Considerations on the physiopathological mechanism of inner ear damage induced by intravenous cocaine abuse: cues from a case report

Objective

Aim of the following paper is to discuss about the possible etiopathogenetic mechanisms of inner ear damage induced by cocaine abuse. Unfortunately the data concerning this topic are very limited; the authors are then presenting a literature review, also discussing the clinical presentation and the possible therapeutical approach of a clinical case of bilateral sudden sensorineural hearing loss following i.v. injection of cocaine.

Patients

Case report.

Intervention

A strictly audiological evaluation has been performed, in order to identify the cochlear lesion site(s) and to provide the best medical treatment.

Conclusions

To our knowledge, this is the first report of acute cocaine intoxication with sudden bilateral hearing loss. Further studies are required in order to understand the effects of these substances on the inner ear cells and metabolism.     

Radiological quiz. Sudden sensorineural hearing loss due to multiple sclerosis

Objective

A case with sudden sensorineural hearing loss (SSNHL) owing to multiple sclerosis (MS) who had clinical and dramatic radiological improvement just after medical therapy was reported in this article.

Method

Case report and review of related literature.

Results

A 22-year-old female patient with MS related SSNHL was presented in this article. Magnetic resonance imaging (MRI) revealed an MS plaque localized at pons extending from right cochlear nucleus to proximal part of the right cochlear nerve. Most dramatic recovery was present in the 5th day control MRI, where the plaque located on pons disappeared completely. On the 10th day control audiogram hearing recovery was observed and pure tone audiogram levels were almost normal.

Conclusion

Sudden sensorineural hearing loss owing to MS is seen more common than expected. It has good prognosis. Magnetic resonance imaging is also thought to have an important role in diagnosis and treatment efficacy of SSNHL owing to MS.     

Middle ear impedance measurements in large vestibular aqueduct syndrome

Objective

To assess the effect of inner ear pressure on middle ear impedance in patients with large vestibular aqueduct syndrome (LVAS).

Methods

Data from admittance tympanometry and multifrequency tympanometry on 8 LVAS patients and control subjects were studied.

Results

Static acoustic compliance (SAC) values for the ears with stable sensorineural hearing loss (SNHL) were within the limits of the mean values of control groups except for two ears. The resonance frequency (RF) values of the ears with stable SNHL were lower than the mean values of control groups except for three ears. SAC values for the two ears with fluctuating SNHL were lower and the RF values were higher than the mean values of control groups.

Conclusion

Decreased SAC values and increased RF values found in the ears with fluctuating SNHL might be an indirect indicator of increased inner ear pressure, while low RF values in the ears with stable SNHL might reflect the decreased inner ear impedance.     

Bilateral secondary neurolymphomatosis of the internal auditory canal nerves: A case report

Background

Neurolymphomatosis describes the malignant lymphomatous infiltration of nerves.

Methods

We encountered a unique case of a 47-year-old patient with non-Hodgkin's lymphoma presenting with bilateral sensorineural hearing loss, vestibular dysfunction and bilateral facial nerve palsy.

Results

Magnetic resonance imaging demonstrated enhancement and thickening of internal auditory canal nerves bilaterally consistent with neurolymphomatosis. Patient was treated with combined intrathecal chemotherapy and total brain irradiation.

Conclusions

One must always remain vigilant for metastatic disease in patients with sensorineural hearing loss and/or vestibular dysfunction and facial nerve palsy in the context of known malignancy.     

Middle ear metastasis of thymoma

Objective

We report an extremely rare case of thymoma which developed middle ear metastasis along with acute sensorineural hearing loss in the contra ear.

Method

We present a case report and a review of the world literature concerning thymoma metastases to the middle ear.

Results

A 54-year-old female patient with thymoma who developed middle ear metastasis along with acute sensorineural hearing loss in the contra ear.

Conclusion

We have not found out thymoma metastases to the middle ear in the past.     

Sudden sensorineural hearing loss associated with inner ear anomaly.

OBJECTIVE: This study was conducted to evaluate the frequency of inner ear anomaly in patients with sudden sensorineural hearing loss and in control subjects. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. PATIENTS AND INTERVENTION: We evaluated 366 patients (165 men and 201 women; age range, 3-91 yr) with sudden sensorineural hearing loss and 228 control subjects without sensorineural hearing loss using magnetic resonance imaging. Three hundred fifty-six patients had unilateral and 10 patients had bilateral sudden sensorineural hearing loss. RESULTS: Eleven (2.9%) of 376 ears with sudden sensorineural hearing loss had inner ear anomaly. Nine patients (2.5%) had inner ear anomaly associated with sudden sensorineural hearing loss, but none of the 228 control subjects had the anomaly. The current study demonstrated that the frequency of inner ear anomaly in patients with sudden sensorineural hearing loss was significantly higher than in control subjects. CONCLUSION: Our study reveals that inner ear anomaly may be associated with sudden sensorineural hearing loss in 2.5% of patients.     

Cochlin in autoimmune inner ear disease: Is the search for an inner ear autoantigen over?

Definition

Autoimmune inner ear disease (AIED) is characterised by a rapidly progressive, often fluctuating, bilateral sensorineural hearing loss over a period of weeks to months. It is an uncommon disease accounting for less than 1% of all cases of hearing impairment or dizziness. The diagnosis is often missed and this impacts on the prognosis as the condition responds well to steroids and immunosuppressants if recognised early.

Lacuna in knowledge

No useful specific test for autoimmunity affecting the inner ear exists.

Objective of study

To gather evidence regarding cochlin in AIED.

Methodology

Systematic review of human studies and animal experimental studies on inner ear antigens was undertaken.

Search strategy

We searched MEDLINE (1965–2012), and Pubmed for relevant studies. A combination of key words for inner ear, autoimmunity (autoimmune, immune mediated) and cochlin were used.

Results

A number of antigens have been implicated in autoimmune inner ear disease. Cochlin is a major component of the extracellular matrix in the inner ear and a promising candidate. We present evidence in literature on the role of this protein in the pathogenesis of autoimmune inner ear disease.     

Clinical features of sudden hearing loss associated with a high signal in the labyrinth on unenhanced T1-weighted magnetic resonance imaging

We report on five patients with high signals in the labyrinth on unenhanced magnetic resonance imaging who developed sudden hearing loss and vertigo. Weissman et al. (1992) suggested the possibility that such high signals were caused by hemorrhage. We assessed these patients using audiograms, caloric tests, and auditory brainstem responses to investigate the possibility of inner ear hemorrhage. Most of the patients were found to have severe and irreversible impairment of both cochlear and vestibular function. These findings were consistent with the hypothesis that their symptoms were caused by inner ear hemorrhage. Received: 3 November 1999 / Accepted: 20 January 2000     

Autoimmune recurrent facial palsy and bilateral sudden sensorineural hearing loss following Ramsay Hunt-like syndrome

Objective

To describe the first published case of recurrent facial nerve palsy associated with bilateral sudden sensorineural hearing loss of autoimmune origin.

Case report

A 33-year-old male presented with acute facial palsy on the left following a vesicular herpetic eruption in the external ear canal on the same side. Serologic measurements demonstrated an elevation of IgM antibodies against herpes simplex virus but not for varicella-zoster virus, confirming a Ramsay Hunt-like syndrome due to herpes simplex virus. The following four months, the patient exhibited other three episodes of facial palsy, well responded to steroid treatment. During the clinical course, a sudden sensorineural hearing loss was also diagnosed, initially on the left side and then on both sides. The autoimmune markers such as the antinuclear antibody and the anti-gangliosides antibodies (anti-GM1, anti-GQ1b) were found positive. Despite steroid treatment, hearing did not show any improvement, remaining moderate on the right and severe on the left.

Conclusion

Recurrent facial nerve palsy and bilateral sudden sensorineural hearing loss could be the expression of autoimmune disturbances. The initial triggered factor could be the herpes simplex virus infection, such as a Ramsay Hunt-like syndrome.     

Sudden hearing loss with simultaneous posterior semicircular canal BPPV: Possible etiology and clinical implications

Objectives

The objectives of this study were to describe the clinical course and outcome of patients with sudden sensorineural hearing loss (SSNHL) in conjunction with benign paroxysmal positional vertigo (BPPV), and hypothesize the possible pathophysiology of this entity.

Study design

Retrospective study of all patients with evidence of SSNHL with any type of BPPV between 2008 and 2012.

Settings

Tertiary care university hospital.

Subjects and methods

Five patients aged 56 to 71 were diagnosed with unilateral profound SSNHL and BPPV. Neurotologic examination revealed an ipsilateral torsional, up-beating nystagmus on Dix–Hallpike exam. Severe or profound ipsilateral–sensorineural hearing loss was recognized on audiometry. The rest of the exam was normal; this was in keeping with the diagnosis of SSNHL with ipsilateral posterior semicircular canal BPPV.

Results

All patients were treated with a modified Epley maneuver; oral steroids were administered for two weeks. In all cases vertigo resolved and the Dix–Hallpike exam became normal within several weeks. However, the hearing loss remained unchanged in two patients. Magnetic resonance imaging of the head was normal and ENG caloric test demonstrated mild ipsilateral canal paresis in two patients.

Conclusions

1. Patients with SSNHL and BPPV can have a variable clinical course and outcome. This entity may be quite common, but the diagnosis of BPPV can be missed if a complete neurological physical examination is not performed. 2. Arterial occlusions or selective multiple vascular or neural involvement may explain the pathophysiology of SSNHL with BPPV of the posterior semicircular canal.     

Sudden Sensorineural Hearing Loss: Results of intratympanic steroids as salvage treatment

Objective

The aim of the present study was to verify the efficacy and the safety of intratympanic dexamethasone to treat sudden sensorineural hearing loss as salvage therapy.

Materials and methods

A prospective study was conducted on patients affected by idiopathic sudden hearing loss who were treated before with some systemic therapy, but without recovery of the hearing The patients able to undergo the study, but who refused salvage treatment were considered as control group. A solution of Dexamethasone 4 mg/ml was then injected through the posterior–inferior quadrant filling completely the middle ear. The follow-up in the following 6 months included an audiogram every month.

Results

The number of patients treated with salvage therapy was 36. The patients who refused treatment were further 10. The salvage treatment was done with a mean delay of 24.3 days from the onset of symptoms. Mean hearing threshold after the onset of sudden hearing loss at PTA was 66.5 dB. After the failed treatment the mean PTA was 59.6 dB. The mean PTA after the intratympanic steroid administration was 46.8 dB, with a mean improvement of 12.8 dB. No hearing change was noted in the 10 patients who refused salvage therapy. The patients that assumed systemic steroid as first therapy showed a better PTA threshold after the salvage intratympanic treatment (p < 0.01). A significant difference (p < 0.05) of hearing recovery was evidenced between non-smoker patients and those with smoking habit.

Conclusions

Our data showed that a salvage treatment with intratympanic dexamethasone should be suggested to all patients who failed the first systemic treatment. The systemic steroid therapy done before the salvage treatment seems to exert a protective role for the inner ear, as shown by our series. On the contrary the smoke habit is a negative prognostic factor in the hearing recovery.     

Computed tomography demonstrates abnormalities of contralateral ear in subjects with unilateral sensorineural hearing loss

Purpose

Prior studies have associated gross inner ear abnormalities with pediatric sensorineural hearing loss (SNHL) using computed tomography (CT). No studies to date have specifically investigated morphologic inner ear abnormalities involving the contralateral unaffected ear in patients with unilateral SNHL. The purpose of this study is to evaluate contralateral inner ear structures of subjects with unilateral SNHL but no grossly abnormal findings on CT.

Materials and methods

IRB-approved retrospective analysis of pediatric temporal bone CT scans. 97 temporal bone CT scans, previously interpreted as “normal” based upon previously accepted guidelines by board certified neuroradiologists, were assessed using 12 measurements of the semicircular canals, cochlea and vestibule. The control-group consisted of 72 “normal” temporal bone CTs with underlying SNHL in the subject excluded. The study-group consisted of 25 normal-hearing contralateral temporal bones in subjects with unilateral SNHL. Multivariate analysis of covariance (MANCOVA) was then conducted to evaluate for differences between the study and control group.

Results

Cochlea basal turn lumen width was significantly greater in magnitude and central lucency of the lateral semicircular canal bony island was significantly lower in density for audiometrically normal ears of subjects with unilateral SNHL compared to controls.

Conclusion

Abnormalities of the inner ear were present in the contralateral audiometrically normal ears of subjects with unilateral SNHL. These data suggest that patients with unilateral SNHL may have a more pervasive disease process that results in abnormalities of both ears. The findings of a cochlea basal turn lumen width disparity >5% from “normal” and/or a lateral semicircular canal bony island central lucency disparity of >5% from “normal” may indicate inherent risk to the contralateral unaffected ear in pediatric patients with unilateral sensorineural hearing loss.     

Lateral petrosectomy with obliteration cavity for spontaneous cerebrospinal otorrhea in children

Purpose

The most common causative factors of CSF otorrhea in children are injuries and congenital abnormalities of the temporal bone. Spontaneous CSF leak as a consequence of congenital temporal bone defects may result in recurrent meningitis. Diagnosis and management of such an entity are particularly difficult in early childhood.

Materials and methods

The aim of this study was to investigate clinical features and to discuss possible methods of treatment of spontaneous CSF otorrhea in children.

Results

Severe unilateral sensorineural hearing loss or total deafness was found in children with CSF otorrhea. CT and MRI of the temporal bones revealed dehiscences in the walls of the tympanic cavity and defects of the inner ear, which were confirmed intraoperatively. Lateral petrosectomy and closure of the fistula with muscle tissue and fat obliteration cavity were performed. The children remain free of otorrhea and recurrences of meningitis.

Conclusion

The diagnosis of spontaneous otorrhea in children is based on the severe unilateral sensorineural hearing loss and presence of CSF in the middl? ear cavity. It may be successfully treated by means of lateral petrosectomy with obliteration of the vestibule with muscle tissue and tympanic cavity with fat tissue.     

Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging findings and prognosis in sudden sensorineural hearing loss

Objectives/Hypothesis: Three‐dimensional fluid‐attenuated inversion recovery (3D‐FLAIR) magnetic resonance imaging (MRI) has recently been developed to detect high concentrations of protein or hemorrhage. We have previously reported that 50% of patients with sudden sensorineural hearing loss (SNHL) show high signals in the affected inner ear on 3D‐FLAIR MRI. However, the relationship between 3D‐FLAIR findings and hearing prognosis is unclear. Our objective was to evaluate the relationship between the results of 3D‐FLAIR MRI at 3 Tesla and prognosis in sudden SNHL. Study Design and Methods: We used 3D‐FLAIR at 3 Tesla with and without gadolinium enhancement to evaluate the pathologic conditions in the inner ears of 48 patients with sudden SNHL. Results: Thirty‐one of 48 patients with sudden SNHL showed high signals in the affected inner ear on precontrast 3D‐FLAIR. Hearing improvement in patients with high signals in the affected inner ear on precontrast 3D‐FLAIR (25 ± 19 dB) was significantly worse than that in patients with no signal (45 ± 27 dB; P < .05). Our analysis suggests that high signals in the affected inner ear on precontrast 3D‐FLAIR MRI is a new prognostic factor for sudden SNHL. Conclusions: 3D‐FLAIR findings show that high signals in the cochlea on precontrast 3D‐FLAIR are related to a poor hearing prognosis. These signals may reflect minor hemorrhage or an increased concentration of protein in the inner ear, which has passed through blood vessels with increased permeability or has originated in disrupted cells in the inner ear.     

Quantification of hearing loss associated with superior semi-circular canal dehiscence

Introduction

Superior semi-circular canal dehiscence (SSCD) is a known cause of hearing loss. This study quantifies hearing loss in SSCD ears in a frequency-specific fashion.

Methods

A meta-analysis of English language literature pertaining to SSCD was performed, with extraction and evaluation of available human audiometric data. Our own institution's case series of SSCD patients was also similarly analysed. Hearing loss in SSCD ears was compared to same patient control ears and to age-matched normative audiometric data.

Results

Ears with SSCD had statistically significant worse hearing as compared to both normative data and to own normal ear controls at 2000 Hz and below. The effect appears to diminish with increasing frequency.

Discussion

The presence of statistically significant conductive hearing loss in the low frequencies was confirmed for SSCD ears. SSCD may also predispose ears to high frequency sensorineural hearing loss.     

Edaravone,a free radical scavenger,in the treatment of idiopathic sudden sensorineural hearing loss with profound hearing loss

Objective

Edaravone, a free radical scavenger, is a clinical drug that is widely used to reduce neuronal damage after acute cerebral infarction in Japan since 2001. The aim of this study was to investigate whether edaravone could improve treatment result in idiopathic sudden sensorineural hearing loss (ISSHL) patients with severe hearing loss.

Methods

Between 2004 and 2006, 14 patients of ISSHL with the mean hearing levels equal or over 90 dB at the initial visit were treated with edaravone. 14 counterpart control patients were selected from 45 patients who had similar prognostic factors and were treated with hyperbaric oxygenation therapy (HBO) in the past decade.

Results

There were no significant differences between edaravone group and the control group in hearing recovery.

Conclusion

We considered that edaravone was not able to bring remarkable effect compared with conventional treatment regimen for ISSHL.     

Non-organic hearing loss in Chinese teenagers

Objective

To heighten the physician's awareness of non-organic hearing loss in teenagers in China.

Methods

Retrospective cases review of seven patients (six girls and one boy) with sudden hearing loss was conducted.

Results

Five patients presented with hearing loss bilaterally and two patients unilaterally. All patients suffered from severe to profound hearing loss. However, the acoustic reflex test indicated direct and indirect responses were present bilaterally at 1000 Hz 100 dB SPL. The results of ABR test revealed hearing threshold within 20–30 dB nHL. Further investigations indicated non-organic hearing loss was associated with school stress or environment conflict. Satisfactory outcomes were achieved in all patients.

Conclusion

Non-organic hearing loss should be considered when teenagers present with severe to profound sudden hearing loss if the acoustic reflex is present. School and home stresses are associated with the occurrence of non-organic hearing loss in the present study.     

Cochlear implantation in 13q deletion syndrome

Purpose

The advent of cochlear implantation has been followed by an explosion in surgical and technological advances with subsequent alterations in the treatment of sensorineural hearing loss management. Many individuals with rare genetic abnormalities who once may have not been considered candidates for cochlear implantation are now benefiting from cochlear implantation. One of these unusual syndromes is the 13q deletion syndrome. The clinical features of 13q deletion syndrome, a rare chromosomal abnormality, were originally described in the early 1960s, though the literature currently lacks defined phenotypic abnormalities. Patients with 13q deletion syndrome present with varying degrees of cognitive and growth delay, craniofacial dysmorphisms, and congenital malformations. The etiology of the sensorineural hearing loss has not been elucidated, and genes associated with other syndromes which include sensorineural hearing loss have been proposed as the causative agents. We describe the unique clinical and surgical considerations for 13q deletion syndrome and review the considerations when deliberating on cochlear implantation in syndromic hearing loss.

Materials and Methods

Case report.

Results

Successful cochlear implantation in a patient with 13q deletion syndrome.

Conclusion

Many patients with 13q deletion syndrome suffer from sensorineural hearing loss, and when appropriately selected may likely benefit from cochlear implantation. Many other syndromic patients following careful selection may also benefit from cochlear implantation and should not be excluded from consideration until appropriately evaluated.     

The limitations of computed tomography in adult cochlear implant evaluation

Objective

To demonstrate the added value of magnetic resonance imaging (MRI) over computed tomography (CT) during adult cochlear implant evaluation.

Patients

Two adult patients are discussed in whom MRI studies diagnosed bilateral vestibular schwannomas during cochlear implant candidacy evaluation.

Interventions

Temporal bone CT and MRI.

Main outcome measure

Diagnosis of NF2.

Results

Two adult patients, ages 67 and 68 years, were evaluated for cochlear implant candidacy. Both patients experienced slowly progressive, bilateral hearing loss without complaints of vertigo, and neither patient had a family history of hearing loss or neurogenic tumors. Both patients had near-symmetric pure tone thresholds on audiometric testing. An MRI and a CT scan were performed on both patients, and bilateral vestibular schwannomas were identified on MRI.

Conclusions

Though MRI is not routinely utilized in adult cochlear implant evaluation, it may be of greater clinical value than CT in the setting of adult-onset hearing loss. MRI allows for sensitive evaluation of cochlear patency and architecture, and cochlear nerve status. As demonstrated in the two index cases, MRI also provides the added benefit of evaluating for causes of retrocochlear hearing loss. These two patients would have likely experienced a significant delay in diagnosis of NF2 without preoperative MRI, particularly given the limitations of scanning following CI magnet placement.     

Minimizing the dead ear in otosclerosis surgery

Objective

Assessment of a specific surgical technique regarding the postoperative hearing results and the incidence of “dead ear” comparing local and general anesthesia.

Methods

We present a retrospective review of all stapedectomies performed by the same surgeon under general and local anesthesia during a 9-year period (1997–2006) in non-revision cases. The analysis of our data (268 operations, 160 under general and 108 under local anesthesia) included hearing results based on the guidelines from the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology-Head and Neck Surgery and complications recorded in the case notes. Excessive vertigo during stapedectomy under local anesthesia considered as a sign of possible profound sensorineural hearing loss.

Results

A postoperative air-bone gap up to 10 dB (Grade A) was successfully obtained in 92.6% of operated ears. The mean postoperative air-bone gap in decibels was 6.8 dB. There were no statistically significant differences between general and local anesthesia in hearing results. Excessive intraoperative dizziness occurred in five patients of local anesthesia group without postoperative sensorineural hearing loss. The incidence of dead ear was 1.8 % (three patients) under general and 0% under local anesthesia.

Conclusion

Stapedectomy is a safe procedure in hands of an experienced otologist with minimum major complications especially when performed under local anesthesia. Local anesthesia offers the immediate evaluation of hearing restoration and the early recognition of possible dead ear. Training programs should consider stapedectomy under local anesthesia as a standard procedure for trainees in non-revision cases.