Ocular vestibular evoked myogenic potentials and intravestibular intralabyrinthine schwannomas.

Intravestibular intralabyrinthine schwannomas (ILSs) are uncommon benign tumors that arise from the saccular, utricular, and lateral and superior ampullary nerves. According to the literature, there is an average delay of 8 years between the onset of symptoms and diagnosis. The diagnosis is based on an audiovestibular examination and magnetic resonance imaging (MRI). We describe a case of intravestibular ILS in which we included the ocular vestibular evoked myogenic potentials (oVEMPs) test in the diagnostic workup. The oVEMPs test is a relatively new neurophysiologic diagnostic modality that evaluates the superior vestibular pathway and the ascending contralateral pathway through the vestibulo-ocular reflex. In our case, a 65-year-old man presented with progressive right-sided sensorineural hearing loss, dizziness, and tinnitus and fullness in his right ear. Audiovestibular examination and MRI detected an intravestibular ILS on the right. We found that oVEMPs were absent on the contralateral side, which contributed to the diagnostic process. The detection of oVEMPs can provide detailed information on the functionality of the macula of the utricle and the lateral and superior ampullary nerves, with a precise identification of the affected area. Based on our findings, we discuss the role of oVEMPs in the diagnosis of an intravestibular ILS.

Unilateral Enlarged Vestibular Aqueduct Syndrome and Bilateral Endolymphatic Hydrops.

Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH.

Day-case stapedotomy: is it a viable option?

CONCLUSIONS: Stapedotomy is, in our opinion, the technique of choice in stapes surgery. The precision of this technique allows clinicians to perform the surgical procedure in day surgery under local anesthesia. OBJECTIVES: There is a strong emphasis on increasing the number of elective day surgery cases, especially in the patients' best interest, as it decreases the likelihood of late cancellation and hospital-acquired morbidity. A prospective study was performed to determine whether stapes surgery for otosclerosis could be performed safely in an outpatient setting. METHODS: We present a series of stapes surgery cases for otosclerosis performed on a day-case basis. We performed a classic stapedotomy in 9 patients, a reverse classic step stapedotomy in 2 patients, a partial reverse classic step stapedotomy in 11 patients, and a hemi-stapedectomy in two patients. RESULTS: Three of 24 patients (12.5%) treated with classic stapedotomy, 1 patient with partial reverse classic step stapedotomy, and 1 patient with hemi-stapedectomy were formally admitted to the hospital after surgery (length of stay, 23 h). The indications were vertigo (two patients) and asthenia (one patient). These patients were treated under general anesthesia. Two of these patients resided more than 250 km away from the hospital.

Vestibular evoked myogenic potential in vestibular neuritis.

This study wants to show the diagnostic value of vestibular evoked myogenic potential (VEMP) in the diagnosis of vestibular neuritis (VN), independently of the caloric test results. Twenty patients were enrolled with acute vertigo caused by VN. VEMP was tested with the binaural simultaneous stimulation method. Surface electromyographic activity was recorded in the supine patients from symmetrical sites over the upper half of each sternocleidomastoid muscle, with a reference electrode on the lateral end of the upper sternum. During the acute attack, 8 days, 1 month and 3 months after the beginning of the acute attack, all the patients underwent the following examinations: Dix-Hallpike manoeuvre, Pagnini-McClure manoeuvre, head shaking test, pure-tone audiometry, tympanometry, caloric labyrinth stimulation according to the Fitzgerald-Hallpike method and VEMP. At the last visit, the 11 patients diagnosed with superior branch vestibular neuritis did not show any improvement at the caloric labyrinth stimulation and presented VEMP on both sides with normal amplitude and latency; in the 9 cases diagnosed with inferior branch vestibular neuritis, there was an improvement of the VEMP reflex and normal caloric test. Our experience highlights that VEMP recording is applicable for patients with VN as a screening test.

Post-traumatic camel-related benign paroxysmal positional vertigo.

INTRODUCTION: Benign paroxysmal positional vertigo (BPPV) is a common form of dizziness. The causes of BPPV are not yet known but a relationship between the onset of vertigo and head trauma has been found. Among the causes of head injury related to BPPV, dropping off a camel has not been reported in literature yet. CASE REPORT: We describe two cases of persons that fell off a camel during a safari in Middle East countries. After the fall they reported vertigo symptoms that were not interpreted as BPPV. When they returned to Italy, due to symptoms persistence, they were referred to our ENT practice: we found evidence of BPPV. In a case it was a bilateral BPPV (bBPPV). DISCUSSION: Falling off a camel may be a relevant cause of BPPV. We suggest a correct evaluation of the labyrinth for BPPV with the appropriate diagnostic maneuvers and, if necessary, a treatment with repositioning maneuvers.

Clinical and audiological follow up of a family with the 8363G>A mutation in the mitochondrial DNA.

Hearing loss is relatively common in mtDNA-related disorders. While auditory function has been assessed fully in the syndrome of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes, few studies have investigated the degree of progressive hearing deficit in individuals bearing other mtDNA mutations. We performed a 4-year clinical and audiological follow up in a family carrying the 8363G>A mutation in the mitochondrial transfer ribonucleic acid lysine (tRNA(Lys)) gene who displayed a progressive neuromuscular disease. In addition to pure tone audiometry, we considered distortion products of otoacoustic emissions, a sensitive indicator of cochlear dysfunction, as well as brainstem auditory evoked responses. A generalized increase in the auditory threshold at follow up, indicating a cochlear impairment in three cases, was noted. Distortion products of otoacoustic emissions may reveal sub-clinical cochlear dysfunction, even in oligosymptomatic patients. A complete and periodical assessment of the hearing function should be encouraged in asymptomatic relatives of patients carrying the tRNA(Lys) 8363G>A mutation.