-search engines, analysing their quality with the CASPe and AGREE tools, and adapted to the characteristics of the specific area and health department. As a result, a protocol for the treatment of peripheral vertigo in adults has been developed, which includes a wide range of therapeutic options that should be considered depending on the aetiology of the condition, as well as a reduced version that facilitates its use in daily clinical practice.

Objective: To describe a case of benign paroxysmal positional vertigo (BPPV) resulting in reversible horizontal semicircular canalith jam successfully treated with horizontal canal occlusion. A brief literature review of similar cases was performed. Methods: Case report and literature review. Results: A 68-year-old female presented with apogeotropic positional nystagmus, attributed to reversible horizontal canalith jam mimicking cupulolithiasis that was refractory to tailored repositioning maneuvers across months. She was unable to work due to the severity of her symptoms. She underwent surgical occlusion of the affected canal with immediate resolution of her symptoms. A literature review revealed similar cases of canalith jam mimicking cupulolithiasis. Conclusions: Reversible canalith jam, in which particles moving with horizontal head position alternate between obstructing the semicircular canal and resting on the cupula, can mimic signs of cupulolithiasis. This variant of BPPV can be effectively managed with surgical canal occlusion should symptoms fail to resolve after tailored repositioning maneuvers.

We describe a case series of labyrinthine fistula, characterized by Hennebert's sign (HS) elicited by tragal compression despite global hypofunction of semicircular canals (SCs) on a video-head impulse test (vHIT), and review the relevant literature. All three patients presented with different amounts of cochleo-vestibular loss, consistent with labyrinthitis likely induced by labyrinthine fistula due to different temporal bone pathologies (squamous cell carcinoma involving the external auditory canal in one case and middle ear cholesteatoma in two cases). Despite global hypofunction on vHIT proving impaired function for each SC for high accelerations, all patients developed pressure-induced nystagmus, presumably through spared and/or recovered activity for low-velocity canal afferents. In particular, two patients with isolated horizontal SC fistula developed HS with ipsilesional horizontal nystagmus due to resulting excitatory ampullopetal endolymphatic flows within horizontal canals. Conversely, the last patient with bony erosion involving all SCs developed mainly torsional nystagmus directed contralaterally due to additional inhibitory ampullopetal flows within vertical canals. Moreover, despite impaired measurements on vHIT, we found simultaneous direction-changing positional nystagmus likely due to a buoyancy mechanism within the affected horizontal canal in a case and benign paroxysmal positional vertigo involving the dehiscent posterior canal in another case. Based on our findings, we might suggest a functional dissociation between high (impaired) and low (spared/recovered) accelerations for SCs. Therefore, it could be hypothesized that HS in labyrinthine fistula might be due to the activation of regular ampullary fibers encoding low-velocity inputs, as pressure-induced nystagmus is perfectly aligned with the planes of dehiscent SCs in accordance with Ewald's laws, despite global vestibular impairment on vHIT. Moreover, we showed how pressure-induced nystagmus could present in a rare case of labyrinthine fistulas involving all canals simultaneously. Nevertheless, definite conclusions on the genesis of pressure-induced nystagmus in our patients are prevented due to the lack of objective measurements of both low-acceleration canal responses and otolith function.

An 84-year-old man presented to the emergency department for acute vomiting associated with rotational vertigo and a sudden right sensorineural hearing loss. A left peripheral vestibular nystagmus was highlighted. The patient was afebrile, without respiratory signs or symptoms. Blood sampling at admission showed lymphopenia, thrombopenia and neutrophil polynucleosis, without elevation of C reactive protein. Cerebral magnetic resonance imaging eliminated a neurovascular origin. Vestibule, right semicircular canals and cochlear FLAIR hypersignals were highlighted, leading to the diagnosis of right labyrinthitis. A nasopharyngeal swab sampled at admission returned positive for SARS CoV2 by polymerase chain reaction. The etiologic investigation, including syphilitic and viral research, was otherwise negative. An oral corticotherapy (prednisone 70 mg daily) was introduced, followed by a progressive clinical recovery. Although acute otitis media have already been highlighted as an unusual presentation of COVID-19, radiology-proven labyrinthitis had to our knowledge, never been described to date.

We report a case of a 67-year-old female patient that presented to the emergency department with complete right facial paralysis, progressive hearing loss and chronic otorrhea. A unique finding on CT scan is reported: a “floating labyrinth” – cochlea, vestibule and semicircular canals extensively surrounded by soft tissue density material but with intact thinned otic capsule walls. A transotic approach was performed for removal of noncholesteatomatous inflammatory tissue; intravenous antibiotics and corticosteroids led to partial recovery of facial nerve function. A chronic suppurative otitis media with necrotic osteomyelitis and bony sequestrum in a severe context of AIDS is the likely cause. An immunosuppressive disease should be suspected in atypical presentations of chronic suppurative otitis media without cholesteatoma. publishersversion published

Superior semicircular canal dehiscence (SSCD) is known as abnormal communication of the superior semicircular canal (SCC) to the intracranial space secondary to a bony defect in the canal. Patients who are subjected to surgical repair usually have intractable symptoms, and recently, plugging of SCC using a transmastoid approach has been widely recommended. In this report, we describe a case of incomplete plugging for SSCD in a 37-year-old woman, along with the high-resolution three dimensional magnetic resonance imaging (3D MRI) findings using Pöschl view reconstruction. Postoperative MRI of 3D T2-wieghted sampling perfection with application optimized contrasts using different flip angle evolution (SPACE) Pöschl plane demonstrated an incomplete plugging of the SCC with partially visible perilymphatic fluid in the posterior limb above the common crus. A 3D fluid-attenuated inversion recovery (FLAIR) sequence showed an enhancement involving the vestibule and SCC, suggesting labyrinthitis. Although there are few reports about incomplete plugging for SSCD, this case could demonstrate postoperative status and complication after plugging of SSCD using a high-resolution 3D MRI sequences with Pöschl view reconstruction.

Labyrinthitis most commonly results from an infectious and less commonly from an inflammatory process of the inner ear, but it can be associated with temporal bone trauma, hemorrhage, or tumor. This inflammation (regardless of the etiology) disrupts the transmission of sensory information from the ear to the brain. Labyrinthitis ossificans is the pathological ossification of the membranous labyrinthine spaces in response to an insult to the inner ear involving membranous labyrinth or the endosteum of the otic capsule. Herein, we present a case of a 67-year-old female with progressive bilateral sensorineural hearing loss and positional vertigo.

Objective To describe a rare case of pneumolabyrinth (PNL) and pneumocephalus (PNC) due to otogenic meningitis in a patient with superior canal dehiscence (SCD) resulting in profound sensorineural hearing loss (SNHL), semicircular canals impairment but preservation of SCD-related enhanced otolith function. Patient A 65-year-old woman with otogenic meningitis. Intervention Temporal bone high-resolution computed tomography (CT) scans, brain-magnetic resonance imaging, audiometry, bedside examination, video-head impulse test, and vestibular-evoked myogenic potentials (VEMPs). Main outcome measures Enhanced otolith function despite canal and cochlear loss. Results The patient developed right profound SNHL and acute labyrinthitis. Imaging showed middle ear inflammatory tissue, right PNL and PNC despite lack of bony fractures. Bilateral SCD and tegmen dehiscence were detected. The patient underwent mastoidectomy, drainage of effusion, and surgical repair of tegmen dehiscence. Exploratory tympanotomy was uneventful. She was treated with intravenous antibiotics and dexamethasone for 3 weeks with improvement of general condition. At 3-weeks follow-up, right profound SNHL persisted with global hypofunction for ipsilateral semicircular canals and selective impairment for left superior canal activity at video-head impulse test. Surprisingly, both cervical and ocular-VEMPs exhibited bilaterally abnormal amplitudes and reduced thresholds, consistently with preserved SCD-related macular hypersensitivity to sounds even on the affected side. Conclusions This case report exhibits a unique clinical scenario as it offers interesting insights concerning PNL aetiology despite lack of either bony fractures or barotrauma and PNC likely conveyed intracranially by SCD. Moreover, it provides an unusual pattern of functional dissociation among inner-ear receptors showing enhanced otolith function despite severe labyrinthine damage.

OBJECTIVE The aim of this study was to evaluate the feasibility and safety of transcanal underwater endoscopic bone resection (TUEBR) of the external auditory canal (EAC) for the management of cholesteatoma involving the antrum and mastoid. STUDY DESIGN Retrospective case review. SETTING Tertiary referral center. PATIENTS Pediatric and adult patients with primary cholesteatoma extending to the antrum and mastoid who underwent transcanal endoscopic ear surgery (TEES) with TUEBR between March 2016 and June 2017. INTERVENTION A rigid 2.7 mm diameter, 18 cm length Hopkins-rod telescope with an endoscopic sheath was inserted in the EAC and continuously perfused with saline during the dissection. TUEBR was performed to expose extensive cholesteatoma by using a high speed drill with curved burrs and a protected shaft. Next, removal of visible disease, reconstruction of the resected EAC, ossiculoplasty, and tympanoplasty were accomplished with TEES. RESULTS There were no intra- or postoperative severe complications such as facial palsy and inner ear injury except one patient suffering from secondary labyrinthitis. There was a negative linear relationship (r = -0.909) between the procedure time and procedure number of TUEBR. There was a weak relationship (r = 0.224) between the procedure time of TUEBR and the degree of the extension of cholesteatoma into the antrum and mastoid. There were two cases with residual cholesteatoma at 12 and 22 months follow-up postoperatively. CONCLUSION TUEBR is a safe and efficient technique for the resection of EAC bone and transcanal exposure of extensive cholesteatoma that would otherwise require mastoid dissection.

Labyrinthitis ossificans (LO) may occur following meningitis and, in cases where cochlear implantation is indicated, complicate electrode insertion. LO is critical to identify for successful cochlear implantation, and histopathology is more sensitive than imaging for identification of LO. Herein we utilize otopathologic techniques to study the timing and location of intracochlear tissue formation following meningitic labyrinthitis (ML).Retrospective review.Academic institution.Temporal bone specimens with a history of bacterial ML were histologically evaluated. The location and extent of intracochlear tissue formation within the scala tympani (ST) and scala vestibuli (SV) were graded, and spiral ganglion neurons were counted.Fifty-one temporal bones were identified: 32 with no intracochlear tissue formation, 9 with fibrosis alone, and 10 with LO. Fibrosis was identified as early as 1.5 weeks after ML, while ossification was found only in specimens that survived multiple years after ML. All LO cases showed ossification of the ST at the round window membrane (RWM) with continuous extension throughout the basal turn. Extent of SV ossification correlated with that in the ST but showed frequent isolated distal involvement of the cochlea. Spiral ganglion neuron counts were lower than those in age-matched controls.In this human temporal bone study, we found that postmeningitic LO results in ossification at the RWM with continuous extension into the ST of the basal turn and variable involvement of the SV. Identification of a patent basal turn beyond RWM ossification of the ST should permit full electrode insertion.Retrospective review.

Objective:The aim of this study was to determine the incidence of inner ear involvement in various forms of acute otitis media (AOM) in ear, nose, and throat (ENT) practices in Germany.Methods:Patients who had been diagnosed with various forms of AOM in the years 2010 to 2017 were enrolled in the study from a nationwide, representative practice database (Disease Analyzer, IQVIA). In these patients, the incidence of simultaneous or subsequent inner ear disorders (IED) was determined within 7 days and within 12 months from the date of an AOM diagnosis.Results:A total of 286 186 patients with AOM were enrolled. The most frequent diagnoses were “nonsuppurative otitis media, unspecified” (47.6%) and “otitis media, unspecified” (39.0%). The diagnoses of hemorrhagic bullous myringitis (BM) or influenza-induced AOM were very rarely found in the database. The highest incidence of IED after 7 days and 12 months was found in “nonsuppurative otitis media, unspecified” (7.7% and 15.9%, respectively), followed by “otitis media, unspecified” (5.6% and 13.5%, respectively). The incidences of the most frequent IED “hearing loss, unspecified” and “sensorineural hearing loss, unspecified” increased proportionally with increasing patient age, while the rare diagnoses of “labyrinthitis” and “ototoxic hearing loss” were evenly distributed among the age groups.Conclusion:In ENT practices in Germany, both various forms of AOM, as well as simultaneous or subsequent IED, are mostly being coded in an unspecific way, while specific forms such as hemorrhagic BM, influenza-induced AOM, and labyrinthitis are coded very rarely. Older patients have a higher risk of IED in AOM. A visit due to AOM seems to be a regular occasion for the initial diagnosis of hearing impairment in the elderly individuals. The highest risk of IED was found in nonsuppurative AOM.

Background Vestibular dysfunction is relatively common in dogs, with a prevalence of 0.08% reported in primary veterinary care in the UK. There are several studies investigating how to differentiate between peripheral and central vestibular disease but only limited information regarding the possible underlying causes for peripheral vestibular dysfunction in dogs. This study therefore aimed to describe the clinical signs, magnetic resonance imaging findings (MRI), underlying causes and outcome in a large population of dogs diagnosed with peripheral vestibular disease. Results One hundred eighty-eight patients were included in the study with a median age of 6.9 years (range 3 months to 14.6 years). Neurological abnormalities included head tilt (n = 185), ataxia (n = 123), facial paralysis (n = 103), nystagmus (n = 97), positional strabismus (n = 93) and Horner syndrome (n = 7). The most prevalent diagnosis was idiopathic vestibular disease (n = 128), followed by otitis media and/or interna (n = 49), hypothyroidism (n = 7), suspected congenital vestibular disease (n = 2), neoplasia (n = 1) and cholesteatoma (n = 1). Long-term follow-up revealed persistence of head tilt (n = 50), facial paresis (n = 41) and ataxia (n = 6) in some cases. Recurrence of clinical signs was observed in 26 dogs. Increasing age was associated with a mild increased chance of diagnosis of idiopathic vestibular syndrome rather than otitis media and/or interna (P = 0.022, OR = 0.866; CI 0.765–0.980). History of previous vestibular episodes (P = 0.017, OR = 3.533; CI 1.251–9.981) was associated with an increased likelihood of resolution of the clinical signs whilst contrast enhancement of cranial nerves VII and/or VIII on MRI (P = 0.018, OR = 0.432; CI 0.251–0.868) was associated with a decreased chance of resolution of the clinical signs. Conclusions Idiopathic vestibular disease is the most common cause of peripheral vestibular dysfunction in dogs and it is associated with advanced age. Incomplete recovery from peripheral vestibular disease is common, especially in dogs presenting with cranial nerve enhancement on MRI but less so if there is previous history of vestibular episodes.

Objective The aim of this study was to report a case of cochlear implantation (CI) for a patient with an otic capsule-sparing traumatic brain injury (TBI) and to review the relevant literature. Methods A patient with history of TBI received a CI for bilateral profound hearing loss. A systematic review of the literature was performed to identify and compare similar cases. Results A 36-year-old male with a history of hearing loss from right acute labyrinthitis was referred for bilateral profound sensorineural hearing loss (SNHL) after a fall with associated injury to the central auditory nervous system (CANS) including the brainstem. On the right, behavioral acoustic threshold measurements were in the profound range with absent OAEs. On the left, testing revealed no measurable behavioral acoustic thresholds and variable physiologic measures. A right unilateral cochlear implant was performed with most recent follow-up demonstrating speech awareness thresholds of 25 dB HL with excellent detection of all 6 Ling sounds. However, the patient also continues to suffer from other neurologic sequelae related to his TBI, which challenge his ability to demonstrate objective and subjective benefit. A systematic review of the literature demonstrates variable outcomes for patients with TBI and SNHL. Conclusions Patients with profound SNHL and TBI present a distinct rehabilitative challenge for clinicians. CI may provide meaningful benefit in this population, though care should be taken in patient selection and counseling.

Introduction Streptococcus suis is responsible for a zoonosis for which Suidae (pigs and wild boars) constitute the reservoir, mainly in Asia, with a much lower prevalence in Europe. The predominant clinical manifestation is meningitis, possibly resulting in deafness. Case report We report the case of a woman hospitalised for meningitis complicated by labyrinthitis, occurring several hours after preparing a meal composed of wild boar meat. Despite the presence of intracochlear fibrosis, the patient was managed by sequential bilateral cochlear implants. Discussion The discussion presents a review of the international literature and describes the mechanisms responsible for hearing loss related to this rare zoonosis.

Background Acute dizziness may be the only symptom of stroke. Prevalence of this disease among patients with isolated dizziness differs significantly and depends on study design, inclusion criteria and diagnostic methods. In available investigations, we did not find any prospective studies where magnetic resonance imaging, positional maneuvers, and Halmagyi-Curthoys test had been used to clarify a pattern of diseases with isolated acute dizziness and suspected stroke.Aim of study To clarify the pattern of the causes of dizziness in patients with suspected acute stroke.Material and methods We examined 160 patients admitted to N.V. Sklifosovsky Research Institute for Emergency Medicine with suspected stroke and single or underlying complaint of dizziness. All patients were examined with assessment of neurological status, Dix-Hollpike and Pagnini-McClure maneuvers, HalmagyiCurthoys test, triplex scans of brachiocephalic arteries, transthoracic echocardiography, computed tomography (CT) and magnetic resonance imaging (MRI) of the brain with magnetic field strength 1.5 T. MRI of the brain was performed in patients without evidence of stroke by CT and in patients with stroke of undetermined etiology according to the TOAST classification.Results In 16 patients (10%), the cause of dizziness was a disease of the brain: ischemic stroke (n=14 (88%)), hemorrhage (n=1 (6%)), transient ischemic attack (TIA) of posterior circulation (n=1 (6%)). In 70.6% patients (n=113), the dizziness was associated with peripheral vestibulopathy: benign paroxysmal positional vertigo (n=85 (75%)), vestibular neuritis (n=19 (17%)), Meniere’s disease (n=7 (6%)), labyrinthitis (n=2 (1,3%)). In 6.9% patients (n=11), the cause of dizziness was hypertensive encephalopathy, 1.9% of patients (n=3) had heart rhythm disturbance, 9.4% of patients (n=15) had psychogenic dizziness, 0.6% of patients (n=1) had demyelinating disease, and 0.6% of patients (n=1) had hemic hypoxia associated with iron deficiency anemia.Conclusion In 70.6% patients with acute dizziness, admitted to hospital with a suspected stroke, peripheral vestibulopathy was revealed. Only 10% of patients had a stroke as a cause of dizziness.

Purpose To report the postoperative magnetic resonance imaging (MRI) features after superior semicircular canal plugging in patients with Minor syndrome. Materials and methods The MRI examinations with 3D T2-weighted SPACE sequence of 12 patients with superior semicircular canal dehiscence syndrome (SCDS) were retrospectively assessed. Two radiologists independently evaluated the presence of a filling defect of the superior semicircular canal above the superior ampulla and the common crus using an oblique plane parallel to the superior semicircular canal (Poschl's plane). Results Postoperative MRI showed a filling defect above the ampulla of the superior semicircular canal and the common crus in 8/12 patients (67%). Three patients (3/12; 25%) had a filling defect involving also the superior ampulla that caused postoperative labyrinthitis with labyrinthine enhancement on MRI in 2 patients. One patient (1/12; 8%) had incomplete plugging of superior semicircular canal with abnormal functional tests and remaining symptoms. Conclusion Postoperative MRI shows a normal plugging aspect of the superior semicircular canal in 67% of patients. MRI can reveal complications that may have therapeutic implications.

Objectives Bacterial meningitis can cause a labyrinthitis. Consequences often are intracochlear soft tissue neoformation (cochlear obliteration) or intracochlear osteoneogenesis (cochlear ossification) and deafness. Cochlear implantation becomes challenging and hearing rehabilitation is complicated. This retrospective case-control-study aimed to find correlations between morphologic, electric and functional parameters. Methods The study group included children, who lost hearing due to a bacterial meningitis (n = 35 cases). Using preoperative computed tomography and intraoperative findings we grouped into ‘unaltered cochleae’, ‘obliterated cochleae’ and ‘ossified cochleae’. Control group children suffered from deafness (n = 16) of other aetiology and presented with radiologically unchanged cochleae. Postoperative routine controls documented impedances, stimulation charge and hearing tests a various time points, which all were analysed. Results Control group patients showed a mean impedance of 6.3 kΩ and the mean charge applied was 19 nC. The study group averaged at 7.9 kΩ and 24.6 nC respectively. Patients with ossified cochleae had increased values of 8.6 kΩ and 29.7 nC. The control group reached a monosyllabic word understanding of 74% and the study group of 58%. Patients with ossified cochleae reached 36%. Conclusions Impedances and stimulation charge influence each other. Increased charge is necessary for higher cochlear implant output. Despite higher charges, patients with obliterated and patients with ossified cochleae significantly perform worse in hearing rehabilitation. Reduced audiological outcome in study group patients without morphologic cochlear changes furthermore hints at additional factors besides cochlear tissue neogenesis like postinflammational changes at the neural pathway.

Objective Distinguishing intralabyrinthine schwannoma (ILS) from labyrinthitis is crucial in deciding patient management between surgery and medication; however, the clinical and radiological differentiation between ILS and labyrinthitis is difficult, especially when labyrinthitis shows contrast-enhancement mimicking ILS on magnetic resonance imaging (MRI). This study aimed to evaluate the quantitative characteristics of signal intensity on 3T MRI for differentiating ILS from contrast-enhancing labyrinthitis (CEL). Study design Retrospective study. Setting Tertiary referral center. Patients Between July 2003 and June 2017, 9 subjects diagnosed with ILS (16 MRI cases) and 9 subjects diagnosed with CEL (9 MRI cases) were enrolled in the study. Main outcome measures Normalized signal intensity on contrast-enhanced T1-weighted image (nCE-T1), normalized signal intensity on T2-weighted image (nT2), and normalized signal intensity on contrast-enhanced fluid-attenuated inversion recovery image (nFLAIR) were measured and compared between ILS and CEL. The diagnostic performance of these values was assessed using receiver operating characteristic (ROC) analysis. Results In ILS, the nCE-T1 and the nT2 were significantly higher and lower, respectively, than in CEL (all, p 0.05). The maximal nCE-T1 achieved the highest area under the curve (AUC) in differentiating ILS and CEL, followed by the mean nCE-T1, the mean nT2, and the maximal nT2. Combination of the nCE-T1 and the nT2 showed higher AUC than the nCE-T1 alone, but without statistical significance (p = 0.340). Conclusions Quantitative measurement of the signal intensity on MRI can be a viable imaging tool for differentiating ILS from CEL.

The purpose of this study was to report the prevalence of vestibular impairment (VI) in children (n = 2,528) referred for complete vestibular testing because of balance disorders (BD) or hearing loss (H). A VI was shown in 51.5% of the children tested (1,304/2,528). For BD (e.g., vertigo, dizziness, instability, delay in posturomotor development), VI was found in 36.5% (n = 379/1,037). The most frequent causes of BD with VI included inner ear malformation (13.5%), delay in posturomotor development (13.4%), hearing loss revealed with vertigo (3.9%), trauma (3.9%), vestibular neuritis (3.3%), meningitis (2.5%), Meniere-like syndrome (1.1%), BPPV posttrauma (1%), labyrinthitis (0.4%), and unknown etiology (19.6%). Normal responses to the complete battery of tests (n = 658, 63.5%) excluded a vestibular origin to BD, leading to other diagnoses: principally migraine (15.6%), ophthalmological disorders (15.1%), neurological disorders (including delay in posturomotor development; 14.4%), orthostatic hypotension, or somatoform dizziness (

Background An aural cholesteatoma, more appropriately named tympanokeratoma, is an epidermoid cyst of the middle ear described in several species, including dogs, humans and Mongolian gerbils. The cyst lining consists of stratified, keratinizing squamous epithelium with central accumulation of a keratin debris. This case report describes vestibular ganglioneuritis and perineuritis in a dog with chronic otitis, bilateral tympanokeratomas and presumed extension of otic infection to the central nervous system. Case presentation An 11-year-old intact male Dalmatian dog with chronic bilateral otitis externa and sudden development of symptoms of vestibular disease was examined. Due to the dog’s old age the owner opted for euthanasia without any further examination or treatment and the dog was submitted for necropsy. Transection of the ears revealed grey soft material in the external ear canals and pearly white, dry material consistent with keratin in the tympanic bullae bilaterally. The brain and meninges were grossly unremarkable. Microscopical findings included bilateral otitis externa and media, unilateral otitis interna, ganglioneuritis and perineuritis of the spiral ganglion of the vestibulocochlear nerve and multifocal to coalescing, purulent meningitis. A keratinizing squamous epithelial layer continuous with the external acoustic meatus lined the middle ear compartments, consistent with bilateral tympanokeratomas. Focal bony erosion of the petrous portion of the temporal bone and squamous epithelium and Gram-positive bacterial cocci were evident in the left cochlea. The findings suggest that meningitis developed secondary to erosion of the temporal bone and ganglioneuritis and/or perineuritis of the vestibulocochlear nerve. Conclusions Middle ear tympanokeratoma is an important and potentially life-threatening otic condition in the dog. Once a tympanokeratoma has developed expansion of the cyst can lead to erosion of bone and extension of otic infection to the inner ear, vestibulocochlear ganglion and nerve potentially leading to bacterial infection of the central nervous system. Electronic supplementary material The online version of this article (10.1186/s13028-018-0386-4) contains supplementary material, which is available to authorized users.