Systemic lupus erythematosus and hearing disorders: literature review and meta-analysis of clinical and temporal bone findings

Objective: This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design: Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed. Methods: PubMed and Google Scholar searches were performed using the following keywords: “auto-immune disease,” “systemic lupus erythematosus (SLE),” “hearing loss,” “temporal bone study,” “vertigo,” “dizziness,” “tinnitus,” “ear symptoms,” “treatment,” “diagnosis,” “symptoms,” “etiopathogenesis,” “Wegener granulomatosis,” “Sjogren,” “polyarteritis nodosa,” “Cogan syndrome,” and “granulomatosis.” Also included were reviews in which the following terms were present: “SLE,” “temporal bone,” and “hearing symptoms.” Review and conclusion: This literature review and meta-analysis focused on the pathological mechanisms through which SLE can damage inner ear structures and determinate hearing and vestibular symptoms. The main mechanisms involved in inner ear damage include the autoimmune response, deposition of immune complexes in the vessels and, to a lesser extent, cytotoxic damage

Relationship between hearing function and myasthenia gravis: a contemporary review

There is increasing evidence of a connection between hearing function and myasthenia gravis (MG). Studies of the pathophysiological basis of this relationship suggest that acetylcholine receptors (AChRs) on outer hair cells (OHCs) play a central role. In patients with MG, autoantibodies against AChRs induce a progressive loss of AChRs on OHCs, decreasing their electromotility. The stapedial reflex decay test can be altered in MG patients, and can be used as an additional tool for diagnosis and monitoring. Transient evoked and distortion product otoacoustic emissions are the main diagnostic tool for monitoring OHC functionality in MG patients, and can be used to record subclinical hearing alterations before the onset of clinically evident hearing loss. Understanding the association between MG and hearing dysfunction requires a multidisciplinary approach. Otolaryngologists should take this relationship into account when approaching patients with a diagnosis of myasthenia gravis and "in patients with MG" with ण128;\u9cin MG patients, and the progress of hearing alterations should always be monitored in patients with MG

Autoimmunity and otolaryngology diseases

Many systemic autoimmune diseases have otolaryngology manifestations that could represent a diagnostic challenge for clinicians, as they often constitute an early sign of an otherwise asymptomatic autoimmune condition and may lead to delayed diagnosis and treatment. In other cases, otolaryngology manifestations can be overlooked in patients with previously diagnosed autoimmune diseases. The pres- ence of concomitant conditions, the heterogeneity of studies available in the literature, and the lack of randomized trials are factors that may limit the prompt diagnosis of otolaryn- gology manifestations in systemic autoimmune diseases, with underestimation of the problem and undertreatment of the related condition

Congenital Aural Atresia: Hearing Rehabilitation by Bone-Anchored Hearing Implant (BAHI)

Auris atresia (AA) is a congenital pathology characterized by aplasia or hypoplasia of the external ear with associated middle ear malformation. The AA has a different degree of severity, and the severe form of the disorder presents no identifiable ear canal (complete atresia) and absence or significative underdevelopment of the middle ear structures. Sometimes AA is associated with a malformation of the ear called “microtia.” The alterations of the external auditory canal and of the middle ear structures are responsible for the conductive hearing loss which affects the patients. The hearing restoration procedures may recreate a normal external and middle ear anatomy to favor the recovery of the hearing function, or the surgeon may simply restore the hearing capacity through bypassing the malformed structures by bone-anchored hearing implants (BAHIs). The restoring of normal anatomy is generally associated with episode of restenosis of the external ear canal due to bony regrowth. The formulation of a therapeutic strategy may be supported by using Jahrsdoerfer classification to identify the severity of malformation. In the chapter we discuss various bone anchoring prostheses currently used (Baha, Ponto, Alpha2 by Sophono, Bonebridge) and the results that can be obtained by the use of these implants

Nucleolus vs nucleus count for identifying spiral ganglion in human temporal bone

OBJECTIVES: Spiral ganglion (SG) counting is used in experimental studies conducted on age-, noise-, and drug-induced sensorineural hearing loss, as well as in the assessment of cochlear implant performances. Different methods of counting have been reported, but no definite standardization of such procedure has been published. The aim of our study is to identify the best method to count human spiral ganglions (SGs). MATERIAL and METHODS: By identification of nuclei or nucleoli as described by Schucknect, seven researchers with different experience levels counted SGs in 123 human temporal bones (TBs). Data on time of post-mortem bone removal post-mortem, methods of specimen’s fixation, decalcification, and coloration were collected to test their possible influence on human tissue. Percentage, two-tailed t-test, Spearman’s test, and one-way ANOVA were used to analyze the data. RESULTS: Nucleoli were identified in 61% of cases, whereas nuclei were recognized in 100% of cases (p<0.005). Nucleoli presence in all four segments in the same temporal bone (TB) was observed in 69 cases (92%), whereas nuclei were identified in all four segments in 103 cases (83.7%) (p<0.001). The junior investigators requested a double check by the seniors in 25 (20.3%) cases for identifying and counting nucleoli, whereas the senior researchers showed no doubts in their identification and count. The only parameter positively affecting nucleoli identification in tissue preparation was bone removal for <12 h with respect to longer post-mortem time (p<0.001). CONCLUSION: We suggest counting nuclei, rather than nucleoli, for spiral ganglion computation because of easier recognition of nuclei, especially in case of investigator’s limited experience

Hearing impairment in MELAS: new prospective in clinical use of microRNA, a systematic review

Aim To evaluate the feasibility of microRNAs (miR) in clinical use to fill in the gap of current methodology commonly used to test hearing impairment in MELAS patients. Material and method A literature review was performed using the following keywords, i.e., MELAS, Hearing Loss, Hearing Impairment, Temporal Bone, Otoacustic Emission (OTOAE), Auditory Brain Response (ABR), and microRNA. We reviewed the literature and focused on the aspect of the temporal bone, the results of electrophysiological tests in human clinical studies, and the use of miR for detecting lesions in the cochlea in patients with MELAS. Results In patients with MELAS, Spiral Ganglions (SG), stria vascularis (SV), and hair cells are damaged, and these damages affect in different ways various structures of the temporal bone. The function of these cells is typically investigated using OTOAE and ABR, but in patients with MELAS these tests provide inconsistent results, since OTOAE response is absent and ABR is normal. The normal ABR responses are unexpected given the SG loss in the temporal bone. Recent studies in humans and animals have shown that miRs, and in particular miRs 34a, 29b, 76, 96, and 431, can detect damage in the cells of the cochlea with high sensitivity. Studies that focus on the temporal bone aspects have reported that miRs increase is correlated with the death of specific cells of the inner ear. MiR − 9/9* was identified as a biomarker of human brain damage, miRs levels increase might be related to damage in the central auditory pathways and these increased levels could identify the damage with higher sensitivity and several months before than electrophysiological testing. Conclusion We suggest that due to their accuracy and sensitivity, miRs might help monitor the progression of SNHL in patients with MELAS

Audio-vestibular symptoms in systemic autoimmune diseases

Immune-mediated inner ear disease can be primary, when the autoimmune response is against the inner ear, or secondary. The latter is characterized by the involvement of the ear in the presence of systemic autoimmune conditions. Sensorineural hearing loss is the most common audiovestibular symptom associated with systemic autoimmune diseases, although conductive hearing impairment may also be present. Hearing loss may present in a sudden, slowly, rapidly progressive or fluctuating form, and is mostly bilateral and asymmetric. Hearing loss shows a good response to corticosteroid therapy that may lead to near-complete hearing restoration. Vestibular symptoms, tinnitus, and aural fullness can be found in patients with systemic autoimmune diseases; they often mimic primary inner ear disorders such as Menière’s disease and mainly affect both ears simultaneously. Awareness of inner ear involvement in systemic autoimmune diseases is essential for the good response shown to appropriate treatment. However, it is often misdiagnosed due to variable clinical presentation, limited knowledge, sparse evidence, and lack of specific diagnostic tests. The aim of this review is to analyse available evidence, often only reported in the form of case reports due to the rarity of some of these conditions, of the different clinical presentations of audiological and vestibular symptoms in systemic autoimmune diseases

Hearing loss, tinnitus, hyperacusis, and diplacusis in professional musicians: a systematic review

Professional musicians (PMs) are at high risk of developing hearing loss (HL) and other audiological symptoms such as tinnitus, hyperacusis, and diplacusis. The aim of this systematic review is to (A) assess the risk of developing HL and audiological symptoms in PMs and (B) evaluate if different music genres (Pop/Rock Music-PR; Classical Music-CL) expose PMs to different levels of risk of developing such conditions. Forty-one articles including 4618 PMs were included in the study. HL was found in 38.6% PMs; prevalence was significantly higher among PR (63.5%) than CL (32.8%) PMs; HL mainly affected the high frequencies in the 3000-6000 Hz range and was symmetric in 68% PR PMs and in 44.5% CL PMs. Tinnitus was the most common audiological symptom, followed by hyperacusis and diplacusis. Tinnitus was almost equally distributed between PR and CL PMs; diplacusis was more common in CL than in PR PMs, while prevalence of hyperacusis was higher among PR PMs. Our review showed that PR musicians have a higher risk of developing HL compared to CL PMs; exposure to sounds of high frequency and intensity and absence of ear protection may justify these results. Difference in HL symmetry could be explained by the type of instruments used and consequent single-sided exposure

Otolaryngologic symptoms in multiple sclerosis: a review

Many symptoms of multiple sclerosis may affect the ear, nose and throat. The most common otolaryngologic symptoms of multiple sclerosis are speech disorders, followed by sleep disorders, vertigo and disequilibrium, dysphagia, smell alterations, and hearing loss. Less common symptoms include sialorrhea, facial palsy, taste alterations, trigeminal neuralgia and tinnitus. The origin of otolaryngologic symptoms in multiple sclerosis is mainly central, although increasing evidence also suggests a peripheral involvement. Otolaryngologic symptoms in multiple sclerosis may have different clinical presentations; they can appear in different stages of the pathology, in some cases they can be the presenting symptoms and their worsening may be correlated with reactivation of the disease. Many of these symptoms significantly affect the quality of life or patients and lead to increased morbidity and mortality. Otolaryngologic symptoms are common in multiple sclerosis; however, they are often overlooked. In many cases, they follow the relapsing-remitting phases of the disease, and may spontaneously disappear, leading to a delay in multiple sclerosis diagnosis. Clinicians should be aware of otolaryngologic symptoms of multiple sclerosis, especially when they are associated to neurologic symptoms, as they may be early signs of a still undiagnosed multiple sclerosis or could help monitor disease progression in already diagnosed patients

A minimally invasive endoscope assisted retrosigmoid approach for removal of arachnoid cysts in the internal auditory canal: a step by step description

Abstract Introduction An arachnoid cyst in the internal auditory canal is a quite rare pathology but due to its compressive action on the nerves in this district should be surgically removed. Several surgical techniques have been proposed but no surgeons have used the minimally assisted endoscope retrosigmoid approach for its removal. Objective To investigate the feasibility of using a minimally invasive endoscope assisted retro-sigmoid approach for surgical removal of arachnoid cysts in the internal auditory canal. Methods Minimally invasive endoscope assisted retrosigmoid approach allows to access to the internal auditory canal through a minimally invasive retrosigmoid approach that combines the use of a microscope and an endoscope. It is performed in six steps: soft tissue step, bone step, dura step, cerebellopontine angle step (performed using an endoscope and a microscope), microscope-endoscope assisted arachnoid cysts removal and closure. We tested minimally invasive endoscope assisted retrosigmoid approach for removal of arachnoid cysts in the internal auditory canal on two human cadaveric heads (specimens) of subjects affected from audio-vestibular disorders and with arachnoid cysts in the internal auditory canal confirmed by magnetic resonance imaging. Results The mass was completely and successfully removed from the two specimens with no damage to the nerves and/or vessels in the surgical area. Conclusion The results of our study are encouraging and support the feasibility of using minimally invasive endoscope assisted retrosigmoid approach for removal of arachnoid cysts in the internal auditory canal. While further clinical in-vivo studies are needed to confirm the accuracy and safety of using the minimally invasive endoscope assisted retrosigmoid approach for this specific surgery, our group has successfully used the minimally invasive endoscope assisted retrosigmoid approach in the treatment of microvascular compressive syndrome, schwannoma removal and vestibular nerve resection