Loop characteristics and audio-vestibular symptoms or hemifacial spasm: is there a correlation? A multiplanar MRI study.

AIM: We investigated if loop characteristics correlate with audio-vestibular symptoms or hemifacial spasm in patients with a vascular loop in the root entry zone (VII and VIII) and in the internal auditory canal. MATERIALS AND METHODS: A retrospective, multicenter study analyzed 2622 consecutive magnetic resonance imaging (MRI) scans of the cerebellopontine angle of patients with asymmetric audio-vestibular symptom or hemifacial spasm; patients' symptoms were confirmed by clinical tests. MRIs displaying vascular loops visible in the axial view were analyzed using multiplanar reconstruction. We evaluated (1) depth of penetration of the loop into the internal auditory canal (IAC); (2) largest diameter of the vessel; (3) nerve(s) involved in the vascular impingement, position of the loop relative to such nerve(s) and number of contacts between vessel and nerve(s); (4) length of such contact. The loop metrics described above were correlated with the patients' audio-vestibular symptoms and hemifacial spasm. RESULTS: Three hundred ninety-nine patients displayed a loop visible in the MRI axial view and out of them only 118 displayed a direct contact between loop and nerve. The cochlear nerve was involved in a contact in 57.7%. Loops in direct nerve contact had a calibre > 0.85 mm, were located in the middle portion of the IAC, and correlated with vertigo (p = 0.002), tinnitus (p = 0.003), and hemifacial spasm (p < 0.001). Asymmetric sensorineural hearing loss (SNHL) correlated with number of contacts (p < 0.001) and length of contact (p < 0.05). The contact was asymptomatic in 41.5% of patients. CONCLUSION: Loop characteristics may help predict whether a vascular impingement is responsible for a symptom and guide the physician to select the best treatment. KEY POINTS: • A vascular loop in the internal auditory canal was observed in 18-20% of the patients in this study; whether a loop can be responsible for a compressive syndrome is still unclear in particular referred to the vestibulocochlear nerve. • Compression by a loop on the facial nerve causes hemifacial spasm; compression by a loop on the cochlear or vestibular nerve may cause audio-vestibular symptoms. • In patients with a loop, the loop calibre, the loop position, and the number of loop-nerve(s) assessed via the multiplanar MRI reconstruction technique may help assess whether the patient will manifest audio-vestibular symptoms or hemifacial spasm.

Inner ear function in patients with obstructive sleep apnea.

OBJECTIVE: Because of their high metabolic activity and low-resting oxygen tension, the organs of the inner ear are vulnerable to hypoxia, a condition that occurs repetitively in obstructive sleep apnea-hypopnea syndrome (OSAHS). The present study aimed to investigate the inner ear function of patients with OSAHS. METHODS: A total of 58 patients with OSAHS (116 ears) and 20 adults without OSAHS were enrolled in the present study. The clinical features, such as air-conduction thresholds, auditory brainstem response (ABR, 11 times/s and 51 times/s stimulation rates), and distorted products otoacoustic emission (DPOAE), were evaluated and compared between these two groups. RESULTS: Air-conduction thresholds at 4 kHz and 8 kHz were higher in patients with OSAHS compared with controls (P < 0.001). At the rate of 11 times per second, biauricular wave I latencies and wave V latencies in the OSAHS group were longer than those in the control group (1.51 ± 0.13 vs. 1.33 ± 0.07 ms, P < 0.001; 5.65 ± 0.23 vs. 5.53 ± 0.23 ms, P = 0.0016). At the rate of 51 times per second, biauricular wave I latencies and wave V latencies in the OSAHS group were longer than those in the control group (1.64 ± 0.12 vs. 1.44 ± 0.06 ms, P = 0.0001; 5.92 ± 0.26 vs. 5.80 ± 0.18 ms, P = 0.0077). However, there was no significant difference in the wave I and wave V interval between these two groups (P = 0.10). DPOAE amplitude was significantly reduced in OSAHS patients, although no hearing loss was observed. CONCLUSION: High-frequency hearing loss was detected in adults with severe OSAHS, and wave I latencies and wave V latencies of ABR were prolonged.

A narrative review of obesity and hearing loss.

The comorbidities related to obesity are already extensive, but as the prevalence of obesity increases globally, so do the number of its associated conditions. The relationship between hearing impairment and obesity is a relatively recent research interest, but is significant as both conditions have the ability to substantially reduce an individual's quality of life both physically and psychologically. Obesity has a significant effect on vascular function, and this may have an impact on highly vascular organs such as the auditory system. This review aims to provide an overview of the existing literature surrounding the association between hearing loss and obesity, in order to emphasise these two highly prevalent conditions, and to identify areas of further investigation. Our literature search identified a total of 298 articles with 11 articles of relevance to the review. The existing literature in this area is sparse, with interest ranging from obesity and its links to age-related hearing impairment (ARHI) and sudden sensorineural hearing loss (SSNHL), to animal models and genetic syndromes that incorporate both disorders. A key hypothesis for the underlying mechanism for the relationship between obesity and hearing loss is that of vasoconstriction in the inner ear, whereby strain on the capillary walls due to excess adipose tissue causes damage to the delicate inner ear system. The identified articles in this review have not established a causal relationship between obesity and hearing impairment. Further research is required to examine the emerging association between obesity and hearing impairment, and identify its potential underlying mechanisms.

Histopathology of the Inner Ear in a Case With Recent Onset of Cogan's Syndrome: Evidence for Vasculitis.

The association of sensorineural hearing loss and vertigo with inflammatory eye disease, usually interstitial keratitis, has been called Cogan's syndrome. The pathogenesis of Cogan's syndrome is unknown, but it has been assumed to be an immune mediated disorder with vasculitis. The histopathology of the inner ear in Cogan's syndrome has been described in 6 case reports. Although common pathologic findings in these reports include degeneration of the auditory and vestibular neuroepithelium, endolymphatic hydrops, fibrosis, and new bone formation, direct pathologic evidence of a vasculitis has not been published. A possible reason for this failure to identify vasculitis was a substantial delay (range, 4-40 years) between the onset of symptoms and examination of the otopathology. In the current case report, the patient had both auditory and vestibular symptoms and interstitial keratitis with a time delay of only 2 to 4 weeks between symptoms and death. Evidence of a vasculitis as a possible underlying etiology included H&E histopathology and anti-CD45 immunostaining of vessels both in the auditory and vestibular systems, supporting the hypothesis of a vasculitis as a mechanism in this disorder.

The clinical characteristics and treatment for sudden sensorineural hearing loss with vestibular schwannoma.

The aim of this study is to analyze the clinical characteristics and treatment of sudden sensorineural hearing loss (SSNHL) patients with vestibular schwannoma (VS). The clinical features of the VS patients were explored by retrospectively analyzing the clinical data from 542 cases of SSNHL patients between January 2008 and March 2013. There were 10 cases (10 ears) diagnosed with VS in 542 cases of SSNHL patients (10 ears, 1.85 %), 3 males, 7 females, with a range of 28-57 years. Among all the cases, eight patients with abnormal ABR, ten with ear ipsilateral stapedius reflexes which were completely not elicited and seven patients with healthy ear contralateral stapedius reflexes which were completely not elicited. Neuromas were classified by Koos grades according to size (8 of grade I, 1 of grade II, 1 of grade IV). Eight small VS  patients were taken waiting and MRI therapy strategies. Meanwhile, we used glucocorticoid treatment and timely and short-term medication to improve the microcirculation of the inner ear for these patients. And four cases' hearing was improved. Some vestibular schwannomas have SSNHL as initial symptoms, especially the small ones in internal auditory canal. To prevent misdiagnosis or leak-diagnosis, MRI should be performed as a routine test for SSNHL, and ABR is sometimes necessary for SSNHL patients. It is also necessary to give appropriate treatment to protect hearing of the small vestibular schwannoma patients whose first symptoms are diagnosed as SSNHL in acute phase.

[The specific features of the anatomical structure of the artery of labyrinth (an anatomical and topographical study)].

The objective of the present work was to study syntopy of the artery of labyrinth using block-preparations of the posterior cranial fossa, variants of its branching-off from the vertebro-basiliar basin (VBB), and peculiar features of its anatomical structure. A total of 12 block-preparations of the posterior cranial fossa were available for the investigation. They were preliminarily stained with red latex and fixed in a three-point system. These procedures were followed by retrosigmoid craniotomy, opening of dura mater in the supero-lateral part of the cerebellomedulllary cistern, traction of the cerebellum, and blunt separation of the basiliar artery (BA). Variants of branching of the antero-inferior cerebellar artery (AICA) and branching of the artery of labyrinth from AICA were studied. It was shown that the artery of labyrinth branches off from the antero-inferior cerebellar artery in 100% of the cases. The latter artery formed a loop in 14% of the cases (3 ears). The average diameter of the labyrinth artery was 0.32 mm and its mean area 0.06 sq.cm. The artery of labyrinth branched off from the posterior para-stem segment of the antero-inferior cerebellar artery in 42.6% of the cases (9 ears), and from the anterior para-stem segment of AICA in 14.2% of the cases (3 ears). Within the conventional «rhombus¼, the artery of labyrinth was straight in 76.2% of the cases (16 ears) and arc-shaped in 23.8% (4 ears).

Cardiovascular risk factors as causes for hearing impairment.

The purpose of this paper is to provide a contemporary review of the correlation between cardiovascular risk factors (CVRFs) and hearing impairment (HI) . We conducted a comprehensive review of the literature in order to assess the effects of the different CVRFs on HI. We focused on the pathological findings in the inner ear and their correlation with cochlear function in population-based studies. We found that CVRFs adversely affect hearing acuity. HI diagnosis should be accompanied by detecting and treating CVRFs, according to the presented outline, which may augment hearing rehabilitation and improve the general health and the well-being of the patient. © 2014 S. Karger AG, Basel.

Idiopathic sudden sensorineural hearing loss: A review focused on the contribution of vascular pathologies.

Idiopathic sudden sensorineural hearing loss (ISSNHL) is characterized by abruptly appearing hearing loss, sometimes accompanied by vertigo. Vascular pathologies (e.g., cochlear ischemia, or cochlear infarction) are one of the most likely causes of ISSNHL. This review aims to present current understanding of inner ear anatomy, clinical features of ISSNHL, and its treatment strategies. The labyrinthine artery is the only end artery supplying blood to the inner ear, and it has three branches: the anterior vestibular artery, the main cochlear artery, and the vestibulo-cochlear artery (VCA). Occlusion of the VCA can be caused by a variety of factors. The VCA courses through a narrow bone canal. ISSNHL is usually diagnosed after excluding retrocochlear pathologies of sudden sensorineural hearing loss (SSNHL), such as vestibular schwannoma. Therefore, a head MRI or assessing auditory brainstem responses are recommended for patients with SSNHL. Severe SSNHL patients with high CHADS2 scores, an index of stroke risk, have a significantly lower rate of vestibular schwannoma than severe SSNHL patients with low CHADS2 scores, suggesting that severe ISSNHL in individuals at high risk of stroke is caused by vascular impairments. Intralabyrinthine hemorrhage causes SSNHL or vertigo, as in ISSNHL. The diagnosis of intralabyrinthine hemorrhage requires careful interpretation of MRI, and a small percentage of patients diagnosed with ISSNHL may in fact have intralabyrinthine hemorrhage. Many studies have reported an association between ISSNHL and atherosclerosis or cardiovascular risk factors (e.g., diabetes mellitus, hypertension, dyslipidemia and cardiovascular disease), and subsequent risk of stroke in patients with ISSNHL may be elevated compared to controls. Increased hearing level on the healthy ear side, high Framingham risk score, high neutrophil-to-lymphocyte ratio, high platelet-to-lymphocyte ratio, and severe white matter lesions may be poor prognostic factors for patients with ISSNHL. The association between thrombosis-related genes and susceptibility to ISSNHL has been reported in many studies (e.g., coagulation factor 2, coagulation factor 5, plasminogen activator inhibitor-1, platelet-associated genes, a homocysteine metabolism-related enzyme gene, endothelin-1, nitric oxide 3, phosphodiesterase 4D, complement factor H, and protein kinase C-eta). Treatment of ISSNHL with the aim of mitigating the vascular impairment in the inner ear includes systemically administered steroids, intratympanic steroid injections, hyperbaric oxygen therapy, prostaglandin E1, defibrinogenation therapy, and hydrogen inhalation therapy, but there is currently no evidence-based treatment for ISSNHL. Breakthroughs in the unequivocal diagnosis and treatment of ISSNHL due to vascular impairment are crucial to improve quality of life.

Hemifacial spasms triggered by compression of tortuous anterior inferior cerebellar artery loop on the facial nerve in the internal auditory canal: A case report.

RATIONALE: Hemifacial spasm (HFS) is triggered by neurovascular compression mostly at the root entry/exit zone of the facial nerve. HFS with the responsible blood vessel located in the internal auditory canal (IAC) is a very rare occurrence. In our case, the HFS was triggered by compression of the anterior inferior cerebellar artery (AICA) loop on the facial nerve in the IAC. PATIENT CONCERNS: A 27-year-old female presented with a 5-year history of right-sided facial twitching with no obvious course. The frequency and severity of the attacks increases when the patient was anxious or agitated which severely affected her quality of life. DIAGNOSIS: Preoperative 3D-TOF magnetic resonance imaging (MRI) evaluation of cranial nerves showed that the right AICA loop had a tortuous course within the IAC and compressed the facial nerve. INTERVENTION: Microvascular decompression (MVD) surgery was carried out to separate the tortuous AICA loop and facial nerve in the IAC using a Teflon pad. OUTCOMES: The abnormal muscle response disappeared intraoperatively and 2-years follow-up revealed no recurrence of her symptomatology. She is current well and go about her daily activities with no neurological deficits. LESSON: The attachment of the facial nerve to the tortuous AICA loop coupled with the pulsatile impulse of tortuous AICA loop may have resulted in the entrapment and compression of the CN VII in the IAC.

New insights into the ear region anatomy and cranial blood supply of advanced stem Strepsirhini: evidence from three primate petrosals from the Eocene of Chambi, Tunisia.

We report the discovery of three isolated primate petrosal fragments from the fossiliferous locality of Chambi (Tunisia), a primate-bearing locality dating from the late early to the early middle Eocene. These fossils display a suite of anatomical characteristics otherwise found only in strepsirhines, and as such might be attributed either to Djebelemur or/and cf. Algeripithecus, the two diminutive stem strepsirhine primates recorded from this locality. Although damaged, the petrosals provide substantial information regarding the ear anatomy of these advanced stem strepsirhines (or pre-tooth-combed primates), notably the patterns of the pathway of the arterial blood supply. Using µCT-scanning techniques and digital segmentation of the structures, we show that the transpromontorial and stapedial branches of the internal carotid artery (ICA) were present (presence of bony tubes), but seemingly too small to supply enough blood to the cranium alone. This suggests that the ICA was not the main cranial blood supply in stem strepsirhines, but that the pharyngeal or vertebral artery primitively ensured a great part of this role instead, an arterial pattern that is reminiscent of modern cheirogaleid, lepilemurid lemuriforms and lorisiforms. This could explain parallel loss of the ICA functionality among these families. Specific measurements made on the cochlea indicate that the small strepsirhine primate(s) from Chambi was (were) highly sensitive to high frequencies and poorly sensitive to low frequencies. Finally, variance from orthogonality of the plane of the semicircular canals (SCs) calculated on one petrosal (CBI-1-569) suggests that Djebelemur or cf. Algeripithecus likely moved (at least its head) in a way similar to that of modern mouse lemurs.

A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndrome.

The Jervell and Lange-Nielsen (JLN) syndrome (MIM 220400) is an inherited autosomal recessive disease characterized by a congenital bilateral deafness associated with a QT prolongation on the electrocardiogram, syncopal attacks due to ventricular arrhythmias and a high risk of sudden death. JLN syndrome is a rare disease, which seems to affect less than one percent of all deaf children. Linkage to chromosome 11p15.5 markers was found by analysing four consanguinous families. Recombinants allowed us to map the JLN gene between D11S922 and D11S4146, to a 6-cM interval where KVLQT1, a potassium channel gene causing Romano-Ward (RW) syndrome, the dominant form of long QT syndrome, has been previously localized. An homozygous deletion-insertion event (1244, -7 +8) in the C-terminal domain of this gene was detected in three affected children of two families. We found that KVLQT1 is expressed in the stria vascularis of mouse inner ear by in situ hybridization. Taken together, our data indicate that KVLQT1 is responsible for both JLN and RW syndromes and has a key role not only in the ventricular repolarization but also in normal hearing, probably via the control of endolymph homeostasis.

Developmental venous anomaly of the internal auditory canal in a child with unilateral sensorineural hearing loss--a rare association.

Developmental venous anomalies (DVAs) are extremely unusual in the cerebellopontine angle region and internal auditory canal, and constitute a rare etiology of unilateral sensorineural hearing loss (SNHL) in children. We report, to the best of our knowledge, one of the first documented cases of DVA as a probable cause of unilateral SNHL in a child. Our emphasis is on the radiological features.

Superior vestibular dysfunction in severe decompression sickness suggests an embolic mechanism.

BACKGROUND: Both nitrogen bubble embolism and the difficulty of inner ear tissues to wash out nitrogen have been discussed as possible reasons for the selective vulnerability of the inner ear to decompression illness. This case report suggests that nitrogen bubble embolism plays a crucial role in the pathogenesis of inner ear lesions in decompression accidents. CASE REPORT: The current patient, a 48-yr-old male dive master, suffered a severe decompression illness with vertigo as the only residual symptom. At the 1-mo follow-up, neuro-otological evaluation revealed a selective lesion of the superior vestibular division of the left labyrinth with normal functioning inferior vestibular division. At vestibular testing, there was no caloric response from the affected left ear, and the head impulse tests for the lateral and anterior semicircular canal were also impaired. Tests of vestibular evoked myogenic potentials (VEMP) showed divergent results. Ocular VEMP in response to left ear stimulation were absent, whereas the cervical VEMP were completely symmetrical and normal. Thus, the lesion profile implies a partial vestibular loss selectively affecting the superior vestibular division of the inner ear. DISCUSSION: The most likely explanation for such a selective injury seems to be bubble microembolism coupled with both the specific anatomy of this terminally supplied subunit, and with the slow nitrogen wash-out of the vestibular organ.

Inner ear decompression sickness in compressed-air diving.

INTRODUCTION: Inner ear decompression sickness (IEDCS) has become more frequently reported in recreational diving. METHODS: We examined 34 divers after IEDCS and analyzed their dive profiles, pattern of symptoms, time of symptom onset and the association with a right-to left shunt (r/l shunt). RESULTS: Four divers used mixed gas and were excluded from the analysis. Of the remaining 30 divers, 25 presented with isolated IEDCS alone, while five divers had additional skin and neurological symptoms. All divers presented with vertigo (100%), and 12 divers reported additional hearing loss (40%). All symptoms occurred within 120 minutes (median 30 minutes) of ascent. Twenty-two of 30 divers (73.3%) showed a r/l shunt. CONCLUSION: A possible explanation for the frequent association of a r/l shunt and the dominance of vestibular rather than cochlear symptoms could be attributed to the different blood supply of the inner ear structures and the different size of the labyrinthine compartments. The cochlea has a blood supply up to four times higher than the vestibular part of the inner ear, whereas the vestibular fluid space is 30% larger. The higher prevalence of symptoms referrable to the less well-perfused vestibular organ provides further evidence that persistent local inert gas supersaturation may cause growth of incoming arterial bubbles and may therefore be an important pathophysiological factor in IEDCS.

Extended lifetimes of bubbles at hyperbaric pressure may contribute to inner ear decompression sickness during saturation diving.

Inner ear decompression sickness (IEDCS) may occur after upward or downward excursions in saturation diving. Previous studies in nonsaturation diving strongly suggest that IEDCS is caused by arterialization of small venous bubbles across intracardiac or intrapulmonary right-to-left shunts and bubble growth through inward diffusion of supersaturated gas when they arrive in the inner ear. The present study used published saturation diving data and models of inner ear inert gas kinetics and bubble dynamics in arterial conditions to assess whether IEDCS after saturation excursions could also be explained by arterialization of venous bubbles and whether such bubbles might survive longer and be more likely to reach the inner ear under deep saturation diving conditions. Previous data show that saturation excursions produce venous bubbles. Modeling shows that gas supersaturation in the inner ear persists longer than in the brain after such excursions, explaining why the inner ear would be more vulnerable to injury by arriving bubbles. Estimated survival of arterialized bubbles is significantly prolonged at high ambient pressure such that bubbles large enough to be filtered by pulmonary capillaries but able to cross right-to-left shunts are more likely to survive transit to the inner ear than at the surface. IEDCS after saturation excursions is plausibly caused by arterialization of venous bubbles whose prolonged arterial survival at deep depths suggests that larger bubbles in greater numbers reach the inner ear.NEW & NOTEWORTHY Inner ear decompression sickness that occurs during deep saturation diving is explained by arterialization of venous bubbles across intracardiac or intrapulmonary right-to-left shunts and growth of these bubbles if they arrive in the inner ear. Bubbles in arterial blood have prolonged lifetimes at hyperbaric pressures compared with at sea level. This can explain why inner ear decompression sickness is more characteristic of rapid decompressions at great depths than of decompression at sea level.

LDL receptor-related protein 1 (LRP1), a novel target for opening the blood-labyrinth barrier (BLB).

Inner ear disorders are a cluster of diseases that cause hearing loss in more than 1.5 billion people worldwide. However, the presence of the blood-labyrinth barrier (BLB) on the surface of the inner ear capillaries greatly hinders the effectiveness of systemic drugs for prevention and intervention due to the low permeability, which restricts the entry of most drug compounds from the bloodstream into the inner ear tissue. Here, we report the finding of a novel receptor, low-density lipoprotein receptor-related protein 1 (LRP1), that is expressed on the BLB, as a potential target for shuttling therapeutics across this barrier. As a proof-of-concept, we developed an LRP1-binding peptide, IETP2, and covalently conjugated a series of model small-molecule compounds to it, including potential drugs and imaging agents. All compounds were successfully delivered into the inner ear and inner ear lymph, indicating that targeting the receptor LRP1 is a promising strategy to enhance the permeability of the BLB. The discovery of the receptor LRP1 will illuminate developing strategies for crossing the BLB and for improving systemic drug delivery for inner ear disorders.

Bone marrow cell recruitment mediated by inducible nitric oxide synthase/stromal cell-derived factor-1alpha signaling repairs the acoustically damaged cochlear blood-labyrinth barrier.

Using a mouse model with noise-induced cochlear blood-labyrinth-barrier (CBLB) injury, we examined the effects of inducible nitric oxide synthase (iNOS) on the recruitment of bone marrow-derived cells (BMDCs) to the CBLB after acoustic injury. Lethally irradiated C57BL/6J and B6.129P2-Nos2(tm1Lau)/J mice were transplanted with GFP(+)-BMDCs from C57Bl/6-Tg (UBC GFP) mice. Four weeks after transplantation, we assessed the population of GFP(+)-BMDCs in the CBLB. Only small numbers of GFP(+)-BMDCs were found to infiltrate the area of the CBLB in the control recipient mice. However, robust GFP(+)-BMDC migration occurred in the area of the CBLB within the injured cochlea during the first week following acoustic trauma, and further BMDC accumulation was seen by 2 weeks posttrauma. After 4 weeks, the BMDCs were integrated into vessels. Local iNOS from perivascular resident macrophages was found to be important for BMDC infiltration, since mice deficient in iNOS (Inos(-/-)) and mice with iNOS that had been inhibited by 1400W displayed reduced BMDC infiltration. Stromal cell-derived factor-1α (SDF-1α) and its chemokine receptor 4 (CXCR4) were required for the iNOS-triggered recruitment. BMDC recruitment was significantly reduced by the inhibition of SDF-1α activity. Inhibition of the iNOS/SDF-1α signaling pathway reduced vascular repair as observed by reduced vascular density. Our study revealed an intrinsic signaling pathway of iNOS that mediates SDF-1α to promote GFP(+)-BMDC infiltration/targeting in cochlear vascular repair.

Contribution of intracranial vertebral artery asymmetry to vestibular neuropathy.

OBJECTIVES: To test the hypothesis that vertebral artery hypoplasia (VAH) may affect the lateralisation of vestibular neuropathy (VN), probably through haemodynamic effect on the vestibular labyrinth. METHODS: 69 patients with unilateral VN were examined with a magnetic resonance angiographic (MRA) and caloric test. 50 healthy subjects served as controls. The diagnosis of intracranial VAH was based on MRA if <0.22 cm in VA diameter and a diameter asymmetry index >40%. The authors then correlated the canal paretic side with the VAH side. RESULTS: MRA study revealed 29 VAH (right/left: 23/6) in VN subjects and six VAH in controls (right/left: 5/1). The RR of VAH in VN subjects compared with controls was elevated (RR=2.2; 95% CI 1.8 to 2.8). There was a high accordance rate between the side of VAH and VN. Among 29 patients with unilateral VAH, 65.5% (N=19) had an ipsilateral VN, in which left VAH showed a higher accordance rate (83.3%) than the right side (60.9%). VN subjects with vascular risk factors also had a higher VAH accordance rate (81%) than those without (25%). CONCLUSIONS: VAH may serve as a regional haemodynamic negative contributor and impede blood supply to the ipsilateral vestibular labyrinth, contributing to the development of VN, which could be enhanced by atherosclerotic risk factors and the left-sided location.

Sudden sensorineural hearing loss as prodromal symptom of anterior inferior cerebellar artery infarction.

Sudden sensorineural hearing loss is a clinical condition characterized by a sudden onset of unilateral or bilateral hearing loss. In recent years sudden deafness has been frequently described in association with anterior inferior cerebellar artery (AICA) infarction generally presenting along with other brainstem and cerebellar signs such as ataxia, dysmetria and peripheral facial palsy. The authors report a rare clinical case of a 53-year-old man who suddenly developed hearing loss and tinnitus without any brainstem or cerebellar signs. Computed tomography of his brain was normal, and the audiological results localized the lesion causing deafness to the inner ear. Surprisingly, magnetic resonance imaging showed an ischemic infarct in the right AICA territory. This case represents the fifth in the literature to date but it confirms that AICA occlusion can cause sudden deafness even without brainstem or cerebellar signs. Therefore, we recommend submitting the patient for neuroimaging, as an emergency, in order to exclude infarction of the AICA territory. By doing this, it may be possible to limit the extent of the lesion by commencing early therapy.

VEGFA165 gene therapy ameliorates blood-labyrinth barrier breakdown and hearing loss.

Millions of people are affected by hearing loss. Hearing loss is frequently caused by noise or aging and often associated with loss of pericytes. Pericytes populate the small vessels in the adult cochlea. However, their role in different types of hearing loss is largely unknown. Using an inducible and conditional pericyte depletion mouse model and noise-exposed mouse model, we show that loss of pericytes leads to marked changes in vascular structure, in turn leading to vascular degeneration and hearing loss. In vitro, using advanced tissue explants from pericyte fluorescence reporter models combined with exogenous donor pericytes, we show that pericytes, signaled by VEGF isoform A165 (VEGFA165), vigorously drive new vessel growth in both adult and neonatal mouse inner ear tissue. In vivo, the delivery of an adeno-associated virus serotype 1-mediated (AAV1-mediated) VEGFA165 viral vector to pericyte-depleted or noise-exposed animals prevented and regenerated lost pericytes, improved blood supply, and attenuated hearing loss. These studies provide the first clear-cut evidence that pericytes are critical for vascular regeneration, vascular stability, and hearing in adults. The restoration of vascular function in the damaged cochlea, including in noise-exposed animals, suggests that VEGFA165 gene therapy could be a new strategy for ameliorating vascular associated hearing disorders.