Assessing the efficacy of perilymphatic fistula repair surgery in alleviating vestibular symptoms and associated auditory impairments.

Perilymph Fistula (PLF), abnormal communication between the fluid-filled space of the inner ear and the air-filled space of the middle ear, is a significant cause of vestibular and auditory symptoms. This is a retrospective study of 22 cases treated with PLF repair surgery, selected based on our surgical indication. We analyzed the characteristics of these 22 cases and evaluated the efficacy of PLF repair surgery in treating vestibular and auditory symptoms. Cases with antecedent events had significantly shorter intervals before surgery. The postoperative recovery from vestibular symptoms following PLF repair surgery was strikingly rapid, with 82% of cases demonstrating marked improvement within a week, even in chronic cases. Despite the notable absence of a control group in the study, the marked improvements in vestibular symptoms and substantial reductions in Dizziness Handicap Inventory (DHI) scores suggest that the observed benefits are attributable to the surgical intervention. Further, timely surgery showed improvements in hearing, with some benefits also seen in late-stage surgeries. Using the perilymph-specific protein Cochlin-tomoprotein (CTP) as a diagnostic biomarker, we could prove that PLF could be responsible for disequilibrium and related auditory disturbances in these patients. A new hypothesis is proposed that the chronic disequilibrium experienced by many PLF patients is due to enhanced mobility of the utricle and not to endolymphatic hydrops. Further research is needed to fully elucidate PLF's symptoms and treatment efficacy using the surgical indication we developed.

Comment on Tabet et al. Vestibular Migraine versus Méniere's Disease: Diagnostic Utility of Electrocochleography. Audiol. Res. 2023, 13, 12-22.

I wish to comment on an aspect of the recently published article by Saliba I. et al., titled Vestibular Migraine versus Meniere's Disease: Diagnostic Utility of Electrocochleograpy [...].

Acute unilateral vestibulopathy/vestibular neuritis: Diagnostic criteria.

This paper describes the diagnostic criteria for Acute Unilateral Vestibulopathy (AUVP), a synonym for vestibular neuritis, as defined by the Committee for the Classification of Vestibular Disorders of the Bárány Society. AUVP manifests as an acute vestibular syndrome due to an acute unilateral loss of peripheral vestibular function without evidence for acute central or acute audiological symptoms or signs. This implies that the diagnosis of AUVP is based on the patient history, bedside examination, and, if necessary, laboratory evaluation. The leading symptom is an acute or rarely subacute onset of spinning or non-spinning vertigo with unsteadiness, nausea/vomiting and/or oscillopsia. A leading clinical sign is a spontaneous peripheral vestibular nystagmus, which is direction-fixed and enhanced by removal of visual fixation with a trajectory appropriate to the semicircular canal afferents involved (generally horizontal-torsional). The diagnostic criteria were classified by the committee for four categories: 1. "Acute Unilateral Vestibulopathy", 2. "Acute Unilateral Vestibulopathy in Evolution", 3. "Probable Acute Unilateral Vestibulopathy" and 4. "History of Acute Unilateral Vestibulopathy". The specific diagnostic criteria for these are as follows:"Acute Unilateral Vestibulopathy": A) Acute or subacute onset of sustained spinning or non-spinning vertigo (i.e., an acute vestibular syndrome) of moderate to severe intensity with symptoms lasting for at least 24 hours. B) Spontaneous peripheral vestibular nystagmus with a trajectory appropriate to the semicircular canal afferents involved, generally horizontal-torsional, direction-fixed, and enhanced by removal of visual fixation. C) Unambiguous evidence of reduced VOR function on the side opposite the direction of the fast phase of the spontaneous nystagmus. D) No evidence for acute central neurological, otological or audiological symptoms. E) No acute central neurological signs, namely no central ocular motor or central vestibular signs, in particular no pronounced skew deviation, no gaze-evoked nystagmus, and no acute audiologic or otological signs. F) Not better accounted for by another disease or disorder."Acute Unilateral Vestibulopathy in Evolution": A) Acute or subacute onset of sustained spinning or non-spinning vertigo with continuous symptoms for more than 3 hours, but not yet lasting for at least 24 h hours, when patient is seen; B) - F) as above. This category is useful for diagnostic reasons to differentiate from acute central vestibular syndromes, to initiate specific treatments, and for research to include patients in clinical studies."Probable Acute Unilateral Vestibulopathy": Identical to AUVP except that the unilateral VOR deficit is not clearly observed or documented."History of acute unilateral vestibulopathy": A) History of acute or subacute onset of vertigo lasting at least 24 hours and slowly decreasing in intensity. B) No history of simultaneous acute audiological or central neurological symptoms. C) Unambiguous evidence of unilaterally reduced VOR function. D) No history of simultaneous acute central neurological signs, namely no central ocular motor or central vestibular signs and no acute audiological or otological signs. E) Not better accounted for by another disease or disorder. This category allows a diagnosis in patients presenting with a unilateral peripheral vestibular deficit and a history of an acute vestibular syndrome who are examined well after the acute phase.It is important to note that there is no definite test for AUVP. Therefore, its diagnosis requires the exclusion of central lesions as well as a variety of other peripheral vestibular disorders. Finally, this consensus paper will discuss other aspects of AUVP such as etiology, pathophysiology and laboratory examinations if they are directly relevant to the classification criteria.

Ductus Reuniens and Its Possible Role in Menière's Disease.

OBJECTIVE: After 160 years the true underlying cause of Meniere's disease remains enigmatic. The aim of our study is to discuss the possible implication of an obstruction of the ductus reuniens as a cause in Menière's disease. METHODOLOGY: We first conducted an historical study of the description of the ductus reuniens. We then reviewed the literature regarding ductus reuniens obstruction in animal experiments, human post-mortem studies and living ear imaging. We completed its description by modern microCT imaging. Limited knowledge on the fate of dislodged saccular otoconia is summarized. The possible implications for Meniere's attacks are discussed. RESULTS: Victor Hensen was the first to describe the ductus reuniens in 1863. He described its length and width and predicted that saccular otoconia might enter the ductus and the cochlea. On microCT the narrowest width of the human ductus reuniens was 0.14 mm. The literature reports cochlear endolymphatic hydrops occurring after animal experimental obstruction of the duct. Human postmortem studies have confirmed saccular otoconial clumps entering the ductus and the cochlea. A postmortem study has shown sites of endolymphatic obstruction, and imaging speculates on blockages in ears with Meniere's disease. Dislodged utricular otoconia can be in clumps of otolithic membranes. CONCLUSION: Blockages of the ductus reuniens and at other endolymphatic system sites appear to be a feature in Meniere's disease ears. The blockages have been postulated to be saccular otoconia either causing or aggravating hydrops. This could be consistent with observed nystagmus reversals during attacks as the endolymphatic sac attempts to clear the hydrops and the otoconia.

Benign paroxysmal positional vertigo in Meniere's disease: systematic review and meta-analysis of frequency and clinical characteristics.

There is a recognized association of Meniere's disease (MD) and benign paroxysmal positional vertigo (BPPV). However, the frequency and clinical characteristics of BPPV in MD are unclear. The aim of this review was to determine the mean frequency and clinical features of BPPV in MD. Three databases were searched: MEDLINE, PubMed and Google Academia. Studies reporting the frequency of BPPV in MD were pooled. A total of 4198 references were identified, of which 20 studies were considered eligible. The pooled frequency of BPPV in MD was 14% (95% CI 9-18%). It was 38% (95% CI 26-49%) in longitudinal studies and 8% (95% CI 6-11%) in cross-sectional ones. BPPV comorbid with MD was mostly observed in the ear affected by hydrops, in females, in patients with more advanced disease. Canalolithiasis of the horizontal semicircular canal was more common in patients with BPPV associated with MD than in idiopathic BPPV. BPPV in MD was more prone to recurrence and required more canal repositioning maneuvers.

Bilateral clicking tinnitus due to myoclonus of middle ear muscles.

Isolated middle ear myoclonus can be a cause of objective tinnitus. We present an acoustically documented case of irregular bilateral middle ear myoclonus with loud clicking, and roaring tinnitus associated with essential palatal tremor. A palatal botulinum toxin injection did not eliminate the tinnitus. Division of both middle ear tendons in both ears abolished the clicking tinnitus with no effect on hearing.

Creaky voice or extreme vocal fry in young women.

An extreme use of a voice feature with a lowered rough sound called vocal fry or creaky voice has become increasingly recognised in American, British and New Zealand English speaking young women. It is not regarded as an involuntary voice disorder, but rather as a volitional strategy. Intermittent vocal fry is recognised as a common voice feature, particularly at the end of a sentence. It occurs at the lowest range of a speaker's F0 (pitch). We present evidence that vocal fry use is increasing in young New Zealand women. This article is to highlight the new phenomenon of extreme and sustained vocal fry as a vocal style, which is the first voice feature to have come to the attention of the general public through the news media.

Tone Burst Electrocochleography for the Diagnosis of Clinically Certain Meniere's Disease.

The technique of transtympanic electrocochleography was initially developed as an objective hearing threshold test by Eggermont. Gibson et al. (1977) claimed that an enlarged direct current component of the action potential (AP) called the summating potential (SP) is an indication of endolymphatic hydrops, later confirmed by Coates who proposed an SP/AP ratio measure. This led to numerous publications using diagnostic ratios of 0.33-0.35. The insensitivity led to an eventual disenchantment with the test as a reliable objective test for Meniere's disease. It was further confused by audiologists employing remote canal or ear drum electrodes which give a response about one-fourth of the magnitude obtained by an electrode in contact with the cochlea. Subsequently Gibson stated that an SP/AP ratio of <0.5 is not diagnostic for hydrops. He then showed that a tone burst stimulus gave the test a significantly higher sensitivity and specificity, which has been supported by others. On MRI inner ear imaging with gadolinium hydrops can be seen, but the quality of images and what is seen may vary according to brand of scanner, settings, mode of gadolinium administration, and the possibility that gadolinium entry may favor the vestibule. Transtympanic tone burst electrocochleography is to date the simplest, cheapest and most sensitive technique for detecting cochlear endolymphatic hydrops to confirm a diagnosis of Meniere's disease.

A New Theory for Ménière's Disease: Detached Saccular Otoconia

Ménière's disease is an inner ear disorder characterized by vertigo attacks, fluctuating and progressive hearing loss, tinnitus, and aural fullness in the affected ear. The pathophysiology of Ménière's disease remains elusive. Theories so far are anatomical variation in the size or position of the endolymphatic sac and duct, viral inflammation or autoimmune involvement of the sac, or a genetically determined abnormality of endolymph control. Animal studies on blocking the ductus reuniens and endolymphatic duct have produced hydrops in the cochlea, saccule, and utricle. Cone beam computed tomography images show a similar pattern with apparent obstruction of the ductus reuniens, saccular duct, and endolymphatic sinus. New studies documenting the age of onset of Ménière's disease show a pattern similar to benign paroxysmal positional vertigo, raising the possibility that the fundamental cause of Ménière's disease might be detached saccular otoconia.

oVEMPs and cVEMPs in patients with 'clinically certain' Menière's disease.

OBJECTIVE: To measure ocular vestibular-evoked myogenic potentials (oVEMPs) and cervical vestibular-evoked myogenic potentials (cVEMPS) in Menière's disease patients with confirmed cochlear hydrops and in the normal ears of volunteers. METHODS: oVEMPs and cVEMPs were measured in 18 patients with a symptomatic diagnosis of Menière's disease and tone burst electrocochleographic confirmation of hydrops, and in the ears of 22 volunteers. RESULTS: Threshold measures: For cVEMP: no significant differences between Menière's ears and controls; for oVEMP: significantly elevated thresholds in affected ears of Menière's ears compared with their unaffected ears, but not with controls. Latency measures: cVEMP N1 peaks were significantly prolonged compared with the left and right ears of controls, but not with the non-affected ear. Amplitude measures: cVEMP P1N1 and N1P2 measures were significantly reduced compared with the right ear of controls, but not with the non-affected ear; For oVEMP, N2P2 amplitudes were significantly reduced compared with both ears of controls but not with the non-affected ear. CONCLUSION: Abnormalities of oVEMPs and cVEMPs were found in 18 Menière's disease patients who had an independent confirmation of cochlear hydrops. The overlap of the results from Menière's patients compared with normal controls limits the use of VEMP abnormalities as a sole reliable diagnostic test for Menière's disease.

MRI Inner Ear Imaging and Tone Burst Electrocochleography in the Diagnosis of Ménière's Disease.

OBJECTIVE: To compare the sensitivity of gadolinium MRI inner imaging with tone burst electrocochleography (EcochG) for diagnosing endolymphatic hydrops. STUDY DESIGN: A prospective study on patients who were to have an MRI scan to exclude retrocochlear pathology. SETTING: Tertiary care center. PATIENTS: One hundred and two patients: 57 patients with Possible, Probable, or Definite Ménière's Disease, 25 with asymmetrical hearing loss, 18 with sudden sensorineural hearing loss, and 2 with unilateral tinnitus had additional MRI inner ear imaging and click and tone burst stimulus EcochG testing. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: To compare the sensitivity of the two techniques. RESULTS: In 30 patients with symptom-based Definite Ménière's Disease, tone burst EcochG was positive in 25 (83%) and the click EcochG was positive in 9/30 (30%), and gadolinium MRI imaging diagnosed hydrops in 14 (47%). A positive result for either MRI imaging or tone burst EcochG was seen in 26 patients (87%). In 14 subjects with symptom-based Probable Ménière's Disease, 10 (71%) had either a positive EcochG or MRI. In 13 with Possible Ménière's Disease, four (31%) had a positive EcochG or MRI. CONCLUSION: This study confirms the greatly enhanced diagnostic sensitivity of tone burst EcochG over click response in diagnosing endolymphatic hydrops in Ménière's disease. Even though adequate MRI imaging was achieved in 90%, tone burst EcochG was a more sensitive test.

A rare cause of nasal septal abscess.

We describe a patient with mid-facial pain and nasal obstruction due to a nasal septal abscess (NSA) complicating an occult fungal ball of the sphenoid sinus. We highlight the importance of suspecting unusual pathology in patients with NSA and no trauma history.

A balance test for chronic perilymph fistula.

Perilymph fistula is defined as a leak of perilymph at the oval or round window. It excludes other conditions with "fistula" tests due to a dehiscent semicircular canal from cholesteotoma and the superior canal dehiscence syndrome. First recognized as a complication of stapedectomy, it then became apparent that head trauma and barotraumatic trauma from flying or diving could be a cause. Descriptions of "spontanenous" perilymph fistulas with no trauma history followed. It is likely that most perilymph fistula patients have a congential potential weakness of the otic capsule at the round or oval window. The vestibular symptoms have been assumed to be due to endolymphatic hydrops, but there is poor evidence. Their unilateral disequilibrium, nausea, and subtle cognitive problems suggest they are due to otolith disfunction and that these patients have a specific balance abnormality, unlike subjects with unilateral vestibular hypofuction. In this series of twenty patients with a confirmed fistula a logical simplification of Singleton's "eyes-closed turning" test predicted a PLF in twelve with a trauma history. In four no cause was found. In three a prior traumatic event was later recalled, but one patient had concealed it.

Evaluating iPhone recordings for acoustic voice assessment.

AIMS: This study examined the viability of using iPhone recordings for acoustic measurements of voice quality. METHODS: Acoustic measures were compared between voice signals simultaneously recorded from 11 normal speakers (6 females and 5 males) through an iPhone (model A1303, Apple, USA) and a comparison recording system. Comparisons were also conducted between the pre- and post-operative voices recorded from 10 voice patients (4 females and 6 males) through the iPhone. Participants aged between 27 and 79 years. RESULTS: Measures from iPhone and comparison signals were found to be highly correlated. Findings of the effects of vowel type on the selected measures were consistent between the two recording systems and congruent with previous findings. Analysis of the patient data revealed that a selection of acoustic measures, such as vowel space area and voice perturbation measures, consistently demonstrated a positive change following phonosurgery. CONCLUSION: The present findings indicated that the iPhone device tested was useful for tracking voice changes for clinical management. Preliminary findings regarding factors such as gender and type of pathology suggest that intra-subject, instead of norm-referenced, comparisons of acoustic measures would be more useful in monitoring the progression of a voice disorder or tracking the treatment effect.

Contemporary surgical management of severe sialorrhea in children.

The causes of severe sialorrhea (drooling) are reviewed, and in particular in children in whom it can become a life-long disability. The history of medical and surgical treatments is discussed. A major advance has been the surgical relocation of the submandibular gland ducts with removal of sublingual glands. The results of this operation, technical considerations, and its outcomes in 16 children are presented. There were no significant complications. Caregivers judged the efficacy with a median score of "75%" improvement. The technique has become the most logical and reliable surgical treatment for drooling, with very good control in most cases. In contrast to "Botox" its effects are permanent.